Neonatal and Infant Dermatology, 3e

Front cover
Neonatal and Infant Dermatology
Copyright page
Table of Contents
Foreword
Preface
List of Contributors
Dedication
1 Fetal Skin Development
	Introduction
	Timing of embryonic and  fetal development
	Epidermis
		Embryonic development
		Early fetal development
		Late fetal development
		Clinical relevance
		Specialized cells within the epidermis
		Clinical relevance
		Nerves and vasculature
	Dermis and subcutis
		Overview
		Embryonic dermal development
		Fetal dermal development
		Clinical relevance
		Development of the hypodermis  and adipose tissue
	Combined dermoepidermal structures
		Dermoepidermal junction
		Clinical applications
		Development of appendages
		Hair follicle and sebaceous  gland development
		Nail development
		Eccrine and apocrine sweat  gland development
		Ectodermal appendages:  clinical relevance
	Clinical paradigms
		Innate immune function
		Prenatal diagnosis of congenital  skin disorders
	Gestational age assessment
	Acknowledgments
	Key references
	References
2 Structure and Function of  Newborn Skin
	Introduction
	Stratum corneum and epidermis
	Dermoepidermal junction
	Dermis and subcutaneous fat
	Pilosebaceous units, apocrine glands, and nails
		Hair follicles
		Sebaceous glands
		Apocrine glands
		The nail
	Eccrine glands and neonatal sweating
	Nerves, vascular networks, and thermoregulation
	Melanocytes and pigmentation  of the skin
	Cutaneous immunosurveillance, Langerhans’ cells, and cytokines
		Cutaneous immunosurveillance
		Langerhans’ cells
		Cytokines
	Key references
	References
3 Lesional Morphology and Assessment
	Introduction
	Reaction patterns
	A comprehensive history and  its impact
		Prenatal history
		Maternal history
	Cutaneous examination and evaluation
	Considerations unique to the neonatal period
	References
4 Skin of the Premature Infant
	Introduction
	The permeability barrier in the preterm infant
	Consequences of permeability  barrier immaturity
		Fluid and electrolyte imbalance and evaporative energy loss
			Strategies for minimizing fluid and evaporative energy losses
		Increased percutaneous absorption  of xenobiotics
			Clinical implications of increased percutaneous absorption of xenobiotics
		Impact of permeability barrier immaturity on other organ systems
	Immaturity of other skin barriers
		Cutaneous barrier to  mechanical injury
		Cutaneous barrier to infection
		Cutaneous barrier to light injury
		Cutaneous contribution to immaturity of thermal homeostasis
		Neurocutaneous development in the premature infant
	Skin diseases in the premature infant
		Barrier function of abnormal skin
		Scars of prematurity
		Hemangiomas
	Summary
	Acknowledgment
	Key References
	References
5 Neonatal Skin Care and Toxicology
	Introduction
	Structure and function of neonatal skin
		Gene–environment interaction
	Skin care practices
		Skin cleansing
		Emollients
		Infant massage
		Diapering and diaper care products
		Ultraviolet protection and  thermal injury
	Percutaneous absorption and toxicity
	Special issues in the NICU (see Chapter 4)
		Skin antisepsis
		Adhesives
		Transepidermal water loss (TEWL)
	Skin care guidelines
	Summary
	Key References
	References
6 Diagnostic and Therapeutic Procedures
	Introduction
	Diagnostic procedures
		Bacterial culture
		KOH preparation
		Fungal culture
		Direct fluorescent antibody test for diagnosis of herpes virus infection
		Viral culture
		Polymerase chain reaction (PCR) test for diagnosis of herpes virus infection
		Scabies preparation
		Dermoscopy
		Microscopic hair examination
		Wood’s light examination
		Skin biopsy
		Electron microscopy
		Immunofluorescence
	Therapeutic procedures
		Immobilization
		Topical anesthetics
			EMLA®
			Topical lidocaine  (LMX®, formerly ELA-Max®)
		Injectable anesthetics
		Giving an injection
		General anesthesia
		Wound care following a procedure
		Laser surgery
	Key references
	References
7 Transient Benign Cutaneous Lesions in the Newborn
	Introduction
	Papules and pustules
		Milia
		Oral mucosal cysts of the newborn (palatal cysts or epstein’s pearls, and alveolar cysts or bohn’s nodules)  (SEE ALSO Chapter 30)
		Perineal median raphe cysts and foreskin cysts
		Miliaria
		Sebaceous hyperplasia
		Erythema toxicum neonatorum  (toxic erythema of the newborn,  ‘flea bite’ dermatitis)
		Transient neonatal pustular melanosis
		Neonatal acne (cephalic pustulosis)
	Sucking blisters, erosions, pads,  and calluses
	Umbilical granuloma, patent urachus and omphalomesenteric duct remnant (umbilical polyp)
	Color changes in the newborn (Box 7.1)
		Pigmentary abnormalities resulting from abnormalities of melanin
			Dermal melanosis (Mongolian spots)
		Transient epidermal hyperpigmentation
		Transient hypopigmentation
		Transient pigmentary changes not caused by melanin
		Color changes resulting from vascular abnormalities
			Cutaneous vasomotor instability
			Rubor resulting from excessive hemoglobin
			Capillary ectasias (nevus simplex, salmon patch)
	Vernix caseosa
	Desquamation
	Key references
	References
8 Iatrogenic and Traumatic Injuries
	Introduction
	Puncture wounds
		Amniocentesis
		Chorionic villus sampling
		Fetal monitoring
		Needle marks and scars
	Birth-related trauma to the skin  and scalp
		PETECHIAE AND ECCHYMOSES
			Caput succedaneum
			Cephalhematoma
			Subgaleal hemorrhage
			Untoward effects of vacuum extraction
			Halo scalp ring
			Lacerations
	Burns and thermal injury
		Chemical burns
		Thermal injury
		Transillumination blisters
		Subcutaneous fat necrosis associated with therapeutic hypothermia
	Mechanical injury
		Dermal stripping
	Pressure ulcers
	Umbilical artery catheterization
	Perinatal gangrene of the buttock
	Complications of phototherapy
		Phototherapy-induced drug eruptions
		Transient porphyrinemia and phototherapy eruptions
		Bronze baby syndrome
	Calcinosis cutis
		Calcinosis cutis from infusion of calcium salts
		Calcified nodules of the heels
	Infiltration and extravasation injuries
	Phlebitis
	Complications of monitoring
		Anetoderma of prematurity
		Pulse oximetry
		Transcutaneous oxygen monitoring
		Transcutaneous carbon  dioxide monitoring
	Meatal ulceration following circumcision
	Cutaneous necrosis following suture ligation of supernumerary digits and accessory tragi
	Auriculotemporal nerve syndrome
	Horner syndrome
	Linear bands of infancy
	Cutaneous vaccination reactions
	Non-accidental trauma
	Key references
	References
9 Developmental Abnormalities
	Introduction
	Supernumerary mammary tissue
		Extracutaneous findings
		Diagnosis
		Treatment
	Preauricular pits and sinuses
		Extracutaneous findings
		Diagnosis and treatment
	Accessory tragi
		Diagnosis and treatment
	Cervical tabs/wattles/congenital cartilaginous rests of the neck
	Supernumerary digits (rudimentary polydactyly)
	Branchial cysts, branchial clefts, and branchial sinuses
	Thyroglossal duct cysts
	Cutaneous bronchogenic cysts  and sinuses
	Median raphe cysts
	Ventral midline clefts/defects
		Supraumbilical cleft
		Midline cervical clefts
	Cutaneous signs of neural  tube dysraphism
		Cranial dysraphism
			Cephaloceles/cutaneous neural heterotopias
			Cutaneous findings
			Extracutaneous findings and diagnosis
			Differential diagnosis and management
		Nasal gliomas
		Cranial dermoid cysts and sinuses
			Cutaneous findings
			Extracutaneous findings
			Diagnosis
	Spinal dysraphism
		Cutaneous findings
		Hypertrichosis
		Lipomas
		Hemangiomas, telangiectasias, and capillary malformations
		Dimples, sinuses, aplasia cutis, and congenital scars
		Acrochordons, tails, and pseudotails
			Diagnosis
	Aplasia cutis
		Cutaneous findings
		Aplasia cutis of the trunk
			Pathogenesis
			Extracutaneous findings
			Diagnosis
			Differential diagnosis
			Prognosis and management
	Cutaneous dimples
	Adnexal polyp
	Developmental anomalies  of the umbilicus
		Anomalies of the urachus
		Anomalies of the  omphalomesenteric duct
		Umbilical granuloma
			Prognosis and management
	Amnion rupture malformation sequence/amniotic bands
		Cutaneous findings
		Extracutaneous findings
		Treatment
	Key references
	References
10 Vesicles, Pustules, Bullae, Erosions, and Ulcerations
	Introduction
	Bacterial infections (see Chapter 12)
		Staphylococcus aureus infections
		Staphylococcus aureus pyoderma
		Staphylococcal scalded skin syndrome
		Streptococcal infection
		Listeria infection
		Haemophilus influenzae infection
		Pseudomonas infection
		Congenital syphilis
	Fungal infections (see Chapter 14)
		Candida
			Congenital candidiasis and invasive candidal ‘dermatitis’
			Candidal infection in older infants
		Aspergillus infection
		Trichosporosis and zygomycosis
	Viral infections (see Chapter 13)
		Herpes simplex infection
			Infantile HSV infection
		Varicella
	Cutaneous infestations
		scabies
		Insect bite hypersensitivity and  wells syndrome
	Transient skin lesions
		Erythema toxicum neonatorum
		Transient neonatal pustular melanosis
		Sterile transient neonatal pustulosis
		Miliaria
		Sucking blisters
		Neonatal cephalic pustulosis  (neonatal ‘acne’)
		Diaper erosions
		Iatrogenic causes of erosions  and ulcerations
	Uncommon and rare causes of vesicles and pustules
		Infantile acropustulosis
		Eosinophilic pustular folliculitis
		Erosive pustular dermatosis  of the scalp
		Langerhans’ cell histiocytosis
		Incontinentia pigmenti
		Hyperimmunoglobulin E syndrome and DOCK8 deficiency
		Pustular eruption in myeloproliferative disorder of down syndrome
		Neonatal eosinophilic pustulosis
		Pustular psoriasis and deficiency of interleukin 1 receptor antagonist
		Acute generalized exanthematous pustulosis
		Neonatal Behçet disease
		Mastocytosis
		Blistering caused by maternal  bullous disease
		Neonatal lupus erythematosus
		Toxic epidermal necrolysis and bullous erythema multiforme
		Intrauterine epidermal necrosis
		Congenital erosive and  vesicular dermatosis
		Lipoid proteinosis
		Pyoderma gangrenosum
		Noma neonatorum
		Phytophotodermatitis
		Acrodermatitis enteropathica and acrodermatitis ‘dysmetabolica’
		Epidermolytic hyperkeratosis and superficial epidermolytic ichthyosis
		Restrictive dermopathy
		Ulcerations in vascular anomalies
		‘Bullae’, ulcerations, and absent skin overlying developmental anomalies
		Linear porokeratosis and porokeratotic adnexal ostial nevus
		Erosions and ulcerations overlying giant melanocytic nevi
		Ectodermal dysplasia syndromes
		Porphyria
		Perinatal gangrene of the buttock
		Purpura fulminans
	Key references
	References
11 Inherited and Acquired Blistering Diseases
	Inherited blistering diseases
		Epidermolysis bullosa
	Clinical features of epidermolysis bullosa
		Neonatal features
	Specific epidermolysis bullosa subtypes
		Suprabasal epidermolysis bullosa simplex
		Basal epidermolysis bullosa simplex
			Genetics and pathogenesis
		Junctional epidermolysis bullosa
			Genetics and pathogenesis
		Dystrophic epidermolysis bullosa
			Genetics and pathogenesis
		Kindler syndrome
			Differential diagnosis
	Evaluating suspected EB
	Management of EB in neonates  and infants
		Additional considerations
	Acquired blistering diseases
		Autoimmune bullous disorders
			Linear IgA disease
			Bullous pemphigoid
			Epidermolysis bullosa acquisita
			Bullous systemic lupus erythematosus
		Non-autoimmune bullous disorders
	Key references
	References
12 Bacterial Infections
	Introduction
	Superficial infections
		Impetigo
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Diagnosis
			Differential diagnosis
			Course, management, treatment, and prognosis
		Intertrigo
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Differential diagnosis
			Diagnosis
			Course, management, treatment, and prognosis
		Folliculitis/pustulosis/furunculosis
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Diagnosis
			Differential diagnosis
			Course, management, treatment, and prognosis
	Infection of pre-existing erosions and ulcerations
		Cutaneous findings
		Extracutaneous findings
		Etiology and pathogenesis
		Diagnosis
		Differential diagnosis
		Course, management, treatment, and prognosis
		Paronychia
		Funisitis/omphalitis
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Diagnosis
			Differential diagnosis
			Course, management, treatment, and prognosis
		Cellulitis
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Diagnosis
			Differential diagnosis
			Course, management, treatment, and prognosis
		Periorbital/orbital cellulitis
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Diagnosis
			Differential diagnosis
			Course, management, treatment, and prognosis
	Subcutaneous and systemic infections
		Abscesses
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Diagnosis
			Course, management, treatment, and prognosis
		Breast abscess
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Diagnosis
			Course, management, treatment, and prognosis
		Scalp abscess
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Diagnosis
			Differential diagnosis
			Course, management, treatment, and prognosis
		Necrotizing fasciitis
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Diagnosis
			Differential diagnosis
			Course, management, treatment, and prognosis
		Ecthyma
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Diagnosis/differential diagnosis
			Course, management, treatment, and prognosis
	Infections at the site of indwelling lines
		Cutaneous findings
		Extracutaneous findings
		Etiology and pathogenesis
		Diagnosis
		Course, management, treatment, and prognosis
	Purpura fulminans
		Cutaneous findings
		Extracutaneous findings
		Etiology/pathogenesis
		Diagnosis
		Differential diagnosis
		Course, management, treatment, and prognosis
	Toxin-mediated disease
		Staphylococcal scalded skin syndrome
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Diagnosis
			Differential diagnosis
			Course, management, treatment, and prognosis
		Toxic shock syndrome
			Etiology and pathogenesis
			Cutaneous findings
			Extracutaneous findings
			Course, management, treatment, and prognosis
	Other infectious agents
		Group B streptococcus
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Diagnosis
			Differential diagnosis
			Course, management, treatment, and prognosis
		Listeria monocytogenes
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Diagnosis
			Differential diagnosis
			Course, management, treatment, and prognosis
		Treponema pallidum
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Diagnosis
			Differential diagnosis
			Course, management, treatment, and prognosis
		Mycobacterium tuberculosis
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Diagnosis
			Course, management, treatment, and prognosis
	Key references
	References
13 Viral Infections
	Introduction
	Herpes simplex virus
		Epidemiology
		Intrauterine HSV infection
			Cutaneous findings
			Extracutaneous findings
		Neonatal (perinatal) herpes  simplex infection
			Cutaneous findings
			Extracutaneous findings
			Diagnosis
			Differential diagnosis
			Management and prognosis
		HSV in children beyond the neonatal period
		Eczema herpeticum
	Varicella
		Fetal varicella syndrome
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Diagnosis
			Treatment
		Neonatal varicella
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Diagnosis
			Treatment
			Special concerns for the neonatal nursery and intensive care unit
				Varicella exposure in the neonatal intensive care unit.
		Infantile herpes zoster
			Cutaneous findings
			Diagnosis
			Etiology and pathogenesis
			Treatment
		Infantile varicella (chickenpox)
	Cytomegalovirus
		Cutaneous findings
		Extracutaneous findings
		Etiology and pathogenesis
		Diagnosis
		Treatment
	Rubella
		Cutaneous findings
		Extracutaneous findings
		Etiology and pathogenesis
		Diagnosis
		Prevention and treatment
	Acquired immunodeficiency syndrome
		Cutaneous findings
			Infectious skin disorders.
			Neoplastic skin disease.
			Inflammatory skin disease.
		Extracutaneous manifestations
		Etiology and pathogenesis
		Diagnosis
		Treatment
	Human parvovirus B19 infections
		Fetal, congenital, and neonatal disease
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Diagnosis
			Differential diagnosis
			Treatment
				Management of the exposed pregnant woman.
				Management of fetal hydrops.
				Congenital and neonatal infection.
				Parvovirus infection in infants and toddlers.
				Prophylaxis.
	Enterovirus
		Cutaneous findings
		Extracutaneous findings
		Etiology and pathogenesis
		Diagnosis
		Treatment
		Infantile enteroviral disease
		Hand, foot, and mouth disease
	Human papilloma virus infections
		Cutaneous findings
		Etiology and pathogenesis
		Diagnosis
		Differential diagnosis
		Treatment
	Molluscum contagiosum
		Cutaneous findings
		Etiology and pathogenesis
		Diagnosis
		Differential diagnosis
		Treatment
	Viral exanthems in infants and toddlers
		Measles (rubeola)
			Clinical findings
			Differential diagnosis
			Diagnosis
			Treatment
		Rubella (german measles)
		Roseola (exanthem subitum,  sixth disease)
		Unilateral laterothoracic exanthem
		Gianotti–Crosti syndrome
	Key References
	References
14 Fungal Infections, Infestations, and Parasitic Infections  in Neonates and Infants
	Fungal infections
		Candidiasis
			Epidemiology and pathogenesis
			Congenital candidiasis
			Systemic candidiasis
			Invasive fungal dermatitis
			Localized candidiasis
				Oral candidiasis (thrush).
				Candida diaper dermatitis.
				Candida infections of the nail plate and related structures.
			Diagnosis
			Treatment
		Malassezia infections
			Skin colonization with Malassezia species
			Tinea versicolor
			Neonatal cephalic pustulosis
			Malassezia sepsis
			Diagnosis and treatment of Malassezia infections
		Trichosporonosis
		Aspergillosis
			Primary cutaneous aspergillosis
			Systemic aspergillosis
			Cutaneous zygomycosis (mucormycosis, phycomycosis)
		Dermatophytosis
			Clinical findings
			Diagnosis
			Treatment
	Ectoparasitic infestations
		Scabies
			Introduction
			Cutaneous findings
			Etiology and pathogenesis
			Diagnosis
			Treatment
		Pediculosis capitis (head lice)
			Introduction
			Cutaneous findings
			Etiology
			Diagnosis
			Treatment
		Enterobiasis (pinworms)
		Cutaneous larva migrans
		Demodicidosis
		Myiasis
	Parasitic infections
		Toxoplasmosis
		Leishmaniasis
	Arthropod bites and stings
		Papular urticaria
			Brief introduction
			Cutaneous findings
			Diagnosis
			Etiology
			Treatment
	Key references
	References
15 Eczematous Disorders
	Introduction
	Atopic dermatitis
		Risk factors
		Clinical findings
			History
			Physical examination
		Diagnosis
		Etiology and pathogenesis
		Differential diagnosis
		Prognosis
		Treatment
			Xerosis/dry skin
			Anti-inflammatory agents
				Topical corticosteroids.
				Topical calcineurin inhibitors.
				Maintenance therapy.
			Pruritus
			Environmental control
			Infections
			Addressing diet and potential allergens
			Adjunctive and alternative therapies
		Primary prevention
	Seborrheic dermatitis
		Cutaneous findings
		Etiology and pathogenesis
		Diagnosis
		Differential diagnosis
		Course, management, treatment,  and prognosis
	Contact dermatitis
		Irritant contact dermatitis
			Pathogenesis
			Diagnosis and differential diagnosis
			Management
		Allergic contact dermatitis
			Pathogenesis
			Diagnosis and differential diagnosis
			Management
	Keratosis pilaris
	Other etiologies of eczematous eruptions (see Boxes 15.2, 15.3)
		ZINC DEFICIENCY
		BIOTIN DEFICIENCY
		CYSTIC FIBROSIS
		HARTNUP DISEASE
		PHENYLKETONURIA
		NETHERTON SYNDROME
		ICHTHYOSIS LINEARIS CIRCUMFLEXA
		OMENN SYNDROME
		WISKOTT-ALDRICH SYNDROME
		HYPER IgE SYNDROME
	Conclusion
	Key references
	References
16 Papulosquamous and Lichenoid Disorders
	Papulosquamous disorders
		Psoriasis
			Introduction
			Cutaneous findings
				Diaper psoriasis.
				Erythrodermic psoriasis.
				Pustular psoriasis.
				Congenital psoriasis.
			Extracutaneous findings of infantile psoriasis
			Differential diagnosis
			Etiology and pathogenesis
			Treatment and prognosis
		Pityriasis rubra pilaris
			Introduction
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Differential diagnosis
			Prognosis
			Treatment
		Pityriasis rosea
			Introduction
			Cutaneous findings
			Etiology and pathogenesis
			Differential diagnosis, prognosis, and treatment
	Lichenoid disorders
		Pityriasis lichenoides
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Differential diagnosis
			Prognosis and treatment
		Lichen planus
			Cutaneous findings
			Etiology and pathogenesis
			Differential diagnosis, prognosis, and treatment
		Lichen striatus
			Cutaneous findings
			Etiology and pathogenesis
			Differential diagnosis
			Prognosis and treatment
		Lichen nitidus
			Cutaneous findings
			Etiology and pathogenesis
			Differential diagnosis, prognosis, and treatment
		Keratosis lichenoides chronica
			Cutaneous findings
			Etiology and pathogenesis
			Differential diagnosis, prognosis, and treatment
	Key references
	References
17 Diaper Area Eruptions
	Introduction
	Care of the diaper area in the newborn
	Diaper-related eruptions
		Irritant diaper dermatitis
			Cutaneous findings
			Etiology and pathogenesis
			Differential diagnosis
			Treatment and care
		Erosive perianal eruption
		Pseudoverrucous papules
		Granuloma gluteale infantum
		Senna laxative-induced blistering dermatitis in toddlers
		Granular parakeratosis
		Infantile seborrheic dermatitis (ISD)
			Cutaneous features
			Differential diagnosis
			Etiology and pathogenesis
			Prognosis and treatment
		Psoriasis
			Cutaneous findings
			Extracutaneous findings
			Differential diagnosis
			Etiology and pathogenesis
			Prognosis and treatment
		Candidal diaper dermatitis with psoriasiform ID
			Cutaneous findings
			Etiology and pathogenesis
			Prognosis and treatment
		Allergic contact dermatitis
	Infections
		Candidiasis
			Clinical picture
			Differential diagnosis
			Etiology and pathogenesis
			Prognosis and treatment
		Staphylococcal infection: impetigo/staphylococcal scalded skin  syndrome (SSSS)
			Cutaneous findings
			Etiology and pathogenesis
			Differential diagnosis
			Prognosis and treatment
		Perianal and perineal  bacterial dermatitis
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Diagnosis and differential diagnosis
			Treatment and care
		Ecthyma gangrenosum
		Diaper dermatophytosis
		Herpes simplex infection
		Condyloma acuminata and  mollusca contagiosa
		Coxsackie viral exanthem (hand, foot, and ‘butt’ exanthem)
	Nutritional and metabolic disorders
		Zinc deficiency
			Clinical presentation
			Extracutaneous manifestations
			Etiology and pathogenesis
			Prognosis and treatment
		Disorders that resemble  acrodermatitis enteropathica
	Miscellaneous
		Langerhans’ cell histiocytosis
			Clinical presentation
			Extracutaneous findings
			Etiology and pathogenesis
			Differential diagnosis
			Prognosis and treatment
		Infantile perianal pyramidal protrusion
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Differential diagnosis
			Prognosis and treatment
		Nascent hemangioma
		Lichen sclerosus (LS)
		Pyoderma gangrenosum
		Chronic bullous disease of  childhood (CBDC)
		Bullous pemphigoid (BP)
		Kawasaki syndrome
		Clear-cell papulosis
	Key References
	References
18 Erythrodermas, Immunodeficiency, and Metabolic Disorders
	Erythrodermas
	Inflammatory diseases
		Atopic dermatitis
		Seborrheic dermatitis
		Psoriasis
		Pityriasis rubra pilaris
		Drug exanthem
		Acute generalized  exanthematous pustulosis
		Boric acid poisoning
		Diffuse cutaneous mastocytosis
	Infectious diseases
		Staphylococcal scalded skin syndrome
		Candidiasis
		Herpes simplex
		Syphilis
	Genodermatoses
		Ichthyosis
	Immunologic diseases
		Erythroderma and failure to thrive
		Severe combined immunodeficiency
		Omenn syndrome
		Digeorge anomaly
		Graft-versus-host disease
		Wiskott–aldrich syndrome
		Hyperimmunoglobulin E syndrome
		Chronic granulomatous disease
		Eosinophilic gastroenteritis
	Metabolic disorders
	Organic acid disorders
		Methylmalonic acidemia
	Fatty acid disorders
		Essential fatty acid deficiency
	Amino acid disorders
		Maple syrup urine disease and other inborn errors of metabolism
		Cystic fibrosis
	Testing for metabolic diseases
	Evaluation and management of the red scaly baby
	Key References
	References
19 Disorders of Cornification (Ichthyosis)
	Introduction
	Collodion baby
		Cutaneous features
		Extracutaneous features
		Etiology and pathogenesis
		Differential diagnosis
		Treatment and care
	Ichthyosis vulgaris
		Cutaneous features
		Extracutaneous features
		Etiology and pathogenesis
		Differential diagnosis
		Treatment and care
	Recessive X-linked ichthyosis
		Cutaneous features
		Extracutaneous features
		Etiology and pathogenesis
		Differential diagnosis
		Treatment and care
	Inherited syndromic ichthyoses –  X-linked
		Ichthyosis follicularis, alopecia, and photophobia (IFAP syndrome)
			Cutaneous features
			Extracutaneous features
			Etiology and pathogenesis
			Differential diagnosis
			Treatment and care
		Conradi–hünermann–happle syndrome (X-linked chondrodysplasia punctata)
			Cutaneous features
			Extracutaneous features
			Etiology and pathogenesis
			Differential diagnosis
			Treatment and care
		CHILD syndrome
			Cutaneous features
			Extracutaneous features
			Etiology and pathogenesis
			Differential diagnosis
			Treatment and care
	Inherited syndromic ichthyoses with prominent hair signs
		Netherton syndrome
			Cutaneous features
			Extracutaneous features
			Etiology and pathogenesis
			Differential diagnosis
			Treatment and care
		Ichthyosis hypotrichosis sclerosing cholangitis syndrome
			Cutaneous features
			Extracutaneous features
			Etiology and pathogenesis
			Differential diagnosis
			Treatment and care
		TRICHOTHIODYSTROPHY (IBIDS syndrome (TAY SYNDROME), PIBIDS syndrome, SIBIDS syndrome)
			Cutaneous features
			Extracutaneous features
			Etiology and pathogenesis
			Differential diagnosis
			Treatment and care
	Other inherited syndromic ichthyoses
		Sjögren–larsson syndrome
			Cutaneous features
			Extracutaneous features
			Etiology and pathogenesis
			Differential diagnosis
			Treatment and care
		Gaucher disease
			Cutaneous features
			Extracutaneous features
			Etiology and pathogenesis
			Treatment and care
		Keratitis, ichthyosis, deafness (KID) syndrome
			Cutaneous features
			Extracutaneous features
			Etiology and pathogenesis
			Differential diagnosis
			Treatment and care
		NEUTRAL LIPID STORAGE DISEASE WITH ICHTHYOSIS (Chanarin–dorfman syndrome)
			Cutaneous features
			Extracutaneous features
			Etiology and pathogenesis
			Differential diagnosis
			Treatment and care
		Ichthyosis prematurity syndrome
			Cutaneous features
			Extracutaneous features
			Etiology and pathogenesis
			Differential diagnosis
			Treatment and care
		CHIME syndrome: colobomas of the eye, heart defects, ichthyosis, mental retardation, ear defects
			Cutaneous features
			Extracutaneous features
			Etiology and pathogenesis
			Differential diagnosis
			Treatment and care
		MEDNIK syndrome: mental retardation-enteropathy-deafness-neuropathy-ichthyosis-keratoderma
	Inherited ichthyoses – non-syndromic
		Autosomal recessive congenital ichthyosis (ARCI)
		Harlequin ichthyosis
			Cutaneous features
			Extracutaneous features
			Differential diagnosis
			Treatment and care
		Lamellar ichthyosis phenotype of ARCI
			Cutaneous features
			Extracutaneous features
			Differential diagnosis
			Treatment and care
		Congenital ichthyosiform erythroderma phenotype of ARCI
			Cutaneous features
			Extracutaneous features
			Differential diagnosis
			Treatment and care
		Etiology and pathogenesis of  the ARCI group
	Keratinopathic ichthyosis
		Epidermolytic ichthyosis (EI)
			Cutaneous features
			Extracutaneous features
			Etiology and pathogenesis
			Differential diagnosis
			Treatment and care
		Ichthyosis curth–macklin
			Cutaneous features
			Extracutaneous features
			Etiology and pathogenesis
			Differential diagnosis
			Treatment and care
	Miscellaneous forms of ichthyosis
		Peeling skin disease
			Cutaneous features
			Extracutaneous features
			Etiology and pathogenesis
			Differential diagnosis
			Treatment and care
		Erythrokeratoderma variabilis (EKV)
			Cutaneous features
			Extracutaneous features
			Etiology and pathogenesis
			Differential diagnosis
			Treatment and care
		Clouston syndrome: hidrotic ectodermal dysplasia
			Cutaneous features
			Extracutaneous features
			Etiology and pathogenesis
			Differential diagnosis
			Treatment and care
		Erythrokeratoderma progressiva symmetrica (EPS)
			Cutaneous features
			Extracutaneous features
			Etiology and pathogenesis
			Differential diagnosis
			Treatment and care
	Palmoplantar keratodermas
		Olmsted syndrome
			Cutaneous features
			Extracutaneous features
			Etiology and pathogenesis
			Differential diagnosis
			Treatment and care
		Tyrosinemia II
			Cutaneous features
			Extracutaneous features
			Etiology and pathogenesis
			Differential diagnosis
			Treatment and care
		Keratosis linearis with ichthyosis congenita and sclerosing keratoderma: KLICK syndrome
			Cutaneous features
			Extracutaneous features
			Etiology and pathogenesis
			Differential diagnosis
			Treatment and care
	Restrictive dermopathy
		Cutaneous features
		Extracutaneous features
		Etiology and pathogenesis
		Differential diagnosis
		Treatment and care
	Neu–laxova syndrome
		Cutaneous features
		Extracutaneous features
		Etiology and pathogenesis
		Differential diagnosis
		Treatment and care
	Neonatal scaling of hypohidrotic ectodermal dysplasia
	Key references
	References
20 Immunologic, Reactive, and Purpuric Disorders
	Introduction
	Annular erythemas
		Annular erythema of infancy
		Erythema annulare centrifugum
		Familial annular erythema
			Differential diagnosis of annular erythemas
	Neonatal lupus erythematosus
		Cutaneous findings
		Extracutaneous findings
		Etiology and pathogenesis
		Laboratory tests and histopathology
		Differential diagnosis
		Course, management, treatment, and prognosis
	Other collagen vascular disorders of the newborn and young infant
	Drug eruptions
		Acute generalized exanthematous pustulosis
		Drug-induced hypersensitivity syndrome
	Stevens–Johnson syndrome and toxic epidermal necrolysis
		Cutaneous findings
		Extracutaneous findings
		Etiology and pathogenesis
		Histopathology
		Differential diagnosis
		Treatment
	Erythema multiforme
		Cutaneous findings
		Extracutaneous findings
		Etiology and pathogenesis
		Laboratory tests and histopathology
		Differential diagnosis
		Course, management, treatment, and prognosis
	Urticaria and urticarial eruptions
		Acute and chronic urticaria
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Diagnosis
			Differential diagnosis
			Course, management, treatment, and prognosis
		Physical urticarias
		Urticarial eruptions associated with autoimmune disorders or autoinflammatory diseases
		Isolated angioedema
	Autoinflammatory syndromes
		Cryopyrin-associated periodic syndromes (CAPS)
		Familial cold urticaria
		Chronic infantile neurological cutaneous and articular syndrome (CINCA)
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Laboratory tests, radiologic findings, and histopathology
			Differential diagnosis
			Course, management, treatment, and prognosis
		Pustular autoinflammatory syndromes in newborns
		Immunoproteasome-related syndromes
	Eosinophilic cellulitis
	Sweet syndrome
		Cutaneous findings
		Extracutaneous findings
		Etiology and pathogenesis
		Laboratory tests and histopathology
		Differential diagnosis
		Course, management, treatment, and prognosis
	Kawasaki disease
		Cutaneous findings
		Extracutaneous findings
		Etiology and pathogenesis
		Laboratory tests and histopathology
		Diagnosis
		Differential diagnosis
		Course, management, treatment, and prognosis
	Acute hemorrhagic edema
		Cutaneous findings
		Extracutaneous findings
		Etiology and pathogenesis
		Laboratory tests and histopathology
		Differential diagnosis
		Course, management, and prognosis
	Henoch–Schönlein purpura
		Cutaneous findings
		Extracutaneous findings
		Etiology and pathogenesis
		Laboratory findings and histopathology
		Differential diagnosis
		Treatment and prognosis
	Photosensitivity disorders
		Immunologically mediated photosensitivity disorders
		Photosensitivity disorders induced by exogenous substances
		Photoaggravated dermatoses
		Genodermatoses with photosensitivity
	The porphyrias
		Congenital erythropoietic porphyria
			Cutaneous and extracutaneous findings
			Laboratory tests and histopathology
			Differential diagnosis
			Management
		Erythropoietic protoporphyria
			Clinical manifestations
			Diagnosis
			Management
		Hepatoerythropoietic porphyria
		Other porphyrias
			Homozygous porphyrias
			Transient porphyrinemias
			Xeroderma pigmentosum
			Cockayne syndrome
			Trichothiodystrophy
			Ultraviolet-sensitive syndrome
			Rothmund–Thomson syndrome
			Bloom syndrome
			Kindler syndrome
			Smith–Lemli–Opitz syndrome
			Hartnup disease
	Purpura and petechiae in infants  and newborns
		Purpura in neonates
		Dermal erythropoiesis (blueberry muffin baby)
			Cutaneous and extracutaneous findings
			Etiology and pathogenesis
			Laboratory tests and histopathology
			Differential diagnosis
			Course, management, treatment, and prognosis
		Purpura fulminans in newborns and young infants
		Congenital proteins C and S deficiencies
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Laboratory tests and histopathology
			Differential diagnosis
			Course, management, treatment, and prognosis
		Purpuric phototherapy-induced eruption
		Pigmented purpuric eruptions (PPE)
		Petechiae and petechial eruptions
	Key references
	References
21 Infantile Hemangiomas and Other Vascular Tumors
	Infantile hemangioma
		Clinical subtypes
		Natural history
			High-risk hemangiomas.
			Locations associated with high risk for disfigurement.
			Periorbital hemangiomas.
			Large cervicofacial hemangiomas and PHACE syndrome.
			Lumbosacral hemangiomas and regional anomalies.
			Multifocal cutaneous and visceral hemangiomas.
			Cutaneous hemangiomas associated with airway hemangiomas.
		Pathogenesis
		Diagnosis
		Histopathology
		Differential diagnosis
		Management
			Active non-intervention.
			Local and topical treatment.
			Intralesional corticosteroids.
			Pulsed dye laser.
			Systemic therapy.
			Corticosteroid therapy.
			Other therapies.
			Surgical excision.
			Treatment of ulceration.
	Other vascular tumors
		Congenital hemangiomas
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Differential diagnosis
			Management
		Tufted angioma and kaposiform hemangioendothelioma
		Kasabach–merritt phenomenon
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Course
			Diagnosis
			Differential diagnosis
			Management
		Multifocal lymphangioendotheliomatosis with thrombocytopenia
		Infantile hemangiopericytoma
		Spindle-cell hemangioendothelioma
		Congenital eccrine angiomatous hamartoma
		Pyogenic granuloma (lobular capillary hemangioma)
	Key References
	References
22 Vascular Malformations
	Introduction to vascular malformations
	Capillary malformations
		Nevus simplex (salmon patch, fading capillary stain)
		Capillary malformations
			Pathogenesis
			Cutaneous findings
			Extracutaneous findings
			Management
		Telangiectasia
	Venous malformations
		Pathogenesis
		Cutaneous findings
		Extracutaneous findings
		Diagnosis
		Differential diagnosis
		Course
		Management
	Lymphatic malformations
		Pathogenesis
		Cutaneous findings
		Extracutaneous findings
		Diagnosis
		Treatment
	Arteriovenous malformations
		Pathogenesis
		Cutaneous findings
		Extracutaneous findings
		Diagnosis
		Management
	Combined malformations
	Syndromes associated with vascular malformations
		Syndromes associated with capillary malformations
			Sturge–Weber syndrome
			Macrocephaly capillary malformation syndrome (megalencephaly capillary malformation polymicrogyria syndrome)
			Beckwith–Wiedemann syndrome
			Nova syndrome
			Phakomatosis pigmentovascularis
		Syndromes associated with venous malformations
			Venous malformations cutaneous and mucosal (VMCM), glomuvenous malformations and blue rubber bleb nevus syndrome (Bean syndrome)
			Maffucci syndrome
			Cutis marmorata telangiectatica congenita
			Verrucous hemangioma and angiokeratoma circumscriptum
		Syndromes associated with arteriovenous malformations
			Capillary malformation-arteriovenous malformation syndrome
			Wyburn–Mason, Bonnet–Dechaume–Blanc, and Brégeat syndromes
			Cobb syndrome
			Hereditary hemorrhagic telangiectasia
			Ataxia–telangiectasia
		Syndromes associated with lymphatic malformations
			Gorham syndrome
			Hennekam syndrome
			Hereditary cholestasis with lymphedema (Aagenaes syndrome)
		Syndromes associated with either combined malformations or overgrowth
			Klippel–Trenaunay syndrome
			Parkes Weber syndrome
				Diagnosis.
				Management.
			CLOVES syndrome
			Proteus syndrome
			PTEN hamartoma tumor syndrome
	Key References
	References
23 Hypopigmentation Disorders
	Introduction
	Generalized hypopigmentation of skin, hair, and eyes
		Oculocutaneous albinism
		Oculocutaneous albinism type 1
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
		Oculocutaneous albinism type 2
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
		Oculocutaneous albinism type 3
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
		Oculocutaneous albinism type 4
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Differential diagnosis
			Treatment and care
		Hermansky–pudlak syndrome
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Differential diagnosis
			Treatment and care
		Chediak–higashi syndrome
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Treatment and care
		Cross syndrome
			Cutaneous findings
			Extracutaneous findings
			Treatment and care
		Phenylketonuria (phenylalanine hydroxylase deficiency)
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Treatment and care
	Generalized hypopigmentation involving skin and hair
		Griscelli syndrome
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Differential diagnosis
			Treatment and care
		Elejalde syndrome
			Cutaneous findings
			Extracutaneous findings
			Differential diagnosis
			Treatment and care
		Menkes disease (and occipital  horn syndrome)
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Treatment and care
	Mosaic hypopigmentation
		Nevoid hypopigmentation
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Differential diagnosis
			Treatment and care
		Nevus depigmentosus
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Differential diagnosis
			Treatment and care
	Localized hypopigmented disorders
		Piebaldism
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Differential diagnosis
			Treatment and care
		Waardenburg syndrome
			Cutaneous and extracutaneous findings
			Etiology and pathogenesis
			Differential diagnosis
			Treatment and care
		Tuberous sclerosis complex
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Differential diagnosis
			Treatment and care
		Nevus anemicus
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Differential diagnosis
		Vitiligo
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Differential diagnosis
			Treatment and care
		Post-inflammatory hypopigmentation
			Cutaneous findings
			Etiology and pathogenesis
			Differential diagnosis
			Treatment and care
		Congenital halo nevi
			Cutaneous findings
			Etiology and pathogenesis
			Treatment and care
	Miscellaneous hypopigmentation
		Alezzandrini syndrome
			Cutaneous and extracutaneous findings
			Etiology and pathogenesis
			Differential diagnosis
			Treatment and care
		Ziprkowski–margolis syndrome
			Cutaneous and extracutaneous findings
			Etiology and pathogenesis
			Treatment and care
	Key References
	References
24 Disorders of Hyperpigmentation and Melanocytes
	Introduction
	Localized hyperpigmentation – tan-brown
		Café-au-lait macules
			Segmental pigmentation disorder (mosaic hyperpigmentation)
		Selected disorders associated with café-au-lait macules
			Neurofibromatosis type 1 (NF-1)
			Legius syndrome
			Familial (inherited) café-au-lait macules
			McCune–Albright syndrome
		Disorders of dermal melanocytosis
			Congenital dermal melanocytosis (Mongolian spots)
				Treatment and care.
				Differential diagnosis.
			Nevus of Ota (nevus fuscoceruleus ophthalmomaxillaris, oculodermal melanocytosis)
				Treatment and care.
				Differential diagnosis.
			Nevus of Ito (nevus fuscoceruleus acromiodeltoideus)
			Blue nevi
				Treatment and care.
		Mosaic conditions and patterned dyspigmentation (whorled and segmental hyperpigmentation)
			Mosaic conditions
			Chimerism
			Linear and whorled nevoid hypermelanosis
				Treatment and care.
			Incontinentia pigmenti
			Phakomatosis pigmentovascularis
			Phakomatosis pigmentokeratotica
			Naegeli–Franceschetti–Jadassohn syndrome
			Striated hyperpigmentation of the torso
			Congenital curvilinear palpable hyperpigmentation (sock-line bands, sock-line hyperpigmentation)
	Spotty pigmentation – diffuse
		Xeroderma pigmentosum
			Treatment and care.
			Post-inflammatory hyperpigmentation
			Universal melanosis and progressive familial hyperpigmentation
			Poikiloderma
		Metabolic causes
			Addison disease and adrenocortical-unresponsiveness syndrome
		Lentigines
			LEOPARD syndrome (multiple lentigines syndrome, Moynahan syndrome)
				Treatment and care.
			Carney complex (NAME/LAMB syndrome)
	Spotty pigmentation – localized
		Centrofacial lentiginosis
		Segmental lentiginosis (partial unilateral lentiginosis, lentiginous mosaicism)
		Peutz–Jeghers syndrome
		Bannayan–Riley–Ruvalcaba syndrome
		Pigmentary variations
			Dyschromatosis
			Pigmentary demarcation lines
		Congenital melanocytic nevi
			Small and medium congenital melanocytic nevi
			Large congenital melanocytic nevi
		Management and treatment of CMN
			Small and medium CMN
			Large CMN
		Neurocutaneous melanoCYTOsis
			Differential diagnosis
		Congenital melanoma
		Speckled lentiginous nevus and other subtypes of CMN
			Treatment and care
		Spitz nevi (spindle and epithelioid nevi)
			Treatment and care
	Key References
	References
25 Acneiform and Sweat Gland Disorders
	Acneiform disorders
		Neonatal acne (neonatal  cephalic pustulosis)
		Infantile acne vulgaris
		Drug-induced acne
		Acne associated with apert syndrome
		Hyper-IgE syndrome
		Pseudoacne of the nasal crease
		Childhood flexural comedones
		Idiopathic facial aseptic granuloma
		Childhood rosacea
		Periorificial dermatitis
		Demodicosis
		Granulosis rubra nasi
		Keratosis pilaris
			Keratosis pilaris variants
		Disseminated congenital comedones and idiopathic disseminated comedones
		Trichodysplasia spinulosa
	Eccrine gland disorders
		Eccrine hidradenitis
			Childhood neutrophilic eccrine hidradenitis
			Palmoplantar eccrine hidradenitis
				Chemotherapy.
	Hyperhidrosis
		Auriculotemporal nerve syndrome  (frey syndrome)
		Harlequin syndrome
	Hypohidrosis and anhidrosis
		Congenital insensitivity to pain  with anhidrosis
	Key References
	References
26 Lumps, Bumps, and Hamartomas
	Lumps and bumps
		Fibromatoses
			Infantile myofibromatosis
				Cutaneous findings.
				Extracutaneous findings.
				Etiology and pathogenesis.
				Diagnosis.
				Differential diagnosis.
				Treatment and prognosis.
			Infantile desmoid-type or aggressive fibromatosis
				Clinical findings.
				Etiology and pathogenesis.
				Diagnosis.
				Differential diagnosis.
				Treatment and prognosis.
			Fibromatosis colli
				Clinical findings.
				Etiology and pathogenesis.
				Diagnosis.
				Differential diagnosis.
				Treatment and prognosis.
			Infantile digital fibromatosis
				Cutaneous findings.
				Extracutaneous findings.
				Etiology and pathogenesis.
				Diagnosis.
				Differential diagnosis.
				Treatment and prognosis.
			Fibrous hamartoma of infancy
				Cutaneous findings.
				Extracutaneous findings.
				Etiology and pathogenesis.
				Diagnosis.
				Differential diagnosis.
				Treatment and prognosis.
			Lipofibromatosis
			Gingival fibromatosis
				Cutaneous findings.
				Extracutaneous findings.
				Etiology and pathogenesis.
				Diagnosis.
				Differential diagnosis.
				Treatment and prognosis.
		Adult-type fibromatoses
			Leiomyoma
				Cutaneous findings.
				Extracutaneous findings.
				Etiology and pathogenesis.
				Diagnosis.
				Differential diagnosis.
				Treatment and prognosis.
		Neurofibromas and other  neural tumors
		Non-langerhans’ cell histiocytoses
			Juvenile xanthogranuloma
				Cutaneous findings.
				Extracutaneous findings.
				Etiology.
				Diagnosis.
				Differential diagnosis.
				Treatment and prognosis.
			Benign cephalic histiocytosis
				Cutaneous findings.
				Extracutaneous findings.
				Diagnosis.
				Differential diagnosis.
				Treatment and prognosis.
	Calcifying disorders of the skin
		Dystrophic calcification
		Metastatic calcification
		Idiopathic calcification
			Calcified ear nodule
				Cutaneous findings.
				Extracutaneous findings.
				Etiology and pathogenesis.
				Diagnosis.
				Differential diagnosis.
				Treatment and prognosis.
			Tumoral calcinosis
				Cutaneous findings.
				Extracutaneous findings.
				Etiology and pathogenesis.
				Diagnosis.
				Treatment.
		Iatrogenic calcification
		Osteoma cutis
			Pseudohypoparathyroidism type 1 and Albright hereditary osteodystrophy
				Cutaneous findings.
				Extracutaneous findings.
				Etiology and pathogenesis.
				Diagnosis.
				Differential diagnosis.
				Treatment and prognosis.
			Progressive osseous heteroplasia
			Congenital plate-like osteoma cutis
			Pilomatricoma
				Cutaneous findings.
				Extracutaneous findings.
				Etiology and pathogenesis.
				Diagnosis.
				Differential diagnosis.
				Treatment and prognosis.
	Granuloma annulare
	Hamartomas
		Epidermal nevus
		Nevus sebaceus
			Cutaneous findings.
			Extracutaneous findings.
			Etiology and pathogenesis.
			Diagnosis.
			Differential diagnosis.
			Treatment and prognosis.
		Syringocystadenoma papilliferum
		Keratinocytic epidermal nevus
			Cutaneous findings.
			Extracutaneous findings.
			Diagnosis.
			Etiology and pathogenesis.
			Differential diagnosis.
			Treatment and prognosis.
		Nevus comedonicus
			Cutaneous findings.
			Extracutaneous findings.
			Etiology and pathogenesis.
			Diagnosis.
			Differential diagnosis.
			Treatment and prognosis.
		Porokeratotic eccrine nevus
			Cutaneous and extracutaneous findings.
			Etiology and pathogenesis.
			Diagnosis.
			Differential diagnosis.
			Treatment and prognosis.
		Congenital smooth muscle hamartoma
			Cutaneous findings.
			Extracutaneous findings.
			Etiology and pathogenesis.
			Diagnosis.
			Differential diagnosis.
			Prognosis.
		Congenital becker nevus and becker nevus syndrome
		Michelin tire baby
		Nevus lipomatosus
		Connective tissue nevus
			Cutaneous findings.
			Extracutaneous findings.
			Etiology and pathogenesis.
			Diagnosis.
			Differential diagnosis.
			Treatment and prognosis.
		Plaque-like myofibroblastic tumor
	Acquired raised bands of infancy
	Key References
	References
27 Disorders of the Subcutaneous Tissue
	Introduction
	Subcutaneous fat necrosis  of the newborn
		Cutaneous findings
		Etiology and pathogenesis
		Diagnosis
		Differential diagnosis
		Management
	Sclerema neonatorum
		Cutaneous findings
		Extracutaneous findings
		Etiology and pathogenesis
		Diagnosis
		Differential diagnosis
		Treatment
	Stiff skin syndrome
		Cutaneous findings
		Extracutaneous findings
		Etiology and pathogenesis
		Diagnosis
		Differential diagnosis
		Treatment
	Panniculitis caused by  physical agents
		Cold panniculitis
			Cutaneous findings
			Etiology and pathogenesis
			Diagnosis
			Differential diagnosis
			Treatment and course
	Mechanical trauma
		Cutaneous findings
		Diagnosis
		Treatment and course
	Injection-site granuloma
		Cutaneous findings
		Diagnosis
		Differential diagnosis
		Treatment and course
	Infectious panniculitis
		Cutaneous findings
		Extracutaneous findings
		Etiology and pathogenesis
		Diagnosis
		Differential diagnosis
		Treatment and course
	Tumors of fat
		Lipoma
			Cutaneous findings
			Extracutaneous findings
			Diagnosis
			Treatment, course, and management
		Nevus lipomatosus cutaneous superficialis
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Diagnosis
			Differential diagnosis
			Treatment, course, and management
		Encephalocraniocutaneous lipomatosis
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Diagnosis
			Differential diagnosis
			Treatment and course
		Congenital diffuse lipomatosis
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Diagnosis
			Differential diagnosis
			Treatment, course, and management
		Congenital pedal papules
			Cutaneous findings
			Etiology and pathogenesis
			Diagnosis and differential diagnosis
			Treatment and course
		Lipoblastoma and lipoblastomatosis
			Cutaneous findings
			Etiology and pathogenesis
			Diagnosis
			Differential diagnosis
			Treatment and course
	Lipodystrophies
		Leprechaunism
			Cutaneous findings
			Etiology and pathogenesis
			Diagnosis
			Differential diagnosis
			Treatment and course
		Congenital generalized lipodystrophy (seip–berardinelli syndrome)
			Cutaneous findings
			Extracutaneous features
			Etiology and pathogenesis
			Diagnosis
			Differential diagnosis
			Treatment and course
		Congenital disorders of  glycosylation (CDG)
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Diagnosis
			Differential diagnosis
			Treatment and course
		Farber lipogranulomatosis
			Clinical findings
			Etiology and pathogenesis
			Diagnosis
			Differential diagnosis
			Treatment and course
	Key References
	References
28 Neoplastic and Infiltrative Diseases
	Introduction
	Neoplastic disorders
		Leukemia
			Cutaneous findings
			Diagnosis
			Treatment and course
		Leukemia in infancy
		Langerhans’ cell histiocytosis
			Cutaneous findings
		Congenital ‘self-healing’ langerhans’ cell histiocytosis
			Extracutaneous findings
			Diagnosis
			Differential diagnosis
			Treatment and course
		Lymphoma
			Mycosis fungoides
			Primary cutaneous anaplastic large cell lymphoma
			Lymphomatoid papulosis
			Cutaneous B-cell lymphoma
		Hemophagocytic lymphohistiocytosis
		Infantile fibrosarcoma
			Cutaneous findings
			Diagnosis
			Differential diagnosis
			Treatment and course
		Dermatofibrosarcoma protuberans
			Cutaneous findings
			Diagnosis
			Differential diagnosis
			Treatment
		Neuroblastoma
			Cutaneous findings
			Extracutaneous findings
			Diagnosis
			Differential diagnosis
			Treatment and course
		Rhabdomyosarcoma
		Rhabdoid tumor
		Malignant melanoma
		Congenital teratoma
	Infiltrative disorders
		Mastocytosis
			Cutaneous findings
		Solitary mastocytoma
		Maculopapular cutaneous mastocytosis (MPCM)/urticaria pigmentosa
		Diffuse cutaneous mastocytosis
		Telangiectasia macularis eruptiva perstans (TMEP)
			Extracutaneous findings in mast cell disease
			Etiology and pathogenesis
			Diagnosis
			Differential diagnosis
			Treatment and care
		Infantile systemic hyalinosis and juvenile hyaline fibromatosis
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Differential diagnosis
			Treatment and course
		Farber disease
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Diagnosis
			Differential diagnosis
			Treatment and course
		Mucopolysaccharidoses
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Diagnosis
			Differential diagnosis
			Treatment, course, and management
		I-cell disease
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Diagnosis
			Differential diagnosis
			Treatment and course
		Lipoid proteinosis
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Diagnosis and differential diagnosis
			Treatment, course, and management
		Cutaneous mucinosis of infancy
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Diagnosis and differential diagnosis
			Treatment, course, and management
	Key References
	References
29 Selected Hereditary Diseases
	Approach to the child with a genetic skin condition
	Disorders of the RAS-MAPK  pathway (RASopathies)
		Neurofibromatosis type 1
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Differential diagnosis and diagnosis
			Treatment and care
		Leguis syndrome
		Noonan syndrome
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Differential diagnosis
			Treatment and care
		Cardiofaciocutaneous syndrome
		Costello syndrome
		Capillary malformation-arteriovenous malformation (CM-AVM) (MIM #608354)
		Neurofibromatosis type 2
	Disorders of the PI3K-AKT/mTOR pathway and overgrowth syndromes
		Tuberous sclerosis
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Differential diagnosis
			Treatment and care
		Proteus syndrome
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Differential diagnosis
				Hemihyperplasia-multiple lipomatosis (HH-ML).
				Megalencephaly-capillary malformation (MCAP).
				Mosaic overgrowth with fibroadipose hyperplasia (MOFH).
				SOLAMEN syndrome.
				CLOVES syndrome (MIM #612918).
				Encephalocraniocutaneous lipomatosis (ECCL).
			Differential diagnosis of Proteus and  similar disorders
			Management
	Additional overgrowth syndromes
		Simpson–golabi–behmel syndrome type I
		Sotos syndrome
	Ectodermal dysplasias
		Hypohidrotic ectodermal dysplasia
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Differential diagnosis
			Treatment and care
		Hypohidrotic ectodermal dysplasia with immunodeficiency
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis/management
		Incontinentia pigmenti
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Differential diagnosis
			Treatment and care
		Hidrotic ectodermal dysplasia
		Ectodermal dysplasias due to  p63 mutations
		Ankyloblepharon–ectodermal dysplasia–clefting (AEC) syndrome
			Cutaneous findings
			Extracutaneous findings
			Differential diagnosis
			Treatment and care
		Ectrodactyly–ectodermal dysplasia–clefting syndrome (EEC)
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Differential diagnosis
			Treatment and care
	Disorders with skin laxity and redundant skin
		Cutis laxa
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Differential diagnosis
			Treatment and care
		De barsy syndrome
		Ehlers–danlos syndromes
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Differential diagnosis
			Treatment and care
	Mosaic disorders
		Focal dermal hypoplasia of goltz
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Differential diagnosis
			Treatment and care
		Epidermal nevus syndrome
		MIDAS syndrome
	Chromosomal disorders
		Turner syndrome
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Differential diagnosis
			Treatment and care
		Trisomy 21
		Trisomy 18
		Trisomy 13
	Miscellaneous disorders
		Cerebello-trigeminal dermal dysplasia
		Familial dysautonomia
			Cutaneous findings
			Extracutaneous findings
			Etiology and pathogenesis
			Differential diagnosis
			Treatment and care
		Coffin–siris syndrome
	Mitochondrial disorders
	Key References
	References
30 Neonatal Mucous Membrane Disorders
	Introduction
	Disorders of the oral mucous membranes
		Developmental defects, growths,  and hamartomas
			Bohn’s nodules
			Congenital epulis
			Congenital ranula
			Epstein’s pearls
			Eruption cysts
			Ectopic thyroid tissue
			Sebaceous hyperplasia of the lip (Fordyce spots  or granules)
			Nevus sebaceus
			White sponge nevus (of Cannon)
			Granular cell tumor
			Neurofibroma
		Infections
			Thrush
			Black hairy tongue (lingua pilosa nigra)
			Herpangina and hand, foot, and mouth  disease (HFMD)
			Herpetic gingivostomatitis
			Human papillomavirus
		Vascular lesions (see Chapters 21  and 22)
			Infantile hemangiomas (IH)
			Lymphatic malformations
			Venous malformations
			Port-wine stain
			Pyogenic granuloma
		Signs of extracutaneous disease
			Macroglossia
			Natal teeth
			Congenital fistulae of the lower lip
			Orofacial–digital syndrome, type I
			Oral and genital ulcerations with immunodeficiency
			Behçet disease
			Acatalasemia
		Pigmentary disorders
			Lingual melanotic macule
			Macular pigmentation
		Miscellaneous lesions
			Annulus migrans (geographic tongue)
			Sucking calluses
			Bednar’s aphthae
	Disorders of the genital  mucous membranes
		Labial adhesions
		Perianal pyramidal protrusion
		Perineal groove
		Hymenal tag
		Urethral retention cyst
		Adhesions after circumcision
		Lichen sclerosis
		Pyoderma gangrenosum
	Disorders of the ocular  mucous membranes
		Congenital obstruction of the nasolacrimal duct
		Mucocele of the lacrimal sac
		Blepharitis and chalazion
		Molluscum conjunctivitis
		Colobomata
		Glaucoma
	Key References
	References
31 Hair Disorders
	Introduction
	Neonatal hair development
	Scalp hair whorls
	The hairline
	Heterochromia of scalp hair
	Hair shaft abnormalities
		Monilethrix
		Pili torti
		Trichorrhexis nodosa
		Trichothiodystrophy
		Woolly hair
		Uncombable hair
		Pili annulati
		Trichorrhexis invaginata
	Diffuse alopecia (hypotrichosis) (Box 31.3)
		Hypotrichosis with hair shaft abnormalities
		Isolated congenital alopecia or hypotrichosis without other defects
		Marie-unna hypotrichosis
		Atrichia with papular lesions
		Congenital hypotrichosis and milia
		Hypotrichosis with juvenile  macular dystrophy
		Hypotrichosis–lymphedema–telangiectasia syndrome
		Hypotrichosis associated with ectodermal dysplasias
			Hidrotic ectodermal dysplasia (Clouston syndrome)
			Hypohidrotic ectodermal dysplasia
			Ankyloblepharon, ectodermal dysplasia and clefting syndrome, and Rapp–Hodgkin syndrome
			Bazex–Dupré–Christol syndrome
			Congenital atrichia with nail dystrophy, abnormal facies, and retarded psychomotor development
			Ectodermal dysplasia/skin fragility syndrome
		Hypotrichosis associated  with ichthyoses
			Ichthyoses presenting as the collodion  baby phenotype
			Congenital ichthyosis, follicular atrophoderma, hypotrichosis, and hypohidrosis
			Keratitis, ichthyosis, and deafness (KID) syndrome
			Ichthyosis follicularis, congenital atrichia, and photophobia (IFAP)
		Hypotrichosis with hereditary mucoepithelial dysplasia
		Hypotrichosis with premature  aging syndromes
		Hypotrichosis with  immunodeficiency syndromes
		Loose anagen syndrome
	Localized alopecia (Box 31.4)
		Trauma
		Neonatal occipital alopecia
		Triangular alopecia
		Tinea capitis
		Traction alopecia and trichotillomania
		Aplasia cutis congenita
		Localized alopecia associated with other nevoid conditions
			Aplastic nevus (minus nevus)
			Nevus sebaceus
			Nevus psiloliparus
			Congenital melanocytic nevus
			Cranial meningoceles, encephaloceles and heterotopic meningeal or brain tissue
		Alopecia areata
		Localized alopecia associated  with syndromes
			Hallermann–Streiff syndrome
			X-linked dominant conditions
	Diffuse hypertrichosis
		Primary hypertrichosis
		Transient diffuse hypertrichosis
		Hypertrichosis lanuginosa
		Prepubertal hypertrichosis
		X-linked dominant hypertrichosis
		Ambras syndrome
		Hypertrichosis as part of other genetically determined disorders
			Hypertrichosis with gingival fibromatosis
			Hypertrichosis with osteochondrodysplasia
			Hypertrichosis with congenital eye disorders
				Hypertrichosis, pigmentary retinopathy, and facial anomalies.
				Hypertrichosis with cone/rod dystrophy.
				Hypertrichosis with congenital cataracts and mental retardation.
			Coffin–Siris syndrome
			Cornelia de Lange syndrome
			Leprechaunism (Donohue syndrome)
			Seip–Berardinelli syndrome (congenital  generalized lipodystrophy)
			Rubinstein–Taybi syndrome
			Barber–Say syndrome
		Drug-induced neonatal hypertrichosis
			Fetal alcohol syndrome
			Maternal minoxidil
			Diazoxide
	Localized congenital hypertrichosis
		Congenital melanocytic nevus
		Congenital smooth muscle hamartoma
		Plexiform neurofibroma
		Tufted angioma
		Hypertrichosis with spinal DYSRAPHISM
		Familial cervical hypertrichosis  dysraphism
		Hypertrichosis with cranial meningoceles, encephaloceles,  and heterotopic meningeal  or brain tissue
		Nevoid hypertrichosis
		Hemihypertrophy with hypertrichosis
		Scrotal hair
		Anterior cervical hypertrichosis
		Hairy cutaneous malformations of palms and soles
		Hypertrichosis cubiti
		Ectopic cilia
		Distichiasis
		Hypertrichosis pinnae auris
	Key references
	References
32 Nail Disorders
	Introduction
	Beau’s lines
	Alterations in nail shape
	Alterations in nail size
	Micronychia
	Nail–patella syndrome
	Congenital onychodysplasia of the index fingers
	Onychoatrophy
	Ectopic nails
	Hypertrophy of the nail
	Pachyonychia congenita
	Ingrown toenails
	Paronychia and infectious  nail dystrophy
	Onychomycosis
	Onycholysis
	Chromonychia
	Key references
	References
Index
	A
	B
	C
	D
	E
	F
	G
	H
	I
	J
	K
	L
	M
	N
	O
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	R
	S
	T
	U
	V
	W
	X
	Y
	Z