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Disorders of Pigmentation
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Postinflammatory Pigmentary Changes
Occurs Following Dermatitis Acne Infection Injury Description Hyperpigmentation Hypopigmentation Course Resolution over a few months
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Pigmentary Mosaicism Genetic mosaicism Timing Description
Born with 2 genetically different cell lines Timing May be seen at birth May appear in infancy or early childhood Description Whorled, fountain-like pattern of alternating hypo- and hyperpigmentation Blaschkoid distribution Macules without vesicular or verrucous lesions Other problems CNS, MSK, eye More likely if skin findings are prominent
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Vitiligo Acquired partial to complete loss of pigmentation Description
Well-demarcated hypopigmented and depigmented macules and patches Enhance under Wood’s lamp Absence of melanocytes Location Eyes, mouth, genitals, elbows, hands and feet Can appear anywhere Cause Autoimmune
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Ash-Leaf Spots Timing Description Location Associated with Congenital
Well demarcated, hypopigmented macules Lancinate shape May enhance with Wood’s light Location Truncal Associated with Tuberous Sclerosis Normal 0.5% newborns Nevus depigmentosus Localized form of pigmentary mosaicism
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Albinism Timing Description X-linked ocular AR Complications
Congenital Description Hypopigmentation of the skin, eyes and hair X-linked ocular Skin is normal AR Oculocutaneous Many variants Complications At risk of skin cancer Must avoid excessive sun and use sunscreen
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Albinism Variants Tyrosinase-negative OCA Tyrosinase-positive OCA
No trace of pigment Snow-white hair, pinkish-white skin, translucent or blue irises Also may have nystagmus, moderate to severe strabismus and poor visual acuity Tyrosinase-positive OCA Look similar at birth Develop variable amounts of pigment with increasing age Eye color – gray to light brown Hair color – blonde or light brown
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Piebaldism Partial albinism AD – rare Description White forelock
Triangular patch of depigmentation and white hair on the frontal scalp Circumscribed congenital leukoderma Hypopigmented or depigmented macules on the face, neck, ventral trunk, flanks or extremities May have scattered patches of normal pigmentation within
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Piebaldism Variants Waardenburg syndrome Wolf syndrome
Lateral displacement of the inner canthi and inferior lacrimal ducts Flattened nasal bridge Sensorineural deafness Wolf syndrome AR Neurologic deficits
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Café-au-lait spots Description May be a marker for Tan macules
Neurofibromatosis type 1 Small and smooth borders McCune-Albright Large and segmental with jagged borders Large or >4
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Question 10 An 8-year-old boy presents with a 2- week history of an enlarging, tender lump on the scalp. The only notable finding on physical examination are alopecia overlying a boggy mass on the scalp and posterior cervical lymphadenopathy. Of the following, the MOST appropriate treatment is Cefazolin orally Griseofulvin orally Incision and drainage Ketoconazole topically Mupirocin topically Answer B
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Disorders of the Hair and Nails
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Alopecias Alopecia Telogen Effluvium Alopecia Anagen Effluvium
Partial, temporary 3 months after an emotional or physical stress <50% loss Alopecia Anagen Effluvium Sudden loss of growing hairs 80% Tapering of the hair shaft and loss of adhesion to the follicle Chemotherapy Telogen Effluvium
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Alopecias Alopecia Areata Description Location Severity Cause
Round or oval patches of hair loss Absence of inflammation and scaling Short (3-6mm) easily removable hairs at the margins Location Scalp, eyebrows, lashes or body Severity May be diffuse or generalized Cause Autoimmune Other findings Scotch-plaid pitting of the nails Course Difficult to predict Treat any comorbid conditions
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Alopecias Trichorrhexis Nodosa Timing Description Cause Course
At any age Description Hair shaft breakage Brittle, short hairs Frayed edges by microscopy Cause Damage to the outer cortex of the hair shaft Trauma, chemicals Course Resolution with removal of insult
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Alopecias Friction alopecia Traction alopecia Infants Self-limited
Prevented/treated with tummy-time If severe or long standing consider neglect Traction alopecia Maintaining a tight pull on the hair shafts Ponytails, pigtails, braids, cornrows Course Shaft fractures and follicular damage Can lead to permanent scarring
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Alopecias Trichotillosis AKA trichotillomania Timing Description
School-age children and adolescents Description Bizarre patterns of hair loss Usually broad linear bands on vertex or sides of scalp Side opposite of dominant hand Short, broken-off hairs with stubs of different lengths Never completely bald Associations Situational stress Sometimes psychiatric
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Aplasia Cutis Congenita
AR Timing Congenital Etiology Absence of or failure of formation of a localized area of scalp or skin Dermis and epidermis Some involve subq tissue and rarely calvarial defects Location Single lesion on vertex of scalp Rarely multiple or involving trunk or extremities
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Aplasia Cutis Congenita
Description Birth Sharply circumscribed open and weeping ulceration that may be covered by a thin membrane or crust Healed in several weeks to months Usually smooth atrophic scar Look for hair collar sign Neural tube defects Associations Limb defects, genetic anomalies Treatment Saline compresses Topical antibiotics Sterile dressings
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Tinea Capitis Most common organism Description is variable
Trichophyton tonsurans Does not fluoresce >95% Microsporum canis Fluoresces bluish-green 5% Description is variable Mild erythema and scaling with partial alopecia Widespread breakage of the scalp “salt-and-pepper” More erythema, edema and pustules Crusting Heaped up scale with small pustules
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Tinea Capitis Description is variable Treatment Course Prevention
Kerion Intense inflammation with raised, tender, boggy plaques or masses studded with pustules KOH mount shows infected hairs Associated occipital, postauricular and posterior cervical adenopathy Treatment Oral antifungal agents 6 weeks to 4 months Selenium sulfide shampoo Steroids Severe inflammation Course May lead to scarring and permanent hair loss if untreated Prevention Do not share!
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Congenital and Genetic Disorders
Monilethrix AD Developmental hair defect Timing 2-3 month of age Description Brittle, beaded hair Periodic narrowing of the hair shaft Location Scalp most severely affected Course Permanent Appearance may improve with age
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Congenital and Genetic Disorders
Pili torti Hair shaft is twisted on its own axis Timing Appears with first terminal hair growth of infancy Location Localized or generalized Associations Menkes kinky hair syndrome Defect of copper absorption CNS, CV, Skeletal systems
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Disorders Affecting Nails
Paronychia Acute Red, swollen, tender nail fold Bacterial invasion Staph or strep Chronic One or several nails Chronic dermatitis or frequent exposure to water Nail dystrophy Candida species Treat with topical antimycotics
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Disorders Affecting Nails
Onychomycosis Fungal infection Uncommon before puberty Dystrophic nails More commonly a result of trauma Or underlying dermatosis
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Disorders Affecting Nails
Subungual hemorrhage Trauma Crush injuries Turf toe Jamming toe into the end of a shoe suddenly Description Purplish-brown pigment Treatment Evacuation if painful
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