Hair Disorders in Children

Stanton K Wesson MD Department of Dermatology University of Florida Disclosures

• I have no conflicts of interest Objectives

• To present some of the common diseases of hair in children and describe their diagnostic features within an easily applied system of categories. • To present some of the uncommon hair disorders in children and describe some diagnostic features • To provide some recommendations for treatment or management for these disorders

Diagnostic categories of hair loss in children • Acquired circumscribed alopecia • Congenital circumscribed alopecia • Acquired diffuse alopecia • Congenital diffuse alopecia Acquired Circumscribed Alopecia

• Alopecia Areata • Tinea Capitis • Traction Alopecia (include trichotillomania) Alopecia Areata

• Hair follicle has “immune privilege”, does not express MHC class I or II antigens. • In alopecia areata these are expressed and result in “autoimmune attack” by activated T- lymphocytes. • Hair loss is usually circumscribed but may rarely involve the entire scalp and/or body hair. • May also have nail pitting and association with “atopy” Alopecia areata and totalis Alopecia areata Nail pitting with AA Nail Pitting

Alopecia Areata management

• For typical AA, 80-90% demonstrate spontaneous regrowth within 1 year • Alopecia totalis occurs less than 5% • Alopecia universalis occurs less than 1% • Topical and very rarely systemic corticosteroids • Topical immuno-therapy with SADBE or diphencyprone • Wigs and support group: Nat. Alopecia Areata Foundation

Tinea Capitis

• Fungus infection of the scalp and hair resulting in circumscribed hair loss • T. tonsurans and M. canis are the more common fungi • M. canis fluoresces with Wood’s light but T. tonsurans does not. • M. canis is usually not spread from human to human but T. tonsurans is

Treatment

• First line treatment is with griseofulvin at 20- 25mg/kg/day for 6-8 weeks may take longer. (Best given with fatty food) • Alternative: Terbinafine 3mg/kg/day for up to 4-6 weeks • Some suggest using 2% ketoconazole shampoo 2-3 times a week for the first two weeks to shortent the time for return to school. Traction, Traumatic Alopecia

• Traction alopecia due to hair styling and care practices • Physical trauma, thermal or chemical • Trichotillomania Traction Alopecia

• Most commonly associated with styling practices • Braiding, cornrows other combing or brushing practices • Treatment is through patient/parent education and gentle hair care practices Traction Alopecia Traction Alopecia Traumatic Alopecia

• Thermal and chemical effects • Relaxers and perms • Hot combs and hot wave or curler Traumatic Alopecia Traumatic Alopecia

Thermal injury Relaxers Relaxers and Straighteners Trichotillomania

• The result of “hair pulling” • Often follows events of emotional trauma. • Thought of as an impulse control emotional disorder. May be associated with more significant psychiatric disorders. • Often appears prior to age 18, mean for boys is 8 years, for girls is 12 years. • May be localized or diffuse. Trichotillomania Trichotillomania Trichotillomania

• Management • Attempt to first identify the emotional or precipitating event. • Parental and patient denial is common • Psychodynamic therapy, behavior modifications therapy • Antidepressants • No good consensus on best choice and outcomes Congenital Circumscribed Alopecia

• Nevoid disorders of scalp: nevus sebaceus, epidermal nevus • Aplasia cutis congenita • Triangular alopecia of the frontal scalp • Usually have some associated scarring

Nevoid Disorders

Nevus Sebaceus Nevus sebaceus Epidermal nevus

Associated with proteus Neck at scalp with alopecia syndrome Mastocytoma Rare associated syndromes with epidermal nevi • May be associated with developmental anomalies of skin, brain, eye and musculo-skeletal systems • PTEN-hamartoma syndromes: have segmental overgrowth, A-V malformations, lipomatosis, and epidermal nevi • Proteus-like syndromes: have rapid asymetric post natal overgrowth with hyperostosis, A-V malformations, dysregulation of fat metabolism, epidermal nevi and cerebriform connective tissue nevi.

Rare associated syndromes

• CHILD syndrome: congenital hemi-dysplasia, ichthyosiform nevi, limb defects. X-linked dominant, lethal in males. NSDHL gene which encodes for 3-beta-hydroxysteroid dehydrogenase involoved in cholesterol metabolism. • FGFR3 fibroblast growth factor receptor 3 syndrome: epidermal nevi, brain defects, cortical atrophy, subdural hemorrhage, hypoplastic corpus callosum and skeletal dysplasia.

Aplasia Cutis Congenita Aplasia Cutis Congenita Triangular alopecia of the frontal scalp Acquired Scarring Diffuse Alopecia

• Differential diagnosis • Post infection including bacterial, viral and fungal • Lichen planus • Lupus erythematosus • Post Injury including trauma, chemical or thermal burn, radiation

Congenital Diffuse Alopecia

• Differential Diagnosis • Trichorrhexis nodosa, (broomstick anomaly) familial and argininosuccinic aciduria • Pili torti, (twisted hair) classic and Menkes kinky hair syndrome • Monilethrix (beaded hair) • Trichorrhexis invaginata, (bamboo hair) Netherton’s syndrome • Ectodermal Dysplasias

Rare syndromes

• Crandall’s syndrome: alopecia, deafness, hypogoandism. • Bjornstad syndrome: deafness and pili torti • Bazex-Dupre-Christol syndrome: follicular atrophoderma, sparse coarse hair, mila, dry skin, localized hypohidrosis and disposed to multiple basal cell ca.

Ectodermal Dysplasias

• 150 types: many mutations • 4 basic subgroups: • ED-1: trichodysplasia • ED-2: dental dysplasia • ED-3: onychodysplasia (nails) • ED-4: dyshidrosis (abnormal seat glands) Ectodermal Dysplasias

• Hair: thin, sparse, light color, coarse, brittle, twisted, pili torti and annulati • Nails: thick, thin, abnormal shape, ridging, brittle, discolored, slow growing, absent • Teeth: abnormal teeth, peg shaped, absent, pointed, defective enamel, caries • Sweat glands: abnormal function, reduced to absent, poor body temp regulation, fever, heat injury, seizures, neurologic development

Evaluation and Management

• Dermatology assessment • Genetics assessment • Many of these conditions cannot be intervened with except for counselling • Management of associated features Acquired Diffuse Alopecia

• Telogen effluvium • Endocrine: hypothyroidism, hypopituitaraism, hypoparathyroidism, diabetes mellitus. • Chemical: thallium (rat poison), antithyroid drugs, heparin, coumarin, antimetabolites. • Nutritional: hypervitaminosis A, acrodermatitis enteropathica (zinc deficiency), malnutrition states. • Androgenetic: adolecent onset (rare), anabolic steroid use.

Thank You ! • Any questions??