October 18, 2015 A Review of Alopecia Saint Joseph Mercy Hospital, Ann Arbor, Michigan Disclosures No financial relationships exist with commercial interests Saint Joseph Mercy Dermatology Residency Outline 1. Non-cicatricial alopecia - Androgenic alopecia - Trichotillomania - Telogen effluvium - Alopecia areata 2. Cicatricial alopecia - Central centrifugal cicatricial alopecia - Dissecting cellulitis - Lichen planopilaris - Folliculitis decalvans - Discoid lupus - Pseudopelade of Brocq - Acne keloidalis 3. Comparative Review of Dermatopathology NON-CICATRICIAL ALOPECIA Androgenic Alopecia (Male/Female Pattern Hair Loss) • Epidemiology: 80% of Caucasian men and 50% of women affected by age 70 • Hereditary • Pathogenesis: 5α-reductase • Testosterone -----------------> Dihydrotestosterone (DHT) • Type I 5α-reductase: sebaceous glands and liver • Type II 5α-reductase: scalp, beard, and chest hair follicles, liver, prostate – DHT leads to miniaturized hair follicles and hair shafts (terminal vellus) Clinical Presentation and Staging Men: Hamilton-Norwood Women: scale Sinclair/Ludwig/Olsen scales Sinclair scale∫ Ludwig scale Androgenic Alopecia Treatment • Topical minoxidil (2% or 5%) • Finasteride – Type II 5α-reductase inhibitor – 1 mg daily – Pregnancy Category X – Stops hair loss in 90% of men for at least 5 years; hair regrows in 65% of cases • Dutasteride – Type I and II 5α-reductase inhibitor – 0.5mg/day – More effective than Finasteride – Pregnancy Category X • Women – Topical minoxidil – OCPs – Spironolactone – Finasteride 2.5 or 5mg daily (extreme caution if child bearing potential) • Surgical Treatment options – Hair transplantation – Scalp reduction Androgenic Alopecia Treatment • Camouflage techniques – Hair pieces – Wigs – Creative styling – Hair dyes/Spray on hair/Hair fibers • New and emerging treatments – Platelet rich plasma (PRP) • More studies needed to evaluate mechanism – Laser combs • 655 nm • Promising results in literature • Safe with few adverse side effects Telogen Effluvium • Pathogenesis: – Premature conversion of anagen hairs to telogen hairs secondary to a precipitating event/trigger • Common triggers: – Surgery, fever, medications – Crash dieting, iron deficiency – Papulosquamous disease affecting scalp – Thyroid disease – Pregnancy (2-3 months after delivery) – Severe emotional distress • Chronic form with no precipitating factors • Clinical Presentation: – Increased shedding of entire scalp (150-400 hairs/day) – Positive hair pull test (>10% club hairs) – Physician may not appreciate a decrease in hair density, while patient may complain of noticeably thinner hair Telogen Effluvium • Trichoscopy: – Empty follicles, short regrowing hairs of normal thickness • Diagnosis: – Confirmed by >20% telogen hairs on biopsy • Treatment: – Identify and avoid trigger – Check TSH, ferritin level – If drug induced, d/c drug – Reassurance, usually remits in 3-6 mos Trichotillomania • Classified under American psychiatric association’s DSM-V as an “obsessive- compulsive disorder” • Epidemiology – MC in girls <10 • Etiology – Habitual hair pulling • Clinical presentation – Irregular patches of alopecia with hairs of varying lengths – Scalp, eyebrows, eyelashes or pubic hair – Uninvolved areas of scalp appearing completely normal – Trichophagia: chewing & swallowing of hairs trichobezoars Trichotillomania • Diagnosis made clinically • Treatment – Behavior modification – Psychotherapy – SSRIs – N-acetylcysteine • Increases glutamate concentration reduced compulsive behavior Alopecia Areata • Epidemiology – Most common in children and young adults • Etiology/pathophysiology – Th1-mediated autoimmune condition • IL-2, IFN-γ and TNF-α – Early onset/familial clustering • HLA-DR4, -DR11, -DQ7 • Associations – Thyroid disease, vitiligo, type 1 diabetes mellitus, pernicious anemia, systemic lupus • Clinical presentation – Smooth round patches (1-5 cm) of hair loss – Exclamation point hairs Alopecia Areata Subtypes • Extent of involvement – Alopecia patchy (transient/persistent) • Hair loss in patches for < or > 6 months respectively – Alopecia totalis • Complete loss of scalp hair – Alopecia universalis • Complete loss of scalp & body hair • Pattern of hair loss – Ophiasis • Band like hair loss on peripheral temple/occiput – Sisaipho • Inverse ophiasis – Diffuse • Generalized thinning – Linear Alopecia Areata • Treatment – First line • Intralesional steroids • Topical immunotherapy – Second line • Topical sensitizers, corticosteroids, prostaglandin analogues, minoxidil and retinoic acid • Camouflage • PUVA – New and emerging therapies • Janus kinase inhibitors (ruxolitinib, tofacitinib) Inhibition of Janus Kinases in Alopecia Areata Inhibition of Janus Kinases in Alopecia Areata CICATRICIAL ALOPECIA Central Centrifugal Cicatricial Alopecia (CCCA) • Epidemiology – 3:1 female to male ratio – Most common form of scarring alopecia in African-Americans • Pathogenesis – Hypothesized secondary to premature desquamation of the inner root sheath – Exacerbated by chemical hair relaxers • Clinical presentation – Pruritus and tenderness – Centered on the vertex of the scalp, gradually expands centrifugally – Polytrichia: Multiple hair shafts emerging from one ostia – Inflammation in peripheral zone Central Centrifugal Cicatricial Alopecia (CCCA) • Treatment – Discontinuation of traumatic hairstyling – Corticosteroids • Topical and intralesional – Oral antibiotics • Tetracycline family • New and emerging therapy – Hair transplantation Lichen Planopilaris • Epidemiology – F > M – Caucasian > African • Pathogenesis – Unknown • Clinical presentation – Variable pattern – Pruritus and tenderness often present – Scattered foci of partial hair loss w/ perifollicular erythema, follicular spines and scarring – >50% associated with cutaneous or oral lichen planus Lichen Planopilaris • LPP clinical patterns – Frontal fibrosing alopecia – Graham Little Piccardi syndrome Lichen Planopilaris • Treatment – Often resistant to therapy – Corticosteroids • Topical, intralesional and oral – Antimalarials – Anecdotal • Cyclosporine, mycophenolate mofetil, systemic retinoids, or low-dose methotrexate • New and emerging treatment – Pioglitazone 15mg daily • PPAR gamma agonist Discoid Lupus • Epidemiology – Discoid lesions may be isolated or in association with systemic lupus • Pathogenesis – Photosensitive disorder cytotoxic keratinocyte damage • Clinical Presentation – Follicular plugging – Central atrophic scarring – Peripheral hyperpigmentation and erythema • Trichoscopy – Follicular red dots • Corresponds to dilated vessels, extravasated RBCs, and keratin plugs Discoid Lupus • Treatment – Photoprotection – Corticosteroids • Topical, intralesional and oral – Antimalarials – Anecdotal • Cyclosporine, mycophenolate mofetil, systemic retinoids, or low-dose methotrexate • New and emerging treatments – Tacrolimus lotion 0.3% • Used as adjunct to antimalarials • Hair regrowth was seen over 3 month period – Imiquimod cream 0.5% • Applied 3x a week every other week for 2 months Acne Keloidalis Nuchae • Most common in African American men, Hispanics, Asians – 20:1 male to female • Etiology unknown – No genetic factors identified – Bacterial infection, ingrown hairs not implicated • Clinical – Posterior scalp and neck – Follicular pustules firm, dome shaped papules smooth keloidal plaques Acne Keloidalis Nuchae • Treatment – Prevention • Avoidance of mechanical irritation to the posterior hairline – Tretinoin gel – Topical and/or systemic antibiotics – ILS corticosteroids • New and Emerging – Targeted UVB therapy • Decreased mean lesion count from 14.8 to 7.0 after 16 weeks Dissecting Cellulitis Perifolliculitis capitis abscedens et suffodiens • Epidemiology – Black males ages 20-40 • Pathogenesis – Follicular occlusion • Clinical Presentation – Pustules, nodules, abscesses and sinuses on the scalp – Evolves into cicatricial alopecia – Secondary Staph aureus infection Dissecting Cellulitis • Treatment – Incision/excision and drainage • No effect on disease progression – Topical antibiotics – Intralesional corticosteroids – Oral antibiotics: doxycycline and rifampin • Moderate improvement with relapse upon discontinuation – Isotretinoin (0.5-0.8 mg/kg/day) • Complete remission within 3 months in 92%, but frequent relapses after discontinuation • New and emerging treatment – Adalimumab 40 mg every 2 weeks • Symptoms relieved within 8 weeks, frequent relapses Folliculitis Decalvans • Epidemiology – Young male adults • Pathogenesis – Altered host response to Staph aureus • Clinical – Pain, itching, burning – Early: follicular papules – Late: crops of pustules with central scarring • Treatment – Topical clindamycin, mupirocin – Tetracyclines – Oral clindamycin + rifampin Pseudopelade of Brocq • Epidemiology – Rare; typically Caucasian adults • Etiology – Likely represents the end-stage scarring alopecia (LPP, DLE, etc) – Diagnosis of exclusion! • Clinical presentation – “Footprints in the snow” – Irregularly shaped, widely distributed patches – Hypopigmentation, atrophy – No follicular hyperkeratosis or perifollicular inflammation DERMATOPATHOLOGY Dermatopathology: Non-Scarring Alopecias Telogen Alopecia Androgenic Trichotillomania Effluvium Areata Anagen:Teloge Decreased Decreased Variable Decreased n Ratio Catagen Hairs Rare - Common Common Miniaturization Yes No No Yes Peribulbar Trichomalacia, >20% lymphocytic Additional pigment casts, Anisotrichosis telogen inflammation Findings empty anagen hairs (swarm of follicles bees) Dermatopathology: Scarring Alopecias