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Cicatricial (scarring) alopecias

Cicatricial (scarring) alopecias
  Epidemiology Clinical features on scalp Nonscalp involvement Pathology
Lymphocytic primary cicatricial alopecias
Discoid lupus erythematosus Onset usually in young adulthood; more common in females than males; may occur independently or in patients with SLE Erythematous, atrophic plaques with follicular plugging present centrally, hyperpigmentation, and/or hypopigmentation Face, ears, and other sites may be affected; a minority of cases are associated with SLE Interface dermatitis, epidermal atrophy, follicular plugging, basement membrane thickening, perifollicular superficial and deep lymphoplasmacytic infiltrate; DIF shows IgG, IgM, and C3 at the BMZ
Lichen planopilaris Most common in White and East Indian females Areas of scarring and follicles with erythema and scale; itching and pain may be severe May be accompanied by cutaneous, nail, or mucous membrane lichen planus Lichenoid infiltrate around the isthmus and infundibulum, interface dermatitis, concentric lamellar fibroplasia
Frontal fibrosing alopecia Mostly postmenopausal females Perifollicular erythema, follicular hyperkeratosis, band-like frontal alopecia Loss of eyebrows common, papules on face Lichenoid infiltrate around the isthmus and infundibulum, apoptotic keratinocytes in outer root sheath, concentric lamellar fibroplasia
Classic pseudopelade of Brocq Most common in middle-aged females Skin-colored scarred plaques resembling "footprints in the snow" N/A Lymphocytic infiltrate around infundibulum, concentric lamellar fibroplasia around follicles in later lesions
Central centrifugal cicatricial alopecia Most common in females of African origin Patch of scarring alopecia on crown of scalp that progresses centrifugally N/A Premature desquamation of the inner root sheath, loss of sebaceous glands, variable perifollicular chronic inflammation at isthmus and infundibulum, concentric lamellar fibroplasia
Alopecia mucinosa May be associated with mycosis fungoides or CTCL Erythematous or skin-colored indurated plaques with alopecia, follicular papules Eyebrow involvement may be present Mucin deposits in outer root sheath, eventual replacement of follicles by mucin, lymphocytic infiltrate
Keratosis folliculars spinulosa decalvans X-linked inheritance of mutation in the MBTPS2 gene; less commonly autosomal dominant transmission Noninflammatory, hyperkeratotic follicular papules and progressive hair loss Eyebrow and eyelash involvement may be present, photophobia Follicular plugging and hypergranulosis, primarily lymphocytic perifollicular infiltrate, fibrosis
Neutrophilic primary cicatricial alopecias
Dissecting cellulitis of the scalp Most common in young Black males Inflammatory papules, pustules, fluctuant nodules, abscesses; may also have keloidal scarring May be associated with hidradenitis suppurativa and acne conglobata Follicular occlusion, intrafollicular and perifollicular neutrophilic and lymphoplasmacytic infiltrate; follicular perforation, deep abscesses and sinus tracts in later stages
Folliculitis decalvans Most common in young and middle-aged adults, particularly males Patches of alopecia, erythematous follicular papules, pustules, follicular hyperkeratosis, tufted folliculitis N/A Interfollicular and perifollicular neutrophilic infiltrate; mixed infiltrate, granulomas, and fibrosis in later lesions
Mixed primary cicatricial alopecias
Acne keloidalis nuchae Most common in young Black males Dome-shaped papules and pustules on the occipital scalp and nape of the neck; keloids may develop N/A Neutrophilic or lymphoplasmacytic inflammatory infiltrate around the isthmus and infundibulum; granulomatous infiltrate may also be present; marked fibrosis
Acne necrotica Usually occurs in adults Umbilicated, pruritic, painful papules that undergo central necrosis and resolve with varioliform scars N/A Infundibular folliculitis with necrosis, lymphocytic or mixed inflammatory infiltrate
Erosive pustular dermatosis of the scalp Most common in older adults, particularly females Pustules, erosions, and crusted plaques N/A Nonspecific early findings; older lesions with a chronic mixed inflammatory infiltrate and fibrosis
Other
Tinea capitis Usually affects children Scaly patches or plaques; kerion may develop Tinea corporis may be present Perifollicular inflammation and fungal elements within or around the hair shaft
SLE: systemic lupus erythematosus; DIF: direct immunofluorescence; IgG: immunoglobulin G; IgM: immunoglobulin M; BMZ: basement membrane zone; CTCL: cutaneous T cell lymphoma.
Data from:
  1. Otberg N, Shapiro J. Hair growth disorders. In: Fitzpatrick's Dermatology in General Medicine, 8th ed, Goldsmith LA, Katz SI, Gilchrest BA, et al. (Eds), McGraw Hill 2012.
  2. Childs JM, Sperling LC. Histopathology of scarring and nonscarring hair loss. Dermatol Clin 2013; 31:43.
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