Perifolliculitis capitis abscedens et suffodiens L66.3

Author: Prof. Dr. med. Peter Altmeyer

Co-Autor: Dr. Behrus Darvishan

Last updated on: 18.12.2020

Synonym(s)

Atrophic disease with tufted hair; Dissecting cellulitis; Dissecting cellulitis of the scalp; Dissecting terminal hair folliculitis; Folliculitis and perifolliculitis capitis abscendes et suffodiens; Folliculitis et perifolliculitis capitis abscedens et suffodiens; Folliculitis profound decalvitating; Profound decalvitating folliculitis; pyoderma fistulans significa

History
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Spitzer, 1903; Hoffmann, 1908

Definition
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Rare, almost only in men occurring, abscessing, fistula, chronic hair follicle inflammation. Occurs within the acne triad. The clinical picture must be separated from the folliculitis decalvans. Folliculitis decalvans lacks the clinical relation to acne conglobata as well as the formation of fistula ducts.

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Acne inversa

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Occurrence/Epidemiology
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Especially common among members of the black population/person of color.

Etiopathogenesis
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Unknown; tufted hairs with a wide acroinfundibulum susceptible to infections and irritation of the epidermis seem to be favourable.

Manifestation
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Occurs in men with severe seborrhoea between the ages of 20 and 40 (Scheinfeld N 2014).

Localization
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Capillitium, hairy neck, perianal, axillary, inguinal.

Clinical features
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Frequently, several follicular papules and pustules, painless and perforating subcutaneous nodules or granulomas, colliquation necroses as well as fox-like, epithelial-lined undertunnelings of the scalp can be seen. Emptying of pus or a hemorrhagic secretion under pressure. Small-spotted alopecia, healing with formation of atrophic reflecting bridges (no more detectable follicles) and hypertrophic tip scars.

Histology
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Abscessing, melting and granulomatous inflammation; foreign body reactions. Hyperplasia of the sebaceous gland apparatus.

Diagnosis
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Clinical picture with complex situation of acne conglobata and chronic fistulizing inflammation of the hairy scalp.

Differential diagnosis
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Folliculitis decalvans: Clinically no evidence of acne conglobata. No evidence of fistula ducts.

Acne keloidalis (Folliculitis scleroticans nuchae): Infestation of the neck region; no fistula ducts. No evidence of acne conglobata.

Hidradenitis suppurativa: Does not affect the capillum

Tuberculosis cutis colliquativa: Extremely rare disease pattern in the western hemisphere. Clinical and histological evidence of the underlying tuberculosis.

Actinomycosis: Localization capilltium atypical; no evidence of acne conglobata

Therapy
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Exclusion of diabetes mellitus.

External therapy
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Glucocorticoids in combination with internal retinoids seem to bring the best results. Glucocorticoid tinctures, if necessary with added salicylic acid such as Triamcinolon-Spiritus with Salicylic Acid R262 should be applied over several days in the area of inflammatory changes. Alternatively: Betamethasone gel(e.g. Diprosis Gel). Also use moist compresses with antiseptic additives such as potassium permanganate (light pink) or quinolinol (e.g. Chinosol 1:1000 or R042 ).

Internal therapy
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Antibiotics: in a smaller study, the combination of clindamycin/rifampicin (each 200mg/2xday p.o.) was described as successful over a period of 10 weeks (Scheinfeld N 2014).

Retinoids such as isotretinoin (e.g. Aknenormin®) 0.2-0.5 mg/kg bw/day. In the further course reduction of the dose to 10mg 2-3x/week.

Moderate success is also seen with hydroxychloroquine 150 mg/day p.o. (own experience is negative).

Individual case reports document positive success with a combination therapy of dapsone (100 mg/day) and isotretinoin (1 mg/kg bw/day) as well as with monotherapeutic TNF-alpha blockers.

Operative therapie
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In circumscribed foci, depilation of affected hairs. Otherwise, fistula tract clefts. After healing, plastic surgery therapy and transplantation of the patient's own hair may be necessary.

Progression/forecast
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Chronic course over years to decades, possible development of secondary amyloidosis; no recurrence after radical surgical excision.

Note(s)
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Together with acne inversa, acne conglobata and pilonidalsinus, the disease is summarized as the "tetrade of follicular occlusion".

Literature
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Bellew SG et al (2003) Successful treatment of recalcitrant dissecting cellulitis of the scalp with complete scalp excision and split-thickness skin graft. Dermatol Surgery 29: 1068-1070
Bolz S et al (2008) Successful combined isotretoinoin and dapsone therapy for perifolliculitis capitis abscedens ert suffodiens. JDDG 6: 44-47
Chen W et al (2017) Should hidradenitis suppurativa/acne inversa best be renamed as "dissectingterminal
hair folliculitis"? Exp Dermatol 26:544-547.
Hoffman E (1908) Perifolliculitis capitis abscedens et suffodiens: case presentation. Dermatol Z (Berlin) 15: 122-123
Karpouzis A et al (2003) Perifolliculitis capitis abscedens et suffodiens successfully controlled with topical isotretinoin. Eur J Dermatol 13: 192-195
Scerri L et al (1996) Dissecting cellulitis of the scalp: response to isotretinoin. Br J Dermatol 134: 1105-1108
Scheinfeld N (201) Dissecting cellulitis (Perifolliculitis Capitis Abscedens et Suffodiens):
acomprehensive review focusing on new treatments and findings of the last decadewith
commentary comparing the therapies and causes of dissecting cellulitis tohidradenitis
suppurativa. Dermatol Online J 20:22692.
Sharp L (1903) Dermatitis follicularis et perifollicularis conglobata. Dermatol Z (Berlin) 10: 109-120
Vañó-Galván S et al (2015) Folliculitis decalvans: a multicentre review of 82 patients.J Eur Acad Dermatol Venereol 29:1750-1757.