Perifolliculitis capitis abscedens et suffodiens treatment with tumor necrosis factor inhibitors: A case report and review of published cases

Abstract

Perifolliculitis capitis abscedens et suffodiens (PCAS) or dissecting cellulitis is a rare condition presenting deep follicular occlusions, follicular ruptures and follicular infections in the scalp area with unknown etiology, which consequently cause primary neutrophilic cicatricial alopecia by the repeated follicular inflammation. PCAS is categorized as one of the “follicular occlusion tetrad” along with hidradenitis suppurativa, acne conglobata and pilonidal cyst. In the pathogenesis of the follicular occlusion tetrad, the involvement of neutrophils and its activator tumor necrosis factor (TNF) have been discussed. Here, we report a case of PCAS that was successfully treated with adalimumab, a human anti‐TNF monoclonal antibody. This is the first Asian case of PCAS that was improved by a TNF inhibitor.

Dissecting Cellulitis of the Scalp: A Review and Case Studies of Surgical Reconstruction

“… 5 , 17 The FO tetrad adds pilonidal disease. 4 , 6 – 9 Members of this tetrad are normally diagnosed without mention of the others; however, there are reports of these diseases affecting individuals concurrently. 6 , 7 , 18 Although debate remains regarding the actual cause of these diseases, a common pathogenesis has been suggested.…”

“… 7 The sinuses become more intense and eventually coalesce to develop as a chronic suprainfection that can only be suppressed with antibiotic therapy. 4 , 19 , 20 Scientific work into the pathogenesis of DCS reflects its rarity as a disease entity. Histology, as described by Scheinfeld, 8 reveals lesions with dense neutrophilic, lymphocytic, histiocytic, and plasma cellular infiltrate early on.…”

“…There are a wide variety of treatments that have been reported, including suppressive antibiotic therapy, 4 , 8 , 19 , 20 , 28 zinc sulfate, 29 , 30 isotretinoin, 31 – 34 corticosteroids, 35 antiandrogens, 36 biologic agents, 4 , 37 – 39 laser therapy, 40 – 43 aminolevulinic acid-photodynamic therapy, 44 – 46 radiation therapy, 47 limited surgical therapy, 48 and radical surgical resection (scalpectomy). 11 , 49 – 51 Each of these therapies has its own mechanism of action.…”

“…3 It is identified as one of the follicular occlusion (FO) triad or tetrad, including hidradenitis suppurativa (HS), acne conglobata, and pilonidal disease. 4 – 9 The primary pathologic event is obstruction of the pilar infundibulum through hyperkeratosis, accumulation of obstructed follicular products with rupture, and an intense inflammatory reaction at the bulb of the hair follicle. 10 This leads to the formation of pustules that develop into tracts ultimately coalescing to chronically inflamed tissue.…”

“… 10 This leads to the formation of pustules that develop into tracts ultimately coalescing to chronically inflamed tissue. 4 Pathologic examination can show reduced follicle number with evidence of follicular rupture and presence of neutrophils. It has been associated with sternoclavicular hyperostosis, polyarticular arthritis, and human leukocyte antigen B27 (HLA-B27) seronegative spondyloarthropathy.…”