Therapeutic options for perifolliculitis capitis abscedens et suffodens: A review

Abstract

Perifolliculitis capitis abscedens et suffodiens (PCAS) is a chronic skin inflammatory disease characterized by relapsing folliculitis and painful, fluctuant abscesses, sinus tracts, and scars. The treatment of PCAS is challenging and clinical practice varies a lot, and how to choose the best treatment for PCAS is a real problem for clinicians. We reviewed articles providing treatment options for patients with PCAS in different databases. Dermatologists may find this review helpful to meet the challenges of PCAS management, but there is still a lack of authoritative guidelines. In the future, more robust randomized control trials are needed to determine the best treatment for PCAS. K E Y W O R D S biologics, dissecting cellulitis of the scalp (DCS), perifolliculitis capitis abscedens et suffodiens (PCAS), retinoids, treatment option 1 | INTRODUCTION Perifolliculitis capitis abscedens et suffodiens (PCAS), also known as dissecting cellulitis of the scalp (DCS) or Hoffman's disease, is a chronic skin inflammatory disease characterized by relapsing folliculitis and painful, fluctuant abscesses in the scalp that lead to the formation of sinus tracts and scarring. 1 PCAS may occur along with hidradenitis suppurativa (HS), acne conglobate and pilonidal sinus, termed the follicular occlusion tetrad. These four different clinical conditions appear to have common pathogenic events including follicular hyperkeratosis, poral obstruction, dilation and rupture, followed by neutrophilic and granulomatous inflammatory response, bacterial overgrowth, scarring, and sinus tract formations. 2 Treatment of PCAS is challenging and clinical practice varies a lot, while evidence-based treatment guidelines for PCAS are quite rare. Despite a paucity of robust and high-quality data on PCAS, this review focuses on different aspects of PCAS, especially on treatment options for PCAS, providing clinical inspirations for dermatologists. | CLINICAL MANIFESTATIONS AND HISTOPATHOLOGIC FEATURESLesions of PCAS are folliculitis at an early stage and becoming nodules and abscesses later, primarily on the occipital scalp or vertex, with symptoms of pain, itching or swelling. The nodules and abscesses rapidly develop into interconnecting, fluctuant, boggy sinus tracts, which discharge purulent and bloody secretions with a foul smell. With time, irreversible atrophic or hypertrophic scarring and cicatricial alopecia occur. PCAS presents as a chronic process with frequent episodes. 1,3 The histopathologic characteristics of PCAS usually depend on the clinical manifestation, which can range from early folliculitis, nodules or abscesses to sinus tracts or scars. In the early stage, histopathology findings show an infiltrate of lymphocytes and neutrophils involving the lower half of the dermis and gradually extended downward to subcutaneous fat. The disease progressed with vascular hyperplasia. In the late stage, the deep dermis and subcutaneous fat layer show the histologic characteristics of granuloma and eventually sinus tract and fibrosis develop. 4,5

Surgery combined with photodynamic therapy for the case of perifolliculitis capitis abscedens et suffodiens: A case report

“…At present, it is believed that this disease is related to hyperkeratosis of hair follicles and pilosebaceous unit occlusion, followed by dilatation and rupture of hair follicle ducts, and the release of its contents, such as keratin and bacteria, leading to the emergence of neutrophilic and granulomatous inflammatory reactions, the formation of abscesses, etc. 3 , 4 The pathological manifestations are characterized by folliculitis and perifollicular inflammation, accompanied by the infiltration of dense lymphocytes, histiocytes, and multinucleated cells, the formation of abscesses, and granulomatous inflammation in the deep dermis and subcutaneous adipose layer, etc., resulting in follicular sebaceous units and skin appendages damages, leads to a series of clinical manifestations such as nodules, cysts, sinus tracts, and fibrosis. 2 , 3 It can occur alone or as part of follicular occlusion tetrad, namely PCAS, acne conglobata, hidradenitis suppurativa, and pilonidal sinus.…”

“… 3 , 4 The pathological manifestations are characterized by folliculitis and perifollicular inflammation, accompanied by the infiltration of dense lymphocytes, histiocytes, and multinucleated cells, the formation of abscesses, and granulomatous inflammation in the deep dermis and subcutaneous adipose layer, etc., resulting in follicular sebaceous units and skin appendages damages, leads to a series of clinical manifestations such as nodules, cysts, sinus tracts, and fibrosis. 2 , 3 It can occur alone or as part of follicular occlusion tetrad, namely PCAS, acne conglobata, hidradenitis suppurativa, and pilonidal sinus. 8…”

“… 1 , 2 Unknown etiology, similar to hidradenitis suppurativa, acne conglobata, including follicular hyperkeratosis, follicular duct obstruction, dilation and rupture, bacterial overgrowth, followed by neutrophils, histiocytes, lymphocytes, plasma cell infiltration and granulomatous inflammatory response, scarring, sinus tract formation, and later fibrosis. 3 , 4 The clinical treatment is challenging, and the practice is quite different. The commonly used treatment methods include oral antibiotics, isotretinoin, hormones and other drugs, TNFα inhibitors, X-rays, lasers, and surgical resections, all of which have varying degrees of effect, but there are drug resistance, recurrence, or systemic side effects in all treatments.…”

Perifolliculitis capitis abscedens et suffodiens treatment with tumor necrosis factor inhibitors and baricitinib: A case report and literature review

“…To explore the potential of the anti-TNF antibody for treating PCAS, we presented the results of a case study and literature review related to an anti-TNF antibody administration to treat PCAS. However, a higher priority may be given to combination therapy because, when considering all of the available therapeutic options, a single treatment frequently has limited efficacy ( 5 ). The combined use of Janus kinase (JAK) inhibitors may potentially be a novel treatment scheme for PCAS.…”

Perifolliculitis capitis abscedens et suffodiens: features of diagnostics and approaches to treatment

“…Це захворювання має досить велику кількість синонімів: розсікаючий целюліт, абсцедуючий підривний фолікуліт, перифолікуліт Гоффмана, целюліт П'юзі, хвороба Гоффмана. Лікування перифолікуліту голови абсцедуючого є досить складним завданням, бо клінічна практика дуже різноманітна, що робить діагностичний процес непростим, а призначення найкращого та ефективного лікування цього захворювання є справжньою проблемою для лікаря, оскільки єдиного протоколу лікування на сьогоднішній день не існує [1].…”