Perifolliculitis Capitis Abscedens et Suffodiens in a 7 Years Male: A Case Report with Review of Literature.

Perifolliculitis capitis abscedens et suffodiens is the least common of the three conditions included in the follicular occlusion triad. It is one of the causes of scarring alopecia in adult males. Clinically it has to be differentiated from other causes of folliculitis affecting the scalp. The histopathology is diagnostic. The treatment is prolonged and the clinical course is characterized by remissions and relapses.

INTRODUCTION

Perifolliculitis capitis abscedens et suffodiens (PCAS) also known as dissecting cellulitis is one of the three diseases included in follicular occlusion triad, the other two being hidradenitis suppurativa, and acne conglobata. PCAS is the least common amongst the three. PCAS is found worldwide. In US, it is predominantly found in African American men between 20 and 40 years.[1] We present a case affecting a male child of 7 years with a brief review of the literature.

CASE REPORT

A 7-year-old male child was admitted to the dermatology ward with complaints of painful discharging nodular lesions on scalp accompanied with localized hair loss since 2 months. On examination, there were multiple patches of alopecia where painful and itchy, hyperpigmented plaques ranging in size from 2.5 cm to 9 cm were seen. There were small pustules on the plaques, and some of them were discharging pus. Cervical and nuchal lymph nodes were not palpable. A clinical diagnosis of inflammatory tinea was made. The erythrocyte sedimentation rate was 25 mm at the end of 1 h (Wintrobe method). The hemogram revealed a raised total leukocyte count of 14,000/cmm with polymorphonuclear leukocytosis of 78%. Other investigations such as urine, blood sugar level, renal function tests, and liver function tests were within normal limits. A potassium hydroxide mount of the scrapings from the skin in the affected area did not show any fungal elements. No fungi were grown in the fungal culture of pus either. The pus culture showed growth of Klebsiella pneumonie. Antibiotic novaclox was prescribed orally with T-Bact cream locally. After 15 days, antifungal drug griseofulvin and prednisolone were added. The pustules and pus discharge subsided, but the plaques remained after 1½ months of treatment [Figure 1]. A skin biopsy was performed. The epidermis was mildly acanthotic. There was follicular hyperkeratosis, plugging, and dilatation. There was perivascular, and perifollicular lymphoplasmacytic infiltrate in the dermis. The deeper dermis and subcutaneous tissue showed intense granulomatous inflammation around naked hair shafts consisting of lymphocytes, plasma cells, epithelioid cells, and multinucleate foreign body type of giant cells [Figure 2]. A histopathological diagnosis of PCAS was made. A 15 days course of ciprofloxacin 250 mg BD was prescribed. There was a gradual resolution of the plaques. The family has since moved to another town, and telephonic contact has elicited the information that the child's symptoms have subsided, and there is some growth of hair in affected areas although the density is very less.

Figure 1

Alopecia and residual edematous plaques after 1½ months of antibiotic treatment

Figure 2

Skin biopsy showing follicular keratosis and plugging, perifollicular chronic inflammation, deep dermal inflammatory reaction with inset of naked hair shafts surrounded by foreign body giant cells (H and E, ×40, inset, ×100)

DISCUSSION

The follicular occlusion triad shows the same histologic changes and shares a common pathogenesis where the initial event is follicular hyperkeratosis and plugging leading to retention of follicular products. They differ in the clinical presentation, with PCAS affecting the scalp, hidradenitis suppurativa affecting axillary and anogenital regions and acne conglobata affecting back, buttocks, and chest. More than one disease may be encountered in a single patient.[2] Clinically PCAS is a chronic, recurrent folliculitis of scalp ending in abscesses and sinus tracts that lead to scarring and alopecia.[2] It is usually seen in young adult males and has been reported in African Americans and Caucasians.[1] Clinical differential diagnosis of PCAS includes several conditions that affect scalp such as acne keloidalis nuchae, pseudopalade of Brocq, inflammatory tinea capitis (kerion), folliculitis decalvans and tufted folliculitis.[1] Acne keloidalis nuchae does not show fluctuant nodules, sinus tracts or severe alopecia, which distinguishes it from PCAS. Pseudopalade of Brocq shows epidermal atrophy and “footprints in the snow” alopecia which are not a feature of PCAS. Kerion is characterized by regional lymphadenopathy and positive fungal culture which are not found in PCAS. Folliculitis decalvans shows central scarred patches of alopecia without pustules and sinuses which are characteristic of PCAS. Tufted folliculitis heals with scarring alopecia showing within it multiple tufts of hair emerging from dilated follicular orifices which distinguish it from PCAS. Other conditions that are reported to mimic PCAS includes lichen planopilaris, malignant proliferating pilar cysts, folliculotropic mycosis fungoides with large cell transformation, cutis verticis gyrate, erosive pustular dermatosis of the scalp.[1] Alopecic and aseptic nodules of the scalp also called pseudocyst of the scalp is a condition characterized clinically by nonscarring alopecia and a small number of nodules with a cysic appearance. It has been reported mostly in Japan and France. Histopathologically it shows multinucleated cells admixed with neutrophils, lymphocytes, and histiocytes, but with absence of fibrosis.[1]

Histopathology of PCAS is characteristic. In the early stage, follicular hyperkeratosis with plugging and dilatation of the follicle is seen. The follicular epithelium may be hyperplastic or may be destroyed. Later perifolliculitis is seen with inflammatory infiltrate composed of neutrophils, lymphocytes, and histiocytes. Abscess formation leading to the destruction of pilosebaceous structures and of other adenexa is seen. Granulation tissue containing lymphoplasmacytic infiltrate and histiocytes along with multinucleate foreign body giant cells around keratin and hair shafts is seen in the deep dermis and subcutis. Extensive fibrosis occurs in healing areas.[2] Pigmented casts are reported in PCAS.[3]

Perifolliculitis capitis abscedens et suffodiens is a chronic condition with remissions and relapses. Healed lesions sometimes progress to hypertrophic scar or keloid.[4] Rarely complications like osteomyelitis of the underlying cranial bones and development of squamous cell carcinoma are reported.[4]

The abscesses of PCAS are usually sterile. A bacterial infection is a secondary event in PCAS. Organisms cultured in reported cases include Pseudomonas species,[56] coagulase negative Staphylococcus epidermidis,[7] Prevotella intermedia, Peptostreptococcus asaccharolyticus,[8] and Propionibacterium acnes.[9] A variety of antibiotics are used for the treatment of PCAS either alone or in combination with other drugs, and there are reports of remissions. Their effectiveness may be due to antibacterial and/or anti-inflammatory mechanisms.

There are studies in the literature which report the trichoscopic findings of PCAS. Black dots which are macrocomedo-like round structures located in the follicular osteum, seen trichoscopically have been reported in patients of PCAS.[10] One report states that “three-dimensional” yellow dots imposed over the dystrophic hair, large and yellow amorphous areas and pinpoint white dots with a whitish halo are characteristic trichoscopic findings of PCAS.[11]

Perifolliculitis capitis abscedens et suffodiens can be associated with other components of the follicular occlusion triad/tetrad.[2] Other skin conditions such as pyoderma vegetans, pityriasis rubra pilaris, and pyoderma gangrenosum have been reported with PCAS.[121314] While systemic conditions such as arthropathy, arthritis, sternocostoclavicular hyperostosis, keratitis, and keratitis-ichthyosis-deafness syndrome are reported to be associated with PCAS.[415]

The exact follicular defect in PCAS is not known, but there is agreement on the role of follicular occlusion. A role for the immune system is suggested by the reports of remissions induced by steroid treatment. A report of familial PCAS was published by Bjellerup and Wallengren in 1990.[16]

Scheinfeld in a review article has reported in detail on the various treatment options now available for patients of PCAS. They stated that the preferred options for first-line treatment are (1) Ciprofloxacin 250 to 500 mg BD and (2) Clindamycin and rifampin 300 mg each BD. Isotrentinoin 0.5-1 mg/kg for 3-12 months is the second line of treatment they recommend. Drugs like Adalimumab, Infliximab, and Alitrentinoin, are used as a third line of treatment. Surgery, radiation and CO2 laser ablation of the scalp are the last resort treatments.[1]

The present case is unique because of its childhood presentation (7 years). The youngest reported patient in literature is 15-year-old at presentation.[5]

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Conflicts of interest

There are no conflicts of interest