Perforating folliculitis with jaundice in an Indian male: a rare case with sclerosing cholangitis

Sir, A 40‐year‐old Indian male, presented with multiple, raised, severely itchy, pea‐sized lesions present all over the body for the last 6 months. There was a history of extrusion of dirty grey material from the lesions on scratching. There was no improvement with topical and systemic medications (nature unknown) taken previously. The patient reported passage of yellow‐coloured urine, loss of appetite and malaise for the last 5 months. There was no history suggestive of diabetes mellitus, chronic renal disease, atopy, exposure to lubricants or industrial solvents or of alcohol intake. The general physical examination revealed marked icterus. On cutaneous examination, there were multiple, hyperkeratotic, skin‐coloured to hyperpigmented, follicular papules of size 0·3–0·8 cm along with a few prurigo nodularis‐like lesions, present mainly on the extremities (Fig. 1). Other sites involved were the buttocks, trunk, face, nape of neck and occiput. The lesions showed a central keratotic plug, the removal of which showed a shallow, bleeding crater. Systemic examination revealed a nontender, soft hepatomegaly 3 cm below the costal margin. His complete haemogram was within normal limits. Erythrocyte sedimentation rate was 23 mm in the first hour. Liver function tests were deranged, with total bilirubin 5·6 mg dL−1 (normal, 0·3–1·0 mg dL−1), conjugated bilirubin 4·8 mg dL−1 (normal, 0·1–0·3 mg dL−1), alanine aminotransferase 168 IU mL−1 (normal, 0–35 IU mL−1), antileucoproteinase 1435 IU mL−1 (normal, 30–120 IU mL−1). Chest X‐ray, kidney function tests and blood glucose levels were normal. Hepatitis B surface antigen and human immunodeficiency virus–enzyme‐linked immunosorbent assay were negative. Grey‐scale B‐mode ultrasonogram of the abdomen revealed a stricture of the common bile duct (CBD) with proximal dilatation of the hepatobiliary tract and was suspected to be a cholangiocarcinoma. Histopathological examination of the skin lesions (papules and nodules) showed hyperkeratotic and acanthotic epidermis with a dilated hair follicle in the superficial dermis showing a keratinous plug with disruption of the follicular epithelium. Degenerate dermal material was seen entering the perforated follicle along with mild inflammatory infiltrate in the surrounding dermis. A hair fragment was also visualized in the keratinous debris (Fig. 2) and Verhoeff–van Gieson stain and Fontana–Masson stain were both negative, confirming the clinical diagnosis of perforating folliculitis. The patient was taken up for laparotomy, which revealed a cord‐like CBD. An operative cholangiography revealed features of predominantly intrahepatic ductal disease with short multiple strictures associated with multifocal mild ductal dilations and no calculi. As it is often difficult to differentiate sclerosing cholangitis from cholangiocarcinoma by radiography, resection with careful pathological examination of the entire specimen may be necessary to establish a final diagnosis. A surgical decision to excise the stricture was taken and concomitantly the surgeons performed a Roux‐en‐Y cholecystojejunostomy. Histopathology of the surgical specimen revealed degeneration of the epithelial cells lining the bile ducts associated with diffuse thickening of the bile duct with luminal narrowing with no evidence of malignancy. Based on the clinical presentation, sonographic evaluation and consequent histopathology of the bile ducts, a diagnosis of sclerosing cholangitis was concluded. Within 15 days of the surgery, the patient's skin lesions started resolving and the patient was free of cutaneous lesions in a month. Both the deranged liver function tests and generalized pruritus gradually normalized over a period of 3 months. Regular clinical and radiological follow‐up for 1 year have been uneventful.