Journal of The Association of Physicians of India ■ Vol. 65 ■ May 2017 98 Bullous Pemphigoid as a Harbinger of Metastatic Cholangiocarcinoma - A Rarity Sumeet Prakash Mirgh 1 , Yash B Jobanputra 2 , Purva Sharma 2 , Vikas A Mishra 3 , Virti D Shah 4 , Akhilesh Sharma 5 Abstract Bullous pemphigoid (BP) is an autoimmune blistering disorder of the skin first described in 1953. A decade later, antibodies were described targeting the cutaneous basement membrane zone. The association of Bullous pemphigoid with malignancy is debatable 1 but reported in many case reports. 2-6 We report a 79 year old male with cholangiocarcinoma that presented with bullous pemphigoid as a paraneoplastic phenomenon. 1 Clinical Hematology Trainee 1st Year, AIIMS, New Delhi; 2 R.C.S.M.G.M.C., Kolhapur, Maharashtra; 3 Trainee, G.S.V.M. Medical College, Kanpur, Uttar Pradesh; 4 Trainee, Sir Gangaram Hospital, New Delhi; 5 Bombay Hospital Institute of Medical Sciences, Mumbai, Maharashtra Received: 23.05.2016; Revised: 08.10.2016; Accepted: 03.01.2017 Introduction M alignancies have typically been associated with paraneoplastic pemphigus. However, the association of bullous pemphigoid and malignant diseases has always been a matter of debate. There have been scattered reports of BP with malignancies such as parotid carcinoma, 2 breast cancer, 3 lung cancer, 4 squamous cell cancer of the groin, 5 B-cell lymphoma 6 and some others. To the best of our knowledge, this is the first case report of BP with cholangiocarcinoma. Bullous pemphigoid (BP) and pemphigus, although autoimmune, should prompt a search for underlying malignancy especially if (a) there is a poor response to standard therapy, (b) it is recurrent 5 or (c) it is associated with gyrate erythema. 1 Although they respond to steroids, surgical excision of tumour is necessary for cure. Similarly, the presence of ultrasound visualised abscesses without fever, palpable hepatomegaly, presence of cirrhotic changes, past or concurrent BP lesions should prompt a search for an underlying hepato-biliary malignancy. It is important that clinicians be cognizant of this association of BP with malignancy as timely treatment can help prolong life. Case A 79 year old male patient, who was a known diabetic and hypertensive, presented with abrupt onset vesiculo- bullous eruptions on both lower limbs for two weeks. It started with a large (approximately 1.5 by 1.5 cm diameter) hyperpigmented lesion on the outer border of left sole followed by multiple crops of similar lesions centrifugally over dorsum of left foot. Subsequently, a crop of tense vesicles and bullae appeared over the dorsum of right foot and volar aspect of both wrists in next 48 hours. The lesions were hyperpigmented, non-pruritic and mainly distributed over the extensor areas of extremities (Figure 1). He also complained of bilateral knee pain without swelling, nasal dryness and excessive lacrimation from both the eyes but no redness, irritation or eye pain. This was associated with anorexia and weight loss over a period of two weeks. On examination, the patient was hemodynamically stable with a pulse rate of 92 per minute and blood pressure of 126/80 mm Hg. General examination revealed mild pallor, but no lymphadenopathy, icterus, edema or clubbing. On local examination of left foot, bullae were large, tense and non- tender on a base of normal appearing skin. Nikolsky sign and bulla spread sign were negative. Ophthalmologic evaluation revealed subconjunctival scarring. Oral cavity examination revealed multiple small erythematous follicles over the soft palate. Systemic examination revealed mild non tender hepatomegaly and mild splenomegaly. Rest of the systemic examination was normal. He was investigated as shown in Table 1 below. Investigations revealed anemia of chronic disease (normocytic normochromic picture, normal total iron binding capacity and normal ferritin on iron studies) with evidence of pre-renal acute kidney injury (increased creatinine, hyponatremia with low urinary sodium) on presumed underlying CKD (history of diabetes mellitus, hypertension and previous baseline creatinine report of 1.2 mg/dl). Creatinine clearance as per Cockgroft Gault equation was 41 suggestive of stage 3 CKD. Liver function tests revealed mild transaminitis with cholestatic jaundice (elevated GGT with direct bilirubin >15 %) and decreased albumin with prolonged prothrombin time (suggestive of long standing liver pathology). Abdominal ultrasound revealed hepatomegaly (liver span 17 cm) with coarse echotexture and multiple hypoechoic lesions in right lobe of liver the largest of which measured 3.6 by 3.5 cm suggestive of non- liquefied abscess. In view of multiple abscesses in liver, IHA. Amoebiasis titre (Indirect Haemagglutination assay) was done, which was high (1:320) and patient was started on intravenous metronidazole. Biopsy of patient’s bullous lesions was planned. A tzanck smear from the vesicle revealed abundant neutrophils with few eosinophils and no acantholytic cells. Biopsy revealed subepidermal blistering dermatitis with a diagnosis of bullous pemphigoid (Figure 2). Direct Immunofluorescence revealed linear IgG/C3 deposition at dermo- epidermal junction. However, anti-BP 180 and 230 ELISA antibodies were not done in view of financial constraints. He was started on oral steroids Tab.