A rare case of toxic epidermal necrolysis (TEN) like presentation of bullous pemphigoid with coexistent esophageal candidiasis in an elderly man

Sir An 82 year old man with no known comorbidities presented with multiple fluid filled lesions rupturing to form raw areas involving the entire body of ten days duration. The patient gave history of recurrent episodes of multiple red raised itchy lesions followed by development of fluid filled lesions since the last two years, for which he was on topical and oral medications off and on. He presented to us with a flare of lesions since ten days. Examination revealed polysized tense bullae asymmetrically distributed over both upper and lower limbs and crusted erosions involving more than 90% body surface area over the trunk, face, scalp, upper and lower limbs [Figures 1 and 2]. Nikolsky sign was negative. Oral and anogenital mucosa was normal.Figure 1:: Polysized tense bullae and crusted erosions involving more than 90% body surface area over the trunk, face, scalp, upper and lower limbsFigure 2:: (AandB)- Polysized tense bullae and crusted erosions over the lower limbsEvaluation revealed low Hemoglobin-6.5 gm% with a microcytic hypochromic anemia picture. Skin biopsy revealed epidermis detached from dermis at subepithelial plane. Dermis showed perivascular lymphocytic infiltration admixed with eosinophils suggestive of bullous pemphigoid [Figure 3A]. Direct immunofluorescence (DIF) of perilesional skin showed IgG and C3 deposited in a granular pattern along basement membrane [Figure 3B].Figure 3:: A- (H and E 400x) Skin biopsy revealed epidermis detached from dermis at subepithelial plane with the dermis showing perivascular lymphocytic infiltration admixed with eosinophils. B- Direct immunofluorescence (DIF) of perilesional skin showed IgG deposited in a granular pattern along basement membraneIndividual was diagnosed as a case of bullous pemphigoid on the basis of clinical and histopathological evidence and was started on tapering course of oral prednisolone (1 mg/kg) and adjuvant azathioprine (3 mg/kg). He was managed in an intensive care setting with reverse barrier nursing on account of extensive erosions over body mimicking toxic epidermal necrosis. He was transfused two packed red blood cell transfusion for iron deficiency anemia. Despite therapy patient continued to get new lesions in form of tense bullae. He also complained of difficulty in swallowing, reflux symptoms, bloating sensation in the abdomen and constipation. In view of the advanced age, a malignancy screening was done and was found to have elevated carcinoembryonic antigen (CEA-8.64). He was evaluated by oncophysician and was advised positron emission tomography (PET) scan which revealed diffuse uptake by oesophagus which was significant [Figure 4]. A gastroenterology (GE) consult was taken and upper gastrointestinal endoscopy (UGIE) was performed which showed features of extensive eosophageal Candidiasis. Esophageal biopsy was consistent with esophageal Candidiasis [Figure 5]. Patient was started on oral fluconazole therapy and following that the cutaneous lesions responded well to the immunosuppressant therapy in form of resolution of erythema, erosions and bullous lesions. He was discharged on azathioprine, tapering course of prednisolone and supportive therapy.Figure 4:: Positron emission tomography (PET) scan revealed diffuse significant uptake by the oesophagusFigure 5:: (400x PAS) stain highlights the numerous yeasts and pseudohyphal forms of fungiBullous pemphigoid (BP) is a common autoimmune bullous disease.[1] It is a subepidermal bullous disorder and is characterised by the production of autoantibodies directed against the hemi-desmosomal anchoring proteins BP180 and BP230.[2] BP generally presents with tense, pruritic cutaneous blisters and mostly affects the elderly.[1] There might be atypical presentations in the form of polycyclic, targetoid, vesicular, nodular, lichenoid lesions or even erythroderma.[3] Infrequently, it can mimic toxic epidermal necrolysis (TEN) at presentation as was seen in our case. This is due to confluence of bullae leading to large areas of epidermal loss giving the appearance of TEN. A case of BP secondary to pembrolizumab mimicking TEN was reported by Qui C et al.[4] Other bullous disorders like paraneoplastic pemphigus and linear IgA bullous disease are also known to present like TEN.[5,6] In our patient the history of pruritus, presence of tense bullae, absence of mucosal involvement and the characteristic histopathological and DIF findings supported a diagnosis of BP. However, persistent disease activity in the form of continued blistering and erosions of skin as well as gastrointestinal (GI) symptoms and the findings of superadded Candidiasis on upper GI endoscopy was a significant turning point in the overall management of the case as treatment of Candidiasis lead to improvement in quality of life of the patient and better acceptance of immunosuppressant therapy, thereby faster healing of cutaneous lesions. Our case is unique as TEN-like BP is a rare condition. The authors also want to impress that esophageal involvement in cases of BP needs to be evaluated in cases not responding to treatment. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.