Paraneoplastic Pemphigus Presenting as Toxic Epidermal Necrolysis: A Case Report

Jignaben K Padhiyar,Mansi Buch,Kishan Ninama,Rashmi Mahajan,Freny E Bilimoria,Nayankumar H Patel,Trusha Gajjar

doi:10.3126/njdvl.v16i1.19416

Abstract

Polymorphous skin lesions have classically been described in paraneoplastic pemphigus (PNP), but it can present as toxic epidermal necrolysis (TEN) though this type of presentation is extremely rare. We report a case of PNP presenting as TEN in a young male patient. Patient had history of fever and diarrhoea six weeks before starting of lesions in oral cavity, for which he was treated with injectable medicines. Then patient developed generalized necrosis and peeling of skin with involvement of conjunctiva, oropharynx and genital mucosa. For this, the patient was given intravenous dexamethasone considering it as TEN, but after transient improvement initially skin lesions recurred when dose of dexamethasone was reduced. On seventh day, patient developed few circular deep ulcers over arms and back. Nikolsky sign was positive with tzanck smear showing acantholytic cells. Hence, we added PNP as one of the differential diagnosis. On further investigations patient was found to have B cell lymphoma in mediastinum and skin biopsy and direct immunofluorescence were confirmative of PNP. Unfortunately, patient then succumbed to death due to multiorgan failure and electrolyte imbalance. The onset of PNP can be as acute as TEN and clinical picture being initially undistinguishable, high index of suspicion is required in diagnosis.

Highlights

Paraneoplas c pemphigus (PNP) or paraneoplas c autoimmune mul organ syndrome (PAMS) is characterized clinically by polymorphic skin lesions with neoplasia
The dis nc ve feature of PNP is presence of dyskerato c kera nocytes in all layers of epidermis and around zone of acantholysis
Though immunoprecipita on is highly sensi ve, direct immunofluorescence (DIF) showing combina on of intercellular and subepidermal deposi on of immunoreactants is a clue to diagnosis

Summary

Introduction

Paraneoplas c pemphigus (PNP) or paraneoplas c autoimmune mul organ syndrome (PAMS) is characterized clinically by polymorphic skin lesions with neoplasia. A 26-year-old male pa ent was referred to department of dermatology from another ter ary care hospital. Evalua on of case record of referring ter ary care hospital revealed that pa ent was treated with intravenous 12 mg dexamethasone along with other suppor ve therapy. We con nued pa ent’s management with injectable 12 mg of dexamethasone, intravenous fluid replacement, broad spectrum an bio cs keeping TEN as our primary differen al diagnosis. Skin biopsy showed dyskerato c kera nocytes, suprabasal cle s with loss of intercellular junc ons between suprabaslal kera nocytes and mixed inflammatory infiltrate in superficial epidermis. DIF showed IgG posi vity in intercellular space in epidermis and at basement membrane zone (Figure 3). Immunohistochemistry of same showed posi vity for LCA and CD-20 sugges ng B cell lymphoma (Figure 4). Pa ent succumbed to death due to metabolic acidosis (pCO2: 27.9, HCO3: 18.2, Lac c acid: 3.02) and mul -organ failure on 13th day of admission

Discussion

Conclusion