{
  "abstract": "A middle-aged male from East India presented with 2 months of oral ulcers, eyelid and lip erosions and difficulty in swallowing ( figure 1A). On examination of the skin, he was found to have thick, adherent, crusted erosions over his lips, eyelids and in the oral cavity, as well as fluid-filled vesicles and bullae on his hands and trunk (figure 1B). The possible differentials were Steven-Johnson syndrome, Erythema multiforme and Pemphigus vulgaris. The patient was noted to have a persistent dry cough in the hospital, for which a chest radiograph was taken, which showed mediastinal widening. CT chest revealed a contrast-enhancing right paratracheal mass (figure 1C). It was identified as adenocarcinoma through an endobronchial ultrasound-guided trans-bronchial needle aspiration (figure 1D). However, the samples were inadequate for immunohistochemistry and mutational analysis due to a lack of rapid onsite evaluation (ROSE). Skin biopsy showed a full-thickness epidermal necrosis and apoptotic keratinocytes with periadnexal lymphocytic infiltrates in the dermis and a negative direct immunofluorescence to IgG, IgA and C3. After a multidisciplinary discussion with the pathologist, dermatologist, pulmonologist and medical and surgical oncologists, with the above clinical picture and skin biopsy, the patient was diagnosed with paraneoplastic pemphigus (PP). Skin lesions progressed to involve most of the skin and mucosa, even with 2 weeks of cyclosporine. Hence, three doses of Inj. Rituximab (500 mg) and four doses of intravenous immunoglobulin (IVIG 2 g per kg) were administered with oral prednisolone, to which he responded. Metastatic workup was negative on FDG-PET, prompting immediate systemic chemotherapy. Due to its proximity to mediastinal structures, chemotherapy was started with carboplatin and etoposide, leading to improvement in skin and mucosal lesions. The patient declined further plans for a biopsy for personal reasons. Two months after discharge, he remained clinically stable with healed skin lesions (figure 1E,F), on a tapering dose of prednisolone and tolerating chemotherapy.",
  "authors": [
    {
      "affiliations": [
        "Pulmonary Medicine, Jawaharlal Institute of Postgraduate Medical Education and Research, Pondicherry, Puducherry, India"
      ],
      "name": "Zeenathalam Nadaf"
    },
    {
      "affiliations": [
        "Pulmonary Medicine, Jawaharlal Institute of Postgraduate Medical Education and Research, Pondicherry, Puducherry, India"
      ],
      "name": "Pratap Upadhya"
    }
  ],
  "title": "Lung adenocarcinoma presenting as paraneoplastic pemphigus",
  "uid": "4a31ce4f-8ffe-5842-a9e4-f1d1f0eefb23"
}