A case of Paraneoplastic Pemphigus associated with Castleman's\ndisease
A 49-year-old man presented with oral erosions, erythema and blistering rashes over his\ntrunk and limbs for 3 months. The patient first developed painful oral mucosal blisters\nand ulcers, which became progressively severe and gradually spread to the eyes, perineum\nand anus. The patient was admitted to our department for further diagnosis and\ntreatment. On physical examination, we found several small lymph nodes on his neck with\ndiameters of under 1 cm. Dermatologic examination revealed hyperemia on the bilateral\nconjunctiva, extensive ulcers and erosions on the oral mucosa, erythema and blisters\nscattered on the chest, back, perineum and anus, but with no obvious ulcers ( and ).\nOne week prior to admission, the patient had attended another hospital where\nhistological examination, serum direct immunofluorescence (DIF) and indirect\nimmunofluorescence (IIF) were performed. Histological examination of a skin biopsy taken\nfrom a lesion on his left arm showed epidermal acantholysis, suprabasal cleft formation,\nerythrocyte extravasation and vacuolar changes at the dermal epidermal junction and\nlymphohistiocytic infiltrate in the upper dermis (). DIF revealed IgG, IgM, and C3 deposit in the basement membrane zone (BMZ).\nIIF revealed IgG reticular staining with an antibody titer of 1:20. CT revealed a\nprominent solid lesion in the right anterior superior mediastinum ().\nThe results of laboratory tests, including full blood count, urea, creatinine,\nelectrolytes, liver function, tumor markers and tests for infectious diseases were\nnormal. Percutaneous mediastinum biopsy guided by CT revealed lymph hematopoietic\nhyperplasia. Immunohistological examination revealed CD20++, Pax-5++, CD43++, CD3+,\nCD163+, CD68 scattered+, Mum-1-, CD30-, CD10-, EMA-, CKpan-, TdT-, Ki673%+, CD5\nscattered +.\nPCR analysis of Ig heavy chain genes, gene clonal rearrangement (IGH, IGK, IGL,\nTCRβ, TCRγ, TCRδ) was detected by using\nBIOMED-2 protocols. DNA extraction from formalin-fixed tissues was performed using\nstandard methods. All the results were negative ( and ).\nDiagnosis: 1. Paraneoplastic Pemphigus 2. Castleman's Disease.\nUpon hospitalization, the patient was given an intravenous methylprednisolone injection\n60mg per day and other supportive therapy. A few days later, the lesions started to dry\nup and form crusts, except for the oral mucosa. According to the advice from the\nCardiothoracic Surgery Department, the lesion in the right anterior superior mediastinum\nwas too large to be completely resected, since abundant blood vessels were present and\nit was too close to the pulmonary artery. In any case, since the patient rejected\nsurgery, conservative treatment was recommended. Considering that the erythema and\ncrusting on the patient's chest and back could perhaps further aggravate after\nradiotherapy, the Radiotherapy Department attempted to use stereotactic radiosurgery,\nwith an irradiated DT:32Gy/2fx and isodose line 80%. After the end of treatment, the\npatient's lesions aggravated and new erythema and blisters appeared. However, these\nrecovered during the following week, and no new lesions manifested during the short\nfollow-up period.