Paraneoplastic pemphigus: a clinical, laboratorial, and therapeutic overview

Abstract: Paraneoplastic pemphigus is a rare and severe autoimmune blistering disease characterized by mucocutaneous lesions associated with benign and malignant neoplasms. Diagnostic criteria include the presence of chronic mucositis and polymorphic cutaneous lesions with occult or confirmed neoplasia; histopathological analysis exhibiting intraepidermal acantholysis, necrotic keratinocytes, and vacuolar interface dermatitis; direct immunofluorescence with intercellular deposits (IgG and C3) and at the basement membrane zone (IgG); indirect immunofluorescence with intercellular deposition of IgG (substrates: monkey esophagus and simple, columnar, and transitional epithelium); and, autoreactivity to desmogleins 1 and 3, desmocollins 1, 2, and 3, desmoplakins I and II, envoplakin, periplakin, epiplakin, plectin, BP230, and α-2-macroglobulin-like protein 1. Neoplasias frequently related to paraneoplastic pemphigus include chronic lymphocytic leukemia, non-Hodgkin lymphoma, carcinomas, Castleman disease, thymoma, and others. Currently, there is no standardized treatment for paraneoplastic pemphigus. Systemic corticosteroids, azathioprine, mycophenolate mofetil, cyclosporine, rituximab, cyclophosphamide, plasmapheresis, and intravenous immunoglobulin have been used, with variable outcomes. Reported survival rates in 1, 2, and 5 years are 49%, 41%, and 38%, respectively.

[Bazex syndrome or acropertosis paraneoplasitic-Review of the literatur]

Bazex syndrome or acrokeratosis paraneoplastica is a rare dermatosis usually associated with a squamous cell carcinoma of upper aero digestive tract or with metastases in the cervical lymph nodes. It presents as psoriasiform violacceous scaly patches over extremities, tip of the nose, earlobes and helices. This syndrome evolves in three stage parallel to the growth of the tumor, thus the utility of recognition of the syndrome which may unmask the associated neoplasia at an early stage. Treatment consists of treating the underlying neoplasia whether by surgery, chemotherapy and/or radiotherapy. Recurrence of cutaneous lesions may reveal the recurrence of the tumor

Hepatocellular carcinoma with persistent hypoglycemia: successful treatment with corticosteroid and frequent high carbohydrate intake.

The authors report a 36-year old man, who had hepatocellular carcinoma with lung metastasis and presented with coma from hypoglycemia. Serum insulin and c-peptide level were suppressed. Serum cortisol level was appropriately increased during the event. He needed glucose at least 3.65 mg/kg/min intravenously to maintain euglycemia. Cytoreduction of the tumor was not possible due to advanced stage disease. The patient had several episodes of morning hypoglycemia, despite having oral prednisolone 40 mg/day. However, glucose requirement was decreased after steroid usage. Normoglycemia was accomplished by adjunctive frequent high carbohydrate meal. Prednisolone was replaced to a lower dosage dexamethasone (2 mg/day). He did not have recurrent hypoglycemia as long as he had a midnight snack. Articles regarding hypoglycemia in HCC were extensively reviewed.

Hypercalcemia induced by parathyroid hormone-related peptide after treatment of squamous cell carcinoma of oral cavity.

Squamous cell carcinoma of the head and neck is a rare cause of humoral hypercalcemia of malignancy. This paraneoplastic syndrome is usually one of the presenting symptoms of the disease. We report a case of squamous cell carcinoma of the oral cavity that presumably elaborated parathyroid hormone-related peptide (PTH-rP) and caused hypercalcemia only after radiotherapy and chemotherapy.