Extramedullary myeloma in an HIV-seropositive subject. Literature review and report of an unusual case

Myeloma is characterized by monoclonal bone marrow plasmacytosis, the presence of M-protein in serum and/or in urine and osteolytic bone lesions. HIV-seropositive subjects with myeloma are younger at the time of diagnosis of the tumour and usually the myeloma has a more aggressive clinical course than it does in HIV-seronegative subjects

Normal expression of the 19-DEJ-1 epitope in two siblings with late-onset junctional epidermolysis bullosa

We describe two siblings with late-onset junctional epidermolysis bullosa (JEB) (formerly called epidermolysis junctionalis progressiva). This is a subtype of autosomal recessive JEB characterized by late onset of the symptoms, between the ages of 5 and 8 years. The symptoms are mechanobullous lesions preferentially situated on hands and feet, nail dystrophy, loss of dermatoglyphic pattern, tooth enamel abnormalities and hyperhidrosis. In most forms of JEB a reduction or absence of a specific hemidesmosomal component can be demonstrated by means of immunohistochemical analysis. In this family, all known involved hemidesmosomal components, including uncein, recognized by the monoclonal antibody 19-DEJ-1, appeared to be normally expressed

Normal expression of the 19-DEJ-1 epitope in two siblings with late-onset junctional epidermolysis bullosa

We describe two siblings with late-onset junctional epidermolysis bullosa (JEB) (formerly called epidermolysis junctionalis progressiva). This is a subtype of autosomal recessive JEB characterized by late onset of the symptoms, between the ages of 5 and 8 years. The symptoms are mechanobullous lesions preferentially situated on hands and feet, nail dystrophy, loss of dermatoglyphic pattern, tooth enamel abnormalities and hyperhidrosis. In most forms of JEB a reduction or absence of a specific hemidesmosomal component can be demonstrated by means of immunohistochemical analysis. In this family, all known involved hemidesmosomal components, including uncein, recognized by the monoclonal antibody 19-DEJ-1, appeared to be normally expressed

Paraneoplastic pemphigus as the presenting symptom of a lymphoma of the tongue

A patient is described who initially presented with an acrovesicular eczema which subsequently developed into erythema multiforme with histopathological features of bullous pemphigoid. Although the various laboratory studies pointed to the diagnosis of paraneoplastic pemphigus (PNP), the underlying neoplasm was not detected until 6 months later, when the biopsies of an oral lesion showed the presence of an underlying non-Hodgkin lymphoma. (C) 1998 Elsevier Science Ltd. All rights reserved

Linear IgA bullous dermatosis in a patient with renal cell carcinoma

Linear IgA bullous dermatosis (LABD) is an autoimmune subepidermal bullous disease with heterogeneous clinical manifestations, characterized by linear deposition of IgA along the epidermal basement membrane zone. We report a patient with a metastasized renal cell carcinoma who developed an extensive blistering eruption. The lesions showed immunopathological findings characteristic of LABD. The patient showed a fair response to prednisolone and dapsone. Treatment to control the LABD was no longer required when interferon-alfa was started as palliative therapy for the metastasized renal cell carcinoma. The association of LABD and malignancies has been documented before and is not due to mere chance alone