1/643. Paraneoplastic pemphigus associated with follicular dendritic cell sarcoma arising from Castleman's tumor. Paraneoplastic pemphigus (PNP) is associated with various forms of underlying neoplasia. We report a patient with PNP associated with an intraabdominal follicular dendritic cell sarcoma that developed from a Castleman's tumor that had preexisted for 40 years. ( info) |
| BACKGROUND: Five children with pemphigus are reported: three with pemphigus vulgaris, one with pemphigus vegetans, and one with pemphigus foliaceus. Only one case of juvenile pemphigus vegetans has been published in the literature. MATERIALS AND methods: All three patients with pemphigus vulgaris were treated with oral corticosteroid; in two cases, azathioprine was added for steroid-sparing effect. The patient with pemphigus vegetans had a clinical presentation resembling pemphigus vulgaris, but the lesions in the perianal area healed as hypertrophic granulation tissue. He was treated with oral corticosteroid, azathioprine, and intralesional corticosteroid. The patient with pemphigus foliaceus presented with exfoliative dermatitis, and was treated with oral corticosteroid; methotrexate was added later for steroid-sparing purposes RESULTS: The patients were followed up for 1-4 years; the prognosis of childhood pemphigus is good. CONCLUSIONS: Long-term follow-up is needed to detect flaring of the disease and the side-effects of immunosuppressive drugs. ( info) |
3/643. Intercellular IgA dermatosis. We report three cases of intercellular IgA dermatosis (IAD) and review the literature. IAD is a spectrum of vesiculobullous or vesiculopustular diseases mediated by intercellular IgA deposition. The clinical picture may vary from a vesiculopustular eruption with centrifugal evolution mainly involving the trunk and extremities, to the typical picture of classic pemphigus variants (foliaceus, vegetans). Histologically, infiltrating polymorphonuclear cells (mainly neutrophils) are observed in the epidermis with formation of pustules and bullae at various levels. However cases with typical histological features of pemphigus (variants) are described. Direct immunofluorescence on perilesonal skin typically displays intercellular IgA deposition at different levels or throughout the epidermis and indirect immunofluorescence often detects low levels of circulating antibodies. The disease has been repeatedly reported in association with monoclonal IgA gammopathy. Most cases respond to dapsone. In some cases IgA is directed against known pemphigus antigens whereas their targets in other cases are newly discovered antigens (105 kD, 115 kD, 120 kD). We observe a heterogeneity within the clinical, histological and immunological characteristics of the disease. Many reported cases feature various combinations of these characteristics. We therefore consider IAD as a disease spectrum with IgA pemphigus (clinical and histological pemphigus) at one end and intercellular IgA vesiculopustular dermatosis at the other end. ( info) |
4/643. Scrape cytology of oral pemphigus. Report of a case with immunocytochemistry and light, scanning electron and transmission electron microscopy. BACKGROUND: pemphigus vulgaris is a disseminated disease of the skin and mucous membranes characterized by recurrent vesicular and bullous lesions due to the autoantigen belonging to the cadherin type of cell adhesion molecules. The presence of acantholysis associated with immunoglobulins in the intercellular spaces and on the cell membrane are diagnostic features. However, the appearance of smears from the oral cavity by scanning (SEM) and transmission electron microscopic (TEM) study as well as immunocytochemistry of cadherin does not appear to have been previously reported. CASE: A 67-year-old female developed erosion on her gingiva with severe pain. On oral examination, there were ulcerations on the palate, and the Nikolsky sign was positive. The characteristic cytologic findings from oral scrapes were high cellularity, a bloody background and a predominant cell population consisting of polygonal basal and parabasal cells with pronounced nucleoli. Also present were degenerative cell changes: e.g., cytoplasmic vacuoles and a homogeneous nuclear appearance. Immunocytochemical staining for IgG and cadherin gave a positive reaction in the intercellular spaces and on the cell membranes. The surface of cells in pemphigus vulgaris by SEM showed somewhat irregularly distributed microridges, and TEM revealed desmosomal attachments, degenerated tonofilaments with pronounced nucleoli and heterochromatin. As a result of cytodiagnosis, additional appropriate specimens were obtained at the time of the scraping for confirmatory immunocytochemistry for cadherin, SEM and TEM studies. CONCLUSION: The results demonstrate that a precise diagnosis of pemphigus vulgaris can be rendered on cellular material and cadherin immunocytochemistry obtained by scrape from the oral mucosa. ( info) |
5/643. mortality associated with concurrent strongyloidosis and cytomegalovirus infection in a patient on steroid therapy. Disseminated strongyloidosis has been recognized with increasing frequency, often in patients who are immunocompromised or have received steroid therapy. In addition, disease due to cytomegalovirus (CMV) is noted in immunodeficient hosts. We report on a 55-year-old Puerto Rican man who received steroid treatment for orpharyngeal pemphigus vulgaris and developed abdominal symptoms with alternating constipation and diarrhea. The clinical work-up did not reveal specific abnormalities, but the patient died of cardiopulmonary failure. At the postmortem examination, the patient had evidence of strongyloidosis and CMV disease. This report reviews both this case and the literature, and discusses the overlapping infections of strongyloidosis and CMV disease in this patient who had received steroid therapy. ( info) |
6/643. Pyodermatitis-pyostomatitis vegetans: report of a case and review of the literature. Pyodermatitis-pyostomatitis vegetans is a benign, rare disorder characterized by a pustular eruption in the oral mucosa and vegetating plaques involving the groin and axillary folds. Its association with inflammatory bowel disease is well established. We report the case of a 49-year-old-white man with ulcerative colitis who manifested a vegetating, annular plaque in the left inguinal region of 2 months' duration. Oral examination disclosed an erythematous mucosa with multiple painful pustules involving the labial and gingival mucosa. Histopathologic study demonstrated epidermal hyperplasia and an inflammatory infiltrate composed mostly of neutrophils and eosinophils, grouped into microabscesses within the epidermis and with a bandlike configuration in the upper dermis. Results of direct and indirect immunofluorescence studies were negative. We discuss the differential diagnosis between pyodermatitis-pyostomatitis vegetans and pemphigus vegetans. ( info) |
7/643. esophagitis dissecans superficialis associated with pemphigus vulgaris. The extension of bullous lesions in pemphigus to the esophagus is relatively uncommon, especially in patients who appear to be in clinical remission. Very rarely, pemphigus vulgaris may affect the entire esophagus, resulting in complete sloughing of the mucous membrane. A 20-year-old man with pemphigus vulgaris presented to the emergency room with acute onset of dysphagia, odynophagia, and hemoptysis. There were no cutaneous or oral findings of pemphigus on presentation, since he was being maintained on corticosteroids and azathioprine with excellent results. During initial evaluation in the emergency room, the patient was observed to vomit a cast of the mucosal lining of the esophagus. The morphologic description of such an esophageal cast is termed esophagitis dissecans superficialis. This is the third case of esophagitis dissecans superficialis in pemphigus vulgaris recorded in the medical literature. ( info) |
8/643. pemphigus vulgaris preceded by herpetiform-like skin lesions with negative immunofluorescence findings. We report a case of pemphigus vulgaris in a 63-year-old woman. At the first onset, she developed herpetiform-like skin lesions. Their histology showed eosinophilic spongiosis and intraepidermal blister formation with an infiltrate of eosinophils. Both direct and indirect immunofluorescence studies were negative. She remained in remission for 6 years. At the second onset, she developed bullous skin lesions; histological examination disclosed suprabasal acantholysis and infiltration of eosinophils. Direct immunofluorescence revealed IgG and C3 deposits at the cell surface, predominantly in the lower epidermis. Indirect immunofluorescence using normal human skin as a substrate demonstrated anti-cell surface IgG antibodies at a titer of 1:160. Immunoblot analysis showed that the patient's serum reacted only with a 130 kD protein. This case indicates that pemphigus vulgaris can follow herpetiform-like skin lesions with negative immunofluorescence findings after a long remission time. ( info) |
9/643. Paraneoplastic pemphigus with fatal pulmonary involvement in a woman with a mesenteric Castleman tumour. A 42-year-old woman presented with oral and labial erosions, conjunctivitis, facial rash and lichenoid erythematous papules on the trunk. Paraneoplastic pemphigus (PNP) was suspected, and a search for a neoplasm revealed an intra-abdominal Castleman tumour sized 7 x 5 x 6 cm. After removal of the Castleman tumour, the skin and mucosal inflammation gradually subsided over the next 12 months. However, due to irreversible pulmonary involvement the patient died of intractable respiratory distress 2 years after the onset of the disease. Systemic corticosteroids, azathioprine, cyclophosphamide, high-dose intravenous immunoglobulins and thalidomide were ineffective. The diagnosis of PNP was confirmed by keratinocyte antigen immunoprecipitation with the patient's serum. ( info) |
10/643. Three different autoimmune bullous diseases in one family: is there a common genetic base? We report an unusual familial occurrence of autoimmune bullous diseases. Three members of a family suffered from three different autoimmune bullous diseases: pemphigus vulgaris (PV), linear IgA disease (LAD) and cicatricial pemphigoid (CP). The HLA type was determined in five family members: all were positive for HLA-DQ5/DR6, which is reported to be associated with susceptibility to PV. The CP patient was DQ7(3) positive, which is in concordance with enhanced susceptibility to ocular CP and CP. The LAD patient was B8 and DR3 negative but positive for HLA-A1. Our study supports the hypothesis that there is a genetically transmitted susceptibility to autoimmune bullous diseases but that additional factors seem necessary actually to develop a particular disease. ( info) |