1/4. Successful treatment of adolescent pemphigus vulgaris by immunoadsorption method.A 15-year-old girl with pemphigus vulgaris did not respond to oral administration of prednisolone at 45 mg/day. The skin and oral mucous membrane lesions recurred after effective treatments with methylprednisolone pulse therapy and combination therapy with prednisolone and cyclosporine. The finally successful treatment involved eleven cycles of immunoadsorption using a tryptophan column and administration of a moderate dose of prednisolone. serum gamma-globulin level and anti-intercellular antibody titer decreased from 1.08 g/dl to 0.5 g/dl and 1:320 to 1:20, respectively. She has been well controlled with 21.5 mg/day prednisolone for 8 months after the final adsorption. Considering the physical, mental and social situation of adolescent student patients, immunoadsorption is a highly preferable choice among a variety of treatment modalities for pemphigus vulgaris because it makes the term of hospitalization shorter and avoids undesirable side effects from initial high dose corticosteroids.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
2/4. Paraneoplastic pemphigus caused by an epithelioid leiomyosarcoma and associated with fatal respiratory failure.A patient is described who initially presented with pemphigus vulgaris, limited to the oral cavity, and weight loss. 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 patient developed shortness of breath and routine physical examination on admission revealed an abdominal mass, which eventually was proven to be an epithelioid leiomyosarcoma. In spite of radical excision of the tumour and intensive treatment of the dyspnoea, the patient died of respiratory failure 19 months after the PNP had been diagnosed. early diagnosis of PNP is stressed to possibly prevent fatal pulmonary involvement.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
3/4. Localized mucosal involvement and severe pulmonary involvement in a young patient with paraneoplastic pemphigus associated with Castleman's tumour.We describe a 19-year-old female patient who developed recurrent ulcerations limited to the orogenital mucosa for the last 3 years. She also developed dyspnoea 5 months after the onset of the orogenital lesions. Castleman's tumour of the retroperitoneum was found incidentally during routine physical examination. The diagnosis of paraneoplastic pemphigus (PNP) was made by pathological and immunological studies. The orogenital ulceration responded well to corticosteroid therapy, but severe bronchiolitis obliterans progressed despite intensive care. The patient eventually died from respiratory failure. This case demonstrates the diversity of clinical features of paraneoplastic pemphigus.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
4/4. Studies on etiologic factors in pemphigus.A survey of 234 cases of pemphigus yielded three observations which suggest that different forms of pemphigus may have different etiologies. 1. While the incidence of pemphigus in the age group of 21 and over was essentially the same in males and females (108 and 113) the incidence was significantly higher in females in the age group under 20. Only two of 13 patients in this group were males. Also pemphigus foliaceus and erythematosus was reported in a significantly higher proportion of the cases in the age group of 2 1/2 to 20 thus suggesting that the juvenile form of the disease differs from the adult form. 2. In four of a group of 15 cases identified as pemphigus foliaceus the disease appeared to be provoked by minor physical insults, a frequency which is significantly higher than the five cases with similar histories in the group of 198 cases identified as pemphigus vulgaris. 3. In the entire group of 234 cases two patients with pemphigus both proven by immunofluorescence had relatives with pemphigus. Also a third patient had a blood relative with bullous pemphigoid. This frequency of three per 234 is higher than would be expected by chance. Studies of one family (not included in this survey) with multiple skin antibodies suggest that such familial predispositions may be due to abnormal immune responses.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |