UK A 79-year-old man presented with palpable purpura and poorly defined erythematous plaques on his limbs ( Fig. 1 ). History, examination, and clinical investigations (which included a full blood count, urea, electrolyte, and liver function tests, negative antinuclear and DNA antibodies, antineutrophil cytoplasmic antibody (ANCA), and cold agglutinins) showed no systemic involvement or triggering factors. He had not lost weight and took no medication. A diagnosis of cutaneous vasculitis was made and the patient's eruption improved with bed rest. He returned 2 months later with widespread tense blisters ( Fig. 2 ) and an erythrocyte sedimentation rate (ESR) of 90. The blisters were symmetrically distributed and sited predominantly on the flexural aspects of the upper limbs and trunk. Additional investigations were performed, with negative blood cultures, chest X-ray, sputum cytology, sigmoidoscopy, fecal occult bloods, mid-stream urine, abdominal ultrasound, and echocardiogram. He responded well to oral corticosteroids, with a rapid reduction in ESR. Twelve months later his condition remains well controlled on 7.5 mg of prednisolone daily. Figure 1 Open in figure viewer PowerPoint Painful palpable purpura on the left thigh 相似文献
We describe a patient with psoriasis who developed bullous pemphigoid in sites of psoriatic plaques, in whom there was a second organ-specific auto-immune disorder, Hashimoto's thyroiditis. 相似文献
Bullous pemphigoid is an autoimmune blistering skin disease of the elderly that may be preceded by a pruritic, urticarial or eczematous eruption. We report a case of bullous pemphigoid preceded by prodromal eczematous eruptions that lasted an unusually long time of 11 years. Elderly patients with persistent pruritic or eczematous eruption of unknown etiology should be carefully followed, as bullous pemphigoid may be a potential diagnosis. 相似文献
Autoantibodies to a normal component of stratified squamous epithelia, the bullous pemphigoid antigen (BPA), are synthesized in patients with the disease bullous pemphigoid. We have used these sera to study the distribution of BPA in vivo and in vitro. At low magnification, indirect immunofluorescent staining for BPA is linear at the basement membrane zone (BMZ) of skin and many other epithelial tissues. At higher magnification however, we observed a punctate staining pattern for BPA which was regular in appearance and suggested localization of BPA to discrete structures at the BMZ. Subsequent immunoelectron microscopy using both peroxidase and colloidal gold labeling techniques with patients' sera or IgG, revealed that BPA is associated with hemidesmosomes--putative adhesion structures at the BMZ, based on their similarity in ultrastructure to desmosomes. More specifically BPA was immunolocalized to the cytoplasmic face of hemidesmosomes and was not observed extracellularly in the basement membrane. In stratifying and nonstratifying cultures of rat keratinocytes, BPA is expressed intracellularly and not in the cell-derived matrix, unlike other known basement membrane components. These cells also synthesize BPA in vitro, and immunoprecipitation from metabolically labeled cultures revealed a 220 kD polypeptide under reducing conditions. From these observations we conclude (1) that BPA is a 220 kD polypeptide component either of or associated with hemidesmosomes, and (2) that it is localized intracellularly both in vivo and in vitro. We suggest that BPA is not normally a lamina lucida component, but that it may form part of a linkage between the cytoskeleton and the basement membrane. 相似文献
The nail can be affected in autoimmune bullous diseases. Because of the different antigens involved, nails are often affected in epidermolysis bullosa acquisita (EBA), but not in bullous pemphigoid (BP). We describe an unusual case of BP with permanent loss of toenails. 相似文献
Bullous pemphigoid is a rather rare disease in childhood. A case report is given about a seven-year-old girl suffering from juvenile bullous pemphigoid and the differential diagnosis is discussed with regard to the other bullous diseases in children. Some clues were noted in the case history of our patient concerning an effective treatment using erythromycin in bullous pemphigoid. A short summary is presented about the theoretical basis with respect to this therapeutical procedure. 相似文献
Bullous pemphigoid (BP) has never before been reported to associate with silicosis, although there are numerous reports of silicosis accompanied by different autoimmune diseases, such as systemic sclerosis, systemic lupus erythematosus, dermatomyositis or rheumatoid arthritis. We report on a 63-year-old Japanese patient with silicosis who developed tensed bullae, erosions and macular pigmentation on the trunk and extremities. Indirect immunofluorescence revealed anti-basement-membrane-zone antibodies; immunoblotting analysis demonstrated that the patient's serum reacted with the 230-kD BP antigen in the epidermal extracts, as well as a recombinant protein of the NC16a domain of 180-kD BP antigen. Clinical symptoms improved after treatment with systemic steroids. To the best of our knowledge, this is the first reported case of BP associated with silicosis. 相似文献
Bullous pemphigoid (BP) is the most common autoimmune blistering disease affecting the elderly but is quite rare in childhood. The majority of pediatric cases have been reported during early childhood. Adolescence is divided into three phases: early (10‐13 years), middle (14‐17), and late (18‐21). This review aimed to identify BP cases in adolescence and demonstrate their clinical features and course. Our literature search was performed in Medline with the terms “bullous pemphigoid in childhood and adolescence,” “childhood bullous pemphigoid,” “juvenile bullous pemphigoid,” and “autoimmune blistering and autoimmune bullous diseases in childhood.” The data extraction for late adolescence was limited by the fact that this age group is included in adult BP registries. We identified nine cases in early adolescence. Mucosa were affected in 5 of 9 cases. Treatment consisted of systemic prednisone (8/9), in combination with dapsone (2/9), azathioprine (2/9), or erythromycin/nicotinamide (1/9). Relapses were reported in 3 of 9 cases. We identified five cases occuring in middle adolescence. Mucosa were not affected. Treatment consisted of systemic prednisone (5/5), in combination with dapsone (3/5), azathioprine (2/5), doxycycline/nicotinamide (1/5), or mycophenolate mofetil (1/5). Relapses were reported in two of five cases. No case of BP in the late adolescence was included in the results, as only one case met the search criteria, and overlapped with pemphigus vulgaris. With only 14 cases found in our review, BP in adolescence appears even rarer than in earlier childhood. Despite its low prevalence, BP should be included in the differential diagnosis of autoimmune blistering diseases in adolescents. 相似文献