Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0235108 (tense)
 2,176 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

During the period 1985-88, 30 children with a chronic blistering dermatosis were studied. Of these 25 were found to have chronic bullous dermatosis of childhood (CBDC) and five had bullous pemphigoid (BP). No case of dermatitis herpetiformis (DH) was seen in the same period. Except for the difference in immunofluorescence (IMF) there were no definite clinical, histological or therapeutic differences between the two groups. All the children were Africans with the exception of one Indian girl. Their ages ranged from 1 year to 12 years with a mean of 5 years. The females outnumbered the males in a ratio of 3:2. All children had a generalized eruption consisting of large tense blisters arising on normal skin. The blisters were more profuse on the lower trunk, pelvic region and limbs. Face and scalp were also affected. Histological features of BP and DH were seen. Direct IMF in the CBDC patients showed linear deposits of IgA at the basement membrane zone (BMZ) while linear deposits of IgG were seen in the BP group. Complement and IgM were also seen in some cases in both groups. Sixty per cent of the CBDC patients showed IgA BMZ antibodies by indirect IMF. There were no symptoms or signs of malabsorption. Serum vitamin B12 and folate levels were normal. HLA studies showed the B-8 antigen in five of the 20 patients studied. Therapy was difficult in most cases. All patients haemolysed on therapeutic doses of dapsone, sulphapyridine and/or prednisone had to be added. Follow-up was generally poor as six patients failed to return after discharge from hospital.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Chronic bullous dermatosis of childhood--clinical and immunological features seen in African patients. 193 64

Chronic bullous dermatosis of childhood is one of the nonhereditary blistering diseases of children. Clinically, it is characterized by predominantly monomorphous, large tense bullae, which often form a "rosette pattern" or "jewel-like" clustering and have a predilection for the lower trunk, pelvic region, and lower extremities. Histologically, a subepidermal blister is seen, which is indistinguishable from either bullous pemphigoid or dermatitis herpetiformis. Although usually responsive to sulfone therapy, some cases require the combination of sulfones and systemic corticosteroids or corticosteroids alone to control the disease. Recent advances in immunologic techniques reveal: 1. a linear band of IgA at the dermal-epidermal junction on direct immunofluorescence that has been reported both in the lamina lucida and below the basal lamina on immunoelectron microscopy; 2. IgA antibasement membrane antibodies on indirect immunofluorescence; 3. normal jejunal biopsies; and 4. a high association with HLA-B8. It remains unclear whether chronic bullous dermatosis of childhood represents a separate disease entity or is merely a variant of dermatitis herpetiformis. Chronic bullous dermatosis of childhood also differs from linear IgA dermatosis of the adult in that the latter is not associated with HLA-B8, and thus should not be confused with this disease by similar nomenclature.
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PMID:Benign chronic bullous dermatosis of childhood: a review. 704 99

Dermatitis herpetiformis (DH) or Duhring-Brocq disease is a chronic bullous disease characterized by intense itching and burning sensation in the erythematous papules and urticarial plaques, grouped vesicles with centrifuge growth, and tense blisters. There is an association with the genotypes HLA DR3, HLA DQw2, found in 80-90% of cases. It is an IgA-mediated cutaneous disease, with immunoglobulin A deposits appearing in a granular pattern at the top of the dermal papilla in the sublamina densa area of the basement membrane, which is present both in affected skin and healthy skin. The same protein IgA1 with J chain is found in the small intestinal mucosa in patients with adult celiac disease, suggesting a strong association with DH. Specific antibodies such as antiendomysium, antireticulina, antigliadin and, recently identified, the epidermal and tissue transglutaminase subtypes, as well as increased zonulin production, are common to both conditions, along with gluten-sensitive enteropathy and DH. Autoimmune diseases present higher levels of prevalence, such as thyroid (5-11%), pernicious anemia (1-3%), type 1 diabetes (1-2%) and collagen tissue disease. The chosen treatment is dapsone and a gluten-free diet.
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PMID:Review: dermatitis herpetiformis. 2406 31