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)

Benign chronic bullous dermatosis of childhood (BCBDC) is characterized by predominantly monomorphous, large, tense bullae with a predilection for the lower part of the trunk, the pelvic region, and the lower extremities. Most patients have blisters for two to three years and experience spontaneous exacerbations and remissions. We report the first case known to us in which a linear bank of IgA (but no lgG or lgM) and complement was found at the epidermal basal lamina as demonstrated by immunofluorescence studies.
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PMID:Benign chronic bullous dermatosis of childhood. Linear IgA and C3 deposition on the basement membrane. 78 63

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

Linear IgA bullous dermatosis is a rare sulfone-responsive subepidermal blistering disorder of unknown etiology in which smooth linear deposits of IgA are found in the basement membrane zone. Chronic bullous dermatosis of childhood is equivalent to linear IgA disease of adulthood and is characterized by an abrupt onset of large, widespread and tense bullae on a normal or erythematous base. In this case, we describe an unusual presentation of chronic bullous dermatosis in a 14-month-old Saudi girl. Histopathological examination revealed subepidermal cell poor blisters with linear deposition of IgA, IgG, IgM, and C3 along the dermoepidermal junction. The unusual clinical, histopathological and immunofluorescence findings in this patient are discussed, with an account on the differential diagnosis in such cases along with a detailed review of the relevant literature.
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PMID:Unusual clinicopathological and immunological presentation of chronic bullous dermatosis of childhood (linear IgA dermatosis). 2212 Dec 82