共查询到20条相似文献,搜索用时 9 毫秒
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Degamber D. Banodkar MD Abdul Raouf Al-Suwaid MD 《International journal of dermatology》1997,36(3):213-216
Background The treatment of chronic bullous dermatosis of childhood (CBDC) so far has been limited to the use of corticosteroids and the sulfa group of drugs, e.g. daspsone and sulfapyridine. Furthermore, the therapy of CBDC cases with associated G6 PD deficiency is restricted only to systemic steroids. Histopathologically CBDC is characterized by the presence of predominantly neutrophilic Infiltration and because it has been proven to exert strong anti-inflammatory effects through the inhibition of neutrophils, colchicine was mandated for its use in CBDC. Methods To avoid the detrimental side-effects of the long-term use of steroids in children, an alternative anti-inflammatory drug like colchicine was considered. Patients with G6 PD deficiency and those who were not satisfactorily controlled with steroid therapy, and who in addition developed unacceptable side-effects like cushingoid faces and hypertrichiosis, were treated with the drug. Results Eight patients were given colchicine, five (62.5%) of which showed complete remission within 4–6 weeks of starting the therapy. The remaining three (37.5%) also responded but required adjuvant small doses of steroids to maintain the remissions. The drug was very well tolerated and no side-effects were observed. Conclusions Colchicine is, therefore, found to be an effective treatment in CBDC and has enhanced our armamentarium of therapeutics for this condition, especially in children with G6 PD deficiency. 相似文献
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患者,女,42岁.周身红斑水疱3个月.经病理和免疫病理确诊为线状IgA大疱性皮病.既往曾使用中等剂量糖皮质激素治疗,效果不佳.经加用氨苯砜后病情得以控制,之后激素逐渐减量之停用,目前单用氨苯砜治疗控制中. 相似文献
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S.B.P. RK K.H.C. O J.I. YOUN D.H. HWANG S.C. KIM† J.H. CHUNG 《Clinical and experimental dermatology》1997,22(5):220-222
Epidermolysis bullosa acquisila (EBA) is rarely reported in childhood, hut we mm describe a 6-year-old Korean girl with the condition. She presented with multiple tense bullae annularly distributed on the perioral, periorbital and genital areas, and was successfully treated with dapsoae. The clinical and histological features were similar to those of chronic bullous dermatosis of childhood. We review seven previously reported childhood EBA cases and contrast their features with those of adult EBA. We suggest that some childhood EBA is different from the adult form and shares features with chronic bullous dermatosis of childhood. Epidermolysis bullosa acquisita (EBA) is an acquired, chronic, subepidermal bullous disease,1 characterised by IgG autoantibodies reacting with type VII collagen of the fibrils beneath the basement membrane Zone of the stratified squamous epithelium.2,3 Its clinical and epidemiological features are not fully understood, and only a few cases in children have so far been reported.4–8 相似文献
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N B Esterly N L Furey B S Kirschner R R Kretschmer R M Septon 《Archives of dermatology》1977,113(1):42-46
The clinical features, laboratory studies, and therapeutic responses of two boys with chronic bullous dermatosis of childhood are described. Direct immunofluorescent preparations of sections from a lesion, skin adjacent to a lesion, and uninvolved skin demonstrated linear deposition of IgA at the dermoepidermal junction in all three biopsy specimens from one patient. Similar preparations from the second child were negative for staining. No circulating antibodies to skin components were detected in either child by means of multiple substrates. Neither child had clinical manifestations nor laboratory findings suggestive of an associated gastrointestinal lesion; therefore, small bowel biopsies were not performed. Immunologic studies failed to demonstrate any of the abnormalities frequently described in dermatitis herpetiformis. Both boys responded dramatically to sulfapyridine therapy. 相似文献
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A case of chronic bullous dermatosis of childhood in a 3-year-old boy is described. Immunoflourescence tests were negative and biopsy of the jejunal mucosa showed marked villous atrophy. The dermatosis was brought under control by a combination of diaminodiphenylsulphone and systemic steroids. The relationship with other bullous eruptions of childhood such as dermatitis herpetiformis and bullous pemphigoid is discussed. 相似文献
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F. Wojnarowska R.A. Marsden † P.H. McKee § M.M. Black ‡ 《The British journal of dermatology》1985,113(S29):17-18
Benign chronic bullous dermatosis of childhood (BCBDC) is an apparently self-limiting vesiculo-bullous disease showing linear IgA deposits in the basement membrane zone and which seems to occur exclusively in children. Its relationship to linear IgA dermatosis of adults (LAD) is uncertain and for this reason we studied 28 patients with BCBDC (onset before puberty) and compared them with 18 patients with LAD (onset after puberty).
No significant clinical, HLA, histological or immunopathological differences were found between the two disorders. Surprisingly, nine patients in each group had conjunctival scarring which in some produced a cicatricial pemphigoid-like appearance and in one case of BCBDC resulted in blindness. Remissions with loss of IgA from the skin occurred in patients from both groups. However, the disease duration varied considerably (BCBDC 2–28 and LAD 1–40 years) with six BCBDC patients having had their disease for over 10 years and five of them passing puberty.
All patients (six BCBDC, eight LAD) studied by immuno-electronmicroscopy showed localization of IgA beneath the basal lamina. Direct and indirect immunofluorescence studies using suction blisters also gave similar results in both groups.
As a result of this study many of our former views on BCBDC have been revised and we now believe that both BCBDC and LAD are manifestations of the same disease. 相似文献
No significant clinical, HLA, histological or immunopathological differences were found between the two disorders. Surprisingly, nine patients in each group had conjunctival scarring which in some produced a cicatricial pemphigoid-like appearance and in one case of BCBDC resulted in blindness. Remissions with loss of IgA from the skin occurred in patients from both groups. However, the disease duration varied considerably (BCBDC 2–28 and LAD 1–40 years) with six BCBDC patients having had their disease for over 10 years and five of them passing puberty.
All patients (six BCBDC, eight LAD) studied by immuno-electronmicroscopy showed localization of IgA beneath the basal lamina. Direct and indirect immunofluorescence studies using suction blisters also gave similar results in both groups.
As a result of this study many of our former views on BCBDC have been revised and we now believe that both BCBDC and LAD are manifestations of the same disease. 相似文献
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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. 相似文献
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R.A. MARSDEN P.H. McKEE† B. BHOGAL M.M. BLACK L.A. KENNEDY‡ 《Clinical and experimental dermatology》1980,5(2):159-172
Eighteen patients with benign chronic bullous dermatosis of childhood were studied and the findings compared with those of dermatitis herpetiformis (twenty-two cases) and bullous pemphigoid (five cases) beginning in childhood. The patients with benign chronic bullous dermatosis of childhood had a moderately pruritic bullous eruption with maximal involvement of the pelvic and perioral regions which tended to occur at an earlier age than either dermatitis herpetiformis or bullous pemphigoid. In contrast to dermatitis herpetiformis one-third of the cases with benign chronic bullous dermaiosis of childhood went into remission. Evidence of coeliac disease was only found in the dermatitis herpetiformis group. Surprisingly both diseases shared HLA-B8. A linear BMZ band of IgA was detected on direct immunofluorescence in all but one of the cases with benign chronic bullous dermatosis of childhood and circulating antibodies were detectable in two-thirds. Routine histopathology was of little value in distinguishing between benign chronic bullous dermaiosis of childhood and dermatitis herpetiformis or bullous pemphigoid. Several paradoxes have yet to be explained before it can be determined whether benign chronic bullous dermatosis of childhood is a variant of dermatitis herpetiformis or linear IgA disease. 相似文献
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We report on a rare case of benign chronic bullous dermatosis of childhood (BCBDC) in a 5-year-old girl. In addition to the characteristic symptoms such as abrupt onset of the disease, tense blisters, predominant affection of the face, and later imitation of impetigo, we observed uncommonly extensive involvement of the oral mucosa. Whereas histopathology was not diagnostic, direct immunofluorescence could verify BCBDC as it showed linear deposits of IgA along the basement membrane zone. This case gives rise to the discussion of the clinical entity and nomenclature of BCBDC in relation to similar bullous diseases of childhood and adults. 相似文献
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H Aram 《Archives of dermatology》1984,120(2):245-247
A 33-year-old woman had many tender, erythematous and pustular plaques on her face, neck, and extremities. Biopsy findings were consistent with a diagnosis of Sweet's syndrome. A chemotaxis test disclosed increased chemotactic activity of the peripheral blood polymorphonuclear neutrophil leukocytes. The patient was successfully treated with dapsone. To the best of my knowledge, this is the first reported case of Sweet's syndrome in which dapsone therapy was used successfully. 相似文献
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Passos L Rabelo RF Matsuo C Santos M Talhari S Talhari C 《Anais brasileiros de dermatologia》2011,86(4):747-750
A 21-year-old female presenting linear IgA and IgG disease initially responded well to dapsone therapy. However, the treatment with dapsone was withdrawn due to severe anemia induced by malaria, which led to worsening of the clinical picture. Although prednisone and methylprednisolone were tried, the patient responded only to the association of dapsone and mycophenolate mofetil. 相似文献