Linear immunoglobulin A/immunoglobulin G bullous dermatosis associated with Vogt-Koyanagi-Harada disease

Vogt-Koyanagi-Harada disease is characterized by marked bilateral uveitis associated with symmetric vitiligo, alopecia, poliosis and dysacousia. Linear immunoglobulin (Ig)A bullous dermatosis (LABD) is characterized by small, tense, subepidermal bullae caused by IgA type autoantibody targeting the basal lamina. LABD patients sometimes show coexistence of IgG type autoantibody, termed linear IgA/IgG bullous dermatosis (LAGBD). We reported a 35-year-old Japanese male case of combined LAGBD and Vogt-Koyanagi-Harada disease. His human leukocyte antigen typing was -A24, B52, C*1202, DR*1502, DQ*0601. Immunoblot revealed that patient sera reacted to both 180- and 230-kDa proteins at the IgA and IgG level. Because Vogt-Koyanagi-Harada disease and LABD are reported to be associated with other autoimmune diseases, it is probable that Vogt-Koyanagi-Harada disease and LAGBD in our case may be associated with each other in the pathomechanism. However, we cannot exclude the possibility of this being mere coincidence.     

Linear immunoglobulin A bullous dermatosis

Linear immunoglobulin A (IgA) bullous dermatosis, also known as linear IgA disease, is an autoimmune mucocutaneous disorder characterized by subepithelial bullae, with IgA autoantibodies directed against several different antigens in the basement membrane zone. Its immunopathologic characteristic resides in the presence of a continuous linear IgA deposit along the basement membrane zone, which is clearly visible on direct immunofluorescence. This disorder shows different clinical features and distribution when adult-onset of linear IgA disease is compared with childhood-onset. Diagnosis is achieved via clinical, histopathologic, and immunopathologic examinations. Two common therapies are dapsone and sulfapyridine, which reduce the inflammatory response and achieve disease remission in a variable period of time.     

Linear IgA bullous dermatosis in a patient with chronic renal failure: response to intravenous immunoglobulin therapy

Linear IgA bullous dermatosis is a blistering disease with a heterogeneous clinical manifestation, characterized by deposition of IgA along the basement membrane zone of perilesional skin on direct immunofluorescence. We describe a patient with chronic renal failure who experienced linear IgA bullous dermatosis. Long-term administration of intravenous immunoglobulin therapy was associated with clinical remission lasting more than 12 months.     

儿童线状IgA大疱性皮病误诊为脓疱疮1例

临床资料患儿,男,7岁。因面部、双小腿反复发生水疱1年,会阴新发皮疹4天,于2008年08月15日来我院就诊。患者一年前无明显诱因于面部、双小腿出现水疱、脓疱及大疱,皮损偶瘙痒,皮疹反复发作,无发热、     

Linear IgA bullous disease of childhood: an experience from Kuwait

Linear IgA bullous disease of childhood is a rare autoimmune blistering disease. We report eight patients with this disease seen in our autoimmune bullous diseases clinic over a span of 12 years. They constituted 89% of the total number of those with linear IgA bullous disease of childhood seen during this period, with an age-adjusted minimum estimated incidence of 2.3 cases/million population/year. Males outnumbered females by a 1.7:1 ratio. The age at onset ranged between 10.5 months and 13 years, with a mean of 6.8 +/- 4.17 years. The majority of patients (62.5%) had moderately severe disease. Fifty percent of patients were observed to have an association with either an autoimmune disease (Crohn disease and post-streptococcal glomerulonephritis in one each) or an infection (beta-hemolytic streptococcal and hepatitis A virus infection in one each). The treatment of choice was dapsone alone or in combination with systemic steroids. Seventy-one percent of patients achieved complete remission by the end of 2 years. The study highlights the significance of systematic clinicoepidemiologic surveys from different regions.     

Infantile bullous pemphigoid treated with intravenous immunoglobulin therapy

A 3-month-old boy presented with a 2-week history of rapidly spreading skin rashes. Physical examination revealed generalized urticarial plaques with tense bullae and small vesicles. Histologic examination and immunofluorescence established the diagnosis of bullous pemphigoid. The disease was resistant to conventional therapies with the combination of corticosteroids, dapsone, and erythromycin. Finally, intravenous immunoglobulin therapy brought about a remarkable improvement. We suggest that intravenous immunoglobulin therapy is a valuable treatment option for intractable bullous pemphigoid in infants as well as in adults.     

The use of intravenous immunoglobulin in autoimmune bullous diseases

Intravenous immunoglobulin (IVIG) has been shown to be effective in the treatment of autoimmune blistering diseases and may be an option if disease is refractory to conventional treatment. IVIG effectiveness appears to increase when administered concurrently with a cytotoxic drug and used in multiple treatment cycles (though a single cycle may give benefit). Tapering administration may improve the duration of remission and subcutaneous injections may be an option. This article provides an introduction to the make-up and use of IVIG, and reviews previous studies.     

儿童线状IgA/IgG大疱性皮病一例

患儿,男,7岁.因躯干、四肢散发红斑、水疱伴瘙痒2个月就诊.发病前有泳池"暴晒"史.背部水疱组织病理:表皮下水疱,真皮乳头中性粒细胞及少许嗜酸粒细胞小脓肿,浅层中性粒细胞、嗜酸粒细胞、淋巴细胞浸润.正常人皮肤盐裂间接免疫荧光:循环抗体IgA、IgG于表皮侧均有沉积,局部区域IgG表皮真皮双侧沉积.综上诊断为儿童线状Ig...     

Treatment of chronic bullous disease of childhood

Chronic bullous disease of childhood (CBDC) is generally a self-limited disease of prepubescent children that resolves within months to years. However, as discussed in the previous issue of Dermatologic Clinics, the disease is associated with significant morbidity and usually requires systemic therapy. Treatment is aimed at controlling blistering while avoiding adverse reactions. There are several anecdotal reports of treatment options, but controlled or comparative studies are lacking. This article discusses the management options of CBDC in more detail.     

Linear immunoglobulin A/G bullous dermatosis associated with ulcerative colitis

Linear immunoglobulin (Ig)A/G bullous dermatosis (LAGBD) is an autoimmune bullous disease characterized by formation of subepidermal blisters and linear deposition of IgA and IgG antibodies along the basement membrane zone (BMZ). The association between linear IgA bullous dermatosis and ulcerative colitis (UC) is well recognized, but reports of UC‐associated LAGBD are lacking. We have reported a 24‐year‐old man suffering from LAGBD associated with UC, which occurred before exacerbations of skin rash. A skin biopsy indicated a subepidermal blister with an infiltration of primarily neutrophils and eosinophils in the dermis. Direct immunofluorescence (IF) studies showed a linear deposition of IgA, IgG and C3c. Indirect IF of human skin revealed IgA and IgG anti‐BMZ autoantibodies. Indirect IF of 1 M NaCl‐split human skin demonstrated reactivity of IgA and IgG antibodies at the epidermal side. Immunoblotting showed that IgG antibodies reacted to the BP180 NC16a domain and 120‐kDa linear IgA dermatosis‐1, and enzyme‐linked immunoassay detected IgG anti‐BP230 antibodies. Administration of prednisolone and diaminodiphenyl sulfone (DDS) via the p.o. route improved skin lesions and bowel conditions. These results suggest that the bowel inflammation observed in UC may have a causative effect of initiation of the immune response to the skin and development of the bullous skin lesions in LAGBD. A combination of DDS and corticosteroid could be a recommended therapeutic option for patients with LAGBD with UC.     

High-dose intravenous immunoglobulin in the treatment of adult patients with bullous pemphigoid

High-dose intravenous immunoglobulin (IVIg) has only been sporadically used in the treatment of bullous pemphigoid (BP), as it is suggested as an adjuvant to systemic corticosteroids in progressive disease or when life-threatening complications are of concern with other therapeutic interventions. The aim of the present study was to report our observations in the treatment of adult BP patients with IVIg, in association with a focused literature review. In our Department we identified five patients (4 women, 1 man) who had received IVIg for BP relatively early in the course of their disease. These cases were added to the 36 adequately documented ones reported in the literature. Most of these patients (33/41) responded to treatment with IVIg and 7/33 responders remained clear one year after the onset of IVIg. However, the time for effective disease control after IVIg treatment depended positively on disease duration before treatment (P<0.01). In conclusion, despite the limited experience with its use, IVIg seems to be a useful therapeutic alternative to conventional modalities for selected BP patients, particularly if it is initiated promptly after BP diagnosis.