Abstract:A 76-year-old man with interstitial lung disease was admitted to our institution after developing persistent dyspnea upon effort. He also had a relapse of bullous eruptions on the skin of the trunk and extremities, previously diagnosed as vesicular pemphigoid. Direct immunofluorescence of a skin biopsy specimen using fluorescence microscopy showed the linear deposition of immunglobulin A (IgA), IgG and C3 along the basement membrane. These findings indicated a definitive diagnosis of linear IgA/IgG bullous der… Show more
“…Hence, the diagnosis in the latter case was established based on the clinical characteristics, the lack of symptoms from the intestines, and a negative screening for coeliac disease. Mixed deposits are previously described in a number of cases (11)(12)(13)(14)(15)(16)(17)(18). Their significance remains uncertain but it may be confusing and make the differential diagnosis more difficult.…”
Linear IgA bullous dermatosis (LAD) is an autoimmune, chronic bullous disease affecting primarily young children and adults. Studies on LAD are relatively sparse and from Scandinavia we could only find a few case reports. Therefore we decided to conduct a retrospective investigation of patients seen at our department since 1972. A total of 23 patients were identified; 7 children (F:M ratio 0.75) and 16 adults (F:M ratio 0.78). Mean age at disease onset in the two age groups were 2.7 and 56.8 years. Estimated incidence rate in our region: 0.67 per million per year. The most commonly used treatment modalities were corticosteroids, dapsone and sulphapyridine.
“…Hence, the diagnosis in the latter case was established based on the clinical characteristics, the lack of symptoms from the intestines, and a negative screening for coeliac disease. Mixed deposits are previously described in a number of cases (11)(12)(13)(14)(15)(16)(17)(18). Their significance remains uncertain but it may be confusing and make the differential diagnosis more difficult.…”
Linear IgA bullous dermatosis (LAD) is an autoimmune, chronic bullous disease affecting primarily young children and adults. Studies on LAD are relatively sparse and from Scandinavia we could only find a few case reports. Therefore we decided to conduct a retrospective investigation of patients seen at our department since 1972. A total of 23 patients were identified; 7 children (F:M ratio 0.75) and 16 adults (F:M ratio 0.78). Mean age at disease onset in the two age groups were 2.7 and 56.8 years. Estimated incidence rate in our region: 0.67 per million per year. The most commonly used treatment modalities were corticosteroids, dapsone and sulphapyridine.
“…There have been numerous reports of interstitial lung disease (ILD) associated with autoimmune diseases such as rheumatoid arthritis (RA) and dermatomyositis (2,3). However, autoimmune bullous diseases (AIBDs)-associated ILD is extremely rare (4)(5)(6). However, autoimmune bullous diseases (AIBDs)-associated ILD is extremely rare (4)(5)(6).…”
Autoimmune bullous diseases (AIBDs)-associated interstitial lung disease (ILD) is extremely rare. Pemphigus vulgaris (PV) is an intraepidermal autoimmune blistering disease caused by circulating autoantibodies against desmoglein. To date, PV-associated ILD has rarely been reported in English literature. We report a rare association of PV and ILD. A 53-year-old Chinese female with PV for 8 months developed ILD after a relapse of PV for 2 months due to discontinuation of oral prednisone by herself. She was successfully treated by systemic methylprednisolone. Taken previously reported bullous pemphigoid-associated ILD and linear IgA/IgG bullous dermatosis-associated ILD together, in general, AIBDs-associated ILD occurs when AIBDs relapse or are not controlled, responds well to systemic corticosteroids, and has a relatively better prognosis when compared with rheumatoid arthritis- or dermatomyositis-associated ILD.
“…Linear IgA/IgG bullous dermatosis is associated with systemic diseases such as malignant B‐cell lymphoma, Vogt–Koyanagi–Harada disease (VKHD) and interstitial pneumonia . This is the first report of LAGBD associated with UC, although the associations between other immunobullous skin diseases and UC have been reported.…”
Section: Discussionmentioning
confidence: 91%
“…To the contrary, there are reports convincing to our speculation. Kakugawa et al . reported a case of interstitial pneumonia associated with LAGBD that developed before lung disease.…”
Section: Discussionmentioning
confidence: 99%
“…To the contrary, there are reports convincing to our speculation. Kakugawa et al 9 reported a case of interstitial pneumonia associated with LAGBD that developed before lung disease. They stated that direct IF of a lung biopsy specimen revealed deposition of IgA, IgG and C3 along the epithelial cell membrane and basement membrane of bronchioles and alveoli.…”
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.
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