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Linear IgA Bullous Dermatosis in Association With Crohn Disease

  • Pei-Chun Weng
    Affiliations
    Department of Dermatology, Chang Gung Memorial Hospital, Linkou Branch, Taoyuan, Taiwan

    Drug Hypersensitivity Clinical and Research Center, Chang Gung Memorial Hospital, Linkou Branch, Taoyuan, Taiwan

    College of Medicine, Chang Gung University, Taoyuan, Taiwan
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  • Yi-Teng Hung
    Affiliations
    Department of Dermatology, Chang Gung Memorial Hospital, Linkou Branch, Taoyuan, Taiwan

    Drug Hypersensitivity Clinical and Research Center, Chang Gung Memorial Hospital, Linkou Branch, Taoyuan, Taiwan

    College of Medicine, Chang Gung University, Taoyuan, Taiwan
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  • Puo-Hsien Le
    Affiliations
    Department of Gastroenterology & Hepatology, Chang Gung Memorial Hospital, Linkou Branch, Taoyuan, Taiwan

    Taiwan Association of the Study of Small Intestinal Disease, Taoyuan, Taiwan

    Chang Gung Microbiota Therapy Center, Chang Gung Memorial Hospital, Taoyuan, Taiwan
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  • Yu-Huei Huang
    Correspondence
    Correspondence: Address to Yu-Huei Huang, MD, Department of Dermatology, Chang Gung Memorial Hospital, Linkou Branch, Taoyuan, Taiwan, Linkou, 5, Fuxing St., Guishan Dist., Taoyuan 33305, Taiwan.
    Affiliations
    Department of Dermatology, Chang Gung Memorial Hospital, Linkou Branch, Taoyuan, Taiwan

    Drug Hypersensitivity Clinical and Research Center, Chang Gung Memorial Hospital, Linkou Branch, Taoyuan, Taiwan

    College of Medicine, Chang Gung University, Taoyuan, Taiwan
    Search for articles by this author
      A man in his 50s with a 3-year history of Crohn disease presented with a 1-week history of pruritic blistering on the trunk and extremities. Examination revealed tense vesicles arranged in annular pattern on the nape, upper back, and extensor side of the upper arms (Figure 1). No new medications were initiated in the past 3 months, including antibiotics, angiotensin-converting enzyme (ACE) inhibitors, and nonsteroidal anti-inflammatory drugs (NSAIDs). A skin biopsy from the right upper arm showed a subepidermal blister with neutrophilic infiltrates at the basement membrane and papillary dermis. Direct immunofluorescence study revealed linear immunoglobulin A (IgA) deposition along the basement membrane (Figure 2). Clinicopathologic correlation led to a diagnosis of linear IgA bullous dermatosis (LABD). Skin lesions resolved a month later following treatment with oral prednisolone (0.4 mg/kg per day) and topical corticosteroids. The activity of Crohn disease remained stable throughout the course of LABD.
      Figure thumbnail gr1
      Figure 1Tense vesicles arranged in annular configuration on the extensor side of the upper arms, with a characteristic “strings of pearls” sign.
      Figure thumbnail gr2
      Figure 2Histopathologic features. A, A subepidermal blister with neutrophilic infiltrates at the basement membrane and papillary dermis (hematoxylin and eosin stain). B, Linear immunoglobulin A deposition along the basement membrane in direct immunofluorescence examination.
      An autoimmune bullous disease, LABD is characterized by annular or polycyclic, tense blisters typically on the extensor extremities, trunk, buttocks, and face with linear deposition of IgA at the basement membrane; LABD can be idiopathic or associated with drug exposure (eg, vancomycin, ACE inhibitors, NSAIDs), inflammatory bowel disease (IBD), systemic lupus erythematosus, and malignancies.
      • Fortuna G.
      • Marinkovich M.P.
      Linear immunoglobulin A bullous dermatosis.
      In cases of IBD associated with LABD, ulcerative colitis is more frequently reported than Crohn disease.
      • Vargas T.J.
      • Fialho M.
      • Santos L.T.
      • et al.
      Linear IgA dermatosis associated with ulcerative colitis: complete and sustained remission after total colectomy.
      LABD usually follows IBD and may occur during flare-ups of IBD.
      • Torres T.
      • Sanches M.
      • Selores M.
      Linear IgA bullous disease in a patient with Crohn's disease.
      The mainstays of treatment for LABD are dapsone, sulfapyridine, and colchicine. Treating IBD may result in resolution of LABD, and vice versa.
      • Vargas T.J.
      • Fialho M.
      • Santos L.T.
      • et al.
      Linear IgA dermatosis associated with ulcerative colitis: complete and sustained remission after total colectomy.
      ,
      • Torres T.
      • Sanches M.
      • Selores M.
      Linear IgA bullous disease in a patient with Crohn's disease.
      Patients presenting with gastrointestinal symptoms and otherwise unexplained LABD should be screened for IBD.

      Potential Competing Interests

      The authors report no competing interests.

      References

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        Linear immunoglobulin A bullous dermatosis.
        Clin Dermatol. 2012; 30: 38-50
        • Vargas T.J.
        • Fialho M.
        • Santos L.T.
        • et al.
        Linear IgA dermatosis associated with ulcerative colitis: complete and sustained remission after total colectomy.
        An Bras Dermatol. 2013; 88: 600-603
        • Torres T.
        • Sanches M.
        • Selores M.
        Linear IgA bullous disease in a patient with Crohn's disease.
        Acta Dermatovenerol Alp Pannonica Adriat. 2010; 19: 29-31