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Atypical pemphigus involving the esophagus with IgG antibodies to desmoglein 3 and IgA antibodies to desmoglein 1

2006; Elsevier BV; Volume: 55; Issue: 2 Linguagem: Inglês

10.1016/j.jaad.2005.08.003

1097-6787

Shigeki Inui, Masayuki Amagai, Shusaku Tsutsui, Saho Fukuhara-Yoshida, Satoshi Itami, Ichiro Katayama,

Coagulation, Bradykinin, Polyphosphates, and Angioedema

To the Editor: The rare variant of pemphigus with deposition of both IgA and IgG autoantibodies against keratinocyte surface has been reported.1Hashimoto T. Ebihara T. Nishikawa T. Studies of autoantigens recognized by IgA anti-keratinocyte cell surface antibodies.J Dermatol Sci. 1996; 12: 10-17Abstract Full Text PDF PubMed Scopus (40) Google Scholar We report here a case of atypical pemphigus involving the esophagus with IgG against desmoglein 3 (Dsg3) and IgA against desmoglein 1 (Dsg1). A 78-year-old woman presented with a 2-month history of pruritic erythematous lesions on her trunk and arms. Physical examination found annular or nummular erythematous lesions with pustules, vesicles, and erosions at their periphery on her trunk (Fig 1, A) and arms, but no oral mucosal lesions. A biopsy specimen from erythematous skin revealed neutrophilic spongiosis with eosinophil infiltration (Fig 1, B) and intraepidermal pustules throughout the epidermis (Fig 1, C). These histopathologic findings were consistent with intraepidermal neutrophilic IgA dermatosis type IgA pemphigus.2Hashimoto T. Immunopathology of IgA pemphigus.Clin Dermatol. 2001; 19: 683-689Abstract Full Text Full Text PDF PubMed Scopus (64) Google Scholar Direct immunofluorescence of the perilesional skin showed IgG, IgA, and C3 deposition at the cell surface of the entire epidermis, but no deposition of IgM or fibrinogen. Indirect immunofluorescence using normal human skin demonstrated IgG anticell surface antibodies at titer 1:40 but no IgA anticell surface antibodies. Before administration of systemic corticosteroids, the patient was examined for gastric ulcers with an upper gastrointestinal endoscopy, which led to the incidental detection of multiple esophageal erosions (Fig 2, A). A biopsy specimen from the esophagus revealed suprabasal desquamation of the epithelia with lymphocyte and eosinophil infiltration (Fig 2, B). Direct immunofluorescence of the detached epithelia showed IgG and IgA deposition at the cell surface, but no deposition of IgM, C3, or fibrinogen. Enzyme-linked immunosorbent assay for anti-Dsg1 and anti-Dsg3 IgG in the patient's serum showed that the indices of anti-Dsg1 and anti-Dsg3 IgG were 18 and 34, respectively (cut-off value 20). Enzyme-linked immunosorbent assay for anti-Dsg1 and anti-Dsg3 IgA was performed as reported previously.3Hashimoto T. Komai A. Futei Y. Nishikawa T. Amagai M. Detection of IgA autoantibodies to desmogleins by an enzyme-linked immunosorbent assay: the presence of new minor subtypes of IgA pemphigus.Arch Dermatol. 2001; 137: 735-738PubMed Google Scholar The OD450 levels of IgA anti-Dsg1 and 3 were 0.202 and 0.051, respectively (cut-off value 0.15). These studies resulted in a diagnosis of atypical pemphigus with IgG autoantibodies against Dsg3 and IgA autoantibodies against Dsg1. Her response to prednisolone (60 mg/d) was good and the disease is now controlled with 5 mg/d of prednisolone.Fig 2A, Multiple esophageal erosions (upper gastrointestinal fiberscope). B, Suprabasal desquamation of epithelia with lymphocyte and eosinophil infiltration. (Hematoxylin-eosin stain, original magnification: ×200.)View Large Image Figure ViewerDownload (PPT) This report describes esophageal pemphigus with IgG and IgA autoantibodies. In our case IgG and IgA recognized two distinct antigens, Dsg3 and Dsg1, respectively. This mixed autoantibody profile correlates well with the clinical features. The histopathologic findings for the skin were identical to those for intraepidermal neutrophilic IgA dermatosis type IgA pemphigus, which is associated with IgA anti-Dsg1.4Wang J. Kwon J. Ding X. Fairley J.A. Woodley D.T. Chan L.S. Nonsecretory IgA1 autoantibodies targeting desmosomal component desmoglein 3 in intraepidermal neutrophilic IgA dermatosis.Am J Pathol. 1997; 150: 1901-1907PubMed Google Scholar The esophageal lesions showed the suprabasal desquamation consistent with pemphigus vulgaris. The fact that our patient had only IgG anti-Dsg3 but not IgG anti-Dsg1 suggests that the esophageal lesions were the equivalent of mucosal dominant pemphigus vulgaris.5Amagai M. Desmoglein as a target in autoimmunity and infection.J Am Acad Dermatol. 2003; 48: 244-252Abstract Full Text Full Text PDF PubMed Scopus (104) Google Scholar Therefore, our case represents an association of mucosal dominant pemphigus vulgaris with intraepidermal neutrophilic IgA dermatosis type IgA pemphigus.