Clinical, pathologic, and immunopathologic features of dermatitis herpetiformis: review of the Mayo Clinic experience

Background Dermatitis herpetiformis (DH) is a cutaneous manifestation of gluten sensitivity, occasionally associated with other autoimmune disorders, and reportedly associated with an increased risk of lymphoproliferative disorders. We describe a series of patients with DH, focusing on associated disorders (particularly celiac disease), incidence of lymphoma, histopathology, and sensitivity of direct immunofluorescence (DIF) testing and serologic testing with antiendomysium antibodies for the diagnosis of DH. Methods The medical records of 264 patients with DH diagnosed between 1970 and 1996 were reviewed retrospectively. In addition, the records of six patients evaluated before the advent of DIF testing between 1932 and 1969 were reviewed. Results Established celiac disease was present in 12.6% of patients with DH, autoimmune systemic disorders in 22.2%, malignant neoplasms in 10.4%, sarcoidosis in four patients, and ulcerative colitis in six patients. Lymphoproliferative disorders were found in seven patients. The histopathologic examinations showed a marked predominance of neutrophils in the inflammatory infiltrate. DIF testing was positive in 92.4% of the patients tested. Indirect immunofluorescence assay indicated circulating antiendomysial antibodies in the sera of 40 of the 63 patients tested (63.5%). Conclusions In this large series of patients with DH from a single institution, patients had a low incidence of symptomatic gluten-sensitive enteropathy, low risk of lymphoproliferative disorders, and associations with other systemic autoimmune disorders. The value of DIF testing in the diagnosis of DH was confirmed. The detection of antiendomysial antibodies by indirect immunofluorescence was less sensitive than indicated by other reports.