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Hereditary mucoepithelial dysplasia: unique histopathological findings in skin lesions
Author(s) -
Leithauser Laurel A.,
Mutasim Diya F.
Publication year - 2012
Publication title -
journal of cutaneous pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.597
H-Index - 75
eISSN - 1600-0560
pISSN - 0303-6987
DOI - 10.1111/j.1600-0560.2011.01857.x
Subject(s) - pathology , intertriginous , medicine , dysplasia , erythema , genodermatosis , histopathology , dermatology , disease , biology , biochemistry , gene
Hereditary mucoepithelial dysplasia (HMD) is a rare, autosomal dominantly inherited, multisystem disease thought to be caused by abnormalities in desmosomes and gap junctions. HMD affects the skin, mucosae, hair, eyes and lungs. Nearly all patients clinically display the triad of non‐scarring alopecia, well‐demarcated mucosal erythema and erythematous intertriginous plaques. Although histopathological findings of mucous membrane epithelium have been well characterized, only three reports have described histopathologic findings in the skin. We report a case with unique histopathological findings not previously reported in HMD. Leithauser LA, Mutasim DF. Hereditary mucoepithelial dysplasia: unique histopathological findings in skin lesions.