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Melkersson Rosenthal syndrome: a histopathologic mystery and dermatologic challenge
Author(s) -
Kaminagakura Estela,
Jorge Jacks
Publication year - 2011
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.1600-0560.2009.01446.x
Subject(s) - pathology , medicine , epithelioid cell , granuloma , melkersson–rosenthal syndrome , immunohistochemistry , dermatology
Melkersson Rosenthal syndrome (MRS) is rare disease of unknown etiology characterized by orofacial edema, facial nerve palsy and fissured tongue. Microscopically, it shows epithelioid non‐caseous granulomas; however, edema and perivascular lymphocytic infiltrates have been described. Two different clinical forms of MRS are presented in this report. In the complete form (Case 1), the main histopathologic finding was a non‐necrotizing granulomatous inflammation with 56% of the total number of cells composed of B cells (CD 20 + ) principally located in the granuloma's center and 33% being T cells predominating in the surrounding area, of which 48% were CD 4 + and 16% were CD 8 + lymphocytes. In the monosymptomatic form (Case 2), the inflammatory cells were dispersed into the connective tissue without granulomatous formation. B cells were scanty, and 78% of the cells were CD 45 + T cells, with 46% and 34%, CD 8 + and CD 4 + phenotype, respectively. These cases showed different clinical, histopathological and immunohistochemical forms of MRS, suggesting different host immune responses. Kaminagakura E, Jorge J Jr. Melkersson Rosenthal syndrome: a histopathologic mystery and dermatologic challenge.