title: Melkersson-Rosenthal Syndrome with Genitalia Involved in a 12-Year-Old Boy.
abstract: Melkersson-Rosenthal syndrome (MRS) is an uncommon granulomatous disease characterized by the triad of relapsing facial paralysis, orofacial swelling, and fissured tongue. Genital swelling in MRS is rarely reported. We presented the first case of complete MRS with genital swelling in a child. Biopsy examinations of both the child's lower lip and penis showed noncaseating granuloma and intralymphatic granuloma infiltration. No symptoms or signs of other systemic disease (Crohn's disease or sarcoidosis) were observed after 2 years of follow-up. Genetic screening for CARD15/NOD2 in this patient showed negative, which further confirmed the diagnosis of MRS. Eleven other cases of suspected complete or incomplete MRS with genitalia involved were reviewed. Our case emphasizes the specific clinical feature of MRS with genitalia involved, which was genetically different from Crohn's disease and could be an independent entity. Lymphatic obstruction is responsible for localized edema in MRS.
authors.affiliation: Department of Dermatology, Second Affiliated Hospital of Xi'an Jiaotong University, Xi'an, China.
authors.forename: Zhaowei
authors.initials: Z
authors.lastname: Chu
authors.affiliation: Department of Dermatology, Second Affiliated Hospital of Xi'an Jiaotong University, Xi'an, China.
authors.forename: Yanting
authors.initials: Y
authors.lastname: Liu
authors.affiliation: Department of Dermatology, Second Affiliated Hospital of Xi'an Jiaotong University, Xi'an, China.
authors.forename: Huan
authors.initials: H
authors.lastname: Zhang
authors.affiliation: Department of Dermatology, Second Affiliated Hospital of Xi'an Jiaotong University, Xi'an, China.
authors.forename: Weihui
authors.initials: W
authors.lastname: Zeng
authors.affiliation: Department of Dermatology, Second Affiliated Hospital of Xi'an Jiaotong University, Xi'an, China.
authors.forename: Songmei
authors.initials: S
authors.lastname: Geng
keywords: Genitalia; Lymphatic vessels; Melkersson–Rosenthal syndrome