Melkersson-Rosenthal Syndrome with Genitalia Involved in a 12-Year-Old Boy
Annals of Dermatology
; : 232-236, 2016.
Article
em En
| WPRIM
| ID: wpr-136926
Biblioteca responsável:
WPRO
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.
Palavras-chave
Texto completo:
1
Índice:
WPRIM
Assunto principal:
Pênis
/
Língua Fissurada
/
Biópsia
/
Doença de Crohn
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Testes Genéticos
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Seguimentos
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Vasos Linfáticos
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Diagnóstico
/
Edema
/
Paralisia Facial
Tipo de estudo:
Diagnostic_studies
/
Observational_studies
/
Prognostic_studies
Limite:
Child
/
Humans
/
Male
Idioma:
En
Revista:
Annals of Dermatology
Ano de publicação:
2016
Tipo de documento:
Article