title: Bowel-associated dermatosis-arthritis syndrome in an adolescent with short bowel syndrome.
abstract: Bowel-associated dermatosis-arthritis syndrome (BADAS) is a neutrophilic dermatosis, characterized by the occurrence of arthritis and skin lesions related to bowel disease with or without bowel bypass. We report an unusual case of BADAS in a 15-year-old white male with congenital aganglionosis of the colon and hypoganglionosis of the small intestine and multiple bowel surgeries in childhood complicated by short bowel syndrome. He presented with recurrent peripheral polyarthritis, tenosynovitis, and painful erythematous subcutaneous nodules located on the dorsolateral regions of the legs and on the dorsa of the feet. Histological examination disclosed a neutrophilic dermatosis confirming the diagnosis of BADAS.Although an uncommon disease, especially at pediatric age, it is important to evoke the diagnosis of BADAS in children and adolescents with bowel disease, because treatment options and prognosis are distinct from other rheumatologic conditions.
authors.affiliation: From the *Hospital Pediátrico de Coimbra and †Department of Dermatology, Centro Hospitalar e Universitário de Coimbra, EPE, Coimbra, Portugal.
authors.forename: Ester
authors.initials: E
authors.lastname: Pereira
authors.forename: Paula
authors.initials: P
authors.lastname: Estanqueiro
authors.forename: Susana
authors.initials: S
authors.lastname: Almeida
authors.forename: Ricardo
authors.initials: R
authors.lastname: Ferreira
authors.forename: Oscar
authors.initials: O
authors.lastname: Tellechea
authors.forename: Manuel
authors.initials: M
authors.lastname: Salgado
mesh_terms: D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D001168:Arthritis / Q000175:diagnosis* / Q000188:drug therapy / Q000453:epidemiology; D015897:Comorbidity; D006627:Hirschsprung Disease / Q000175:diagnosis* / Q000188:drug therapy / Q000453:epidemiology; D006801:Humans; D008297:Male; D012778:Short Bowel Syndrome / Q000175:diagnosis* / Q000188:drug therapy / Q000453:epidemiology; D012871:Skin Diseases / Q000175:diagnosis* / Q000188:drug therapy / Q000453:epidemiology; D013717:Tenosynovitis / Q000175:diagnosis* / Q000188:drug therapy / Q000453:epidemiology; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones; D000900:Anti-Bacterial Agents; D000894:Anti-Inflammatory Agents, Non-Steroidal