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Published on: September 20, 2018
Bowel-associated dermatosis-arthritis syndrome in an adolescent with short bowel syndrome
Ester Pereira1, Paula Estanqueiro, Susana Almeida
1From the *Hospital Pediátrico de Coimbra and †Department of Dermatology, Centro Hospitalar e Universitário de Coimbra, EPE, Coimbra, Portugal.
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.
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