A case of intestinal Behçet’s disease with pyoderma gangrenosum initially treated as Sweet’s syndrome

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  • Sato Noriko
    Department of Pediatrics, Graduate School of Medicine, Chiba University
  • Yamaide Fumiya
    Department of Pediatrics, Graduate School of Medicine, Chiba University
  • Aoyagi Naoki
    Department of Dermatology, Graduate School of Medicine, Chiba University
  • Shibata Ryohei
    Department of Pediatric Surgery, Graduate School of Medicine, Chiba University
  • Nakano Taiji
    Department of Pediatrics, Graduate School of Medicine, Chiba University
  • Saito Takeshi
    Department of Pediatric Surgery, Graduate School of Medicine, Chiba University
  • Shimojo Naoki
    Department of Pediatrics, Graduate School of Medicine, Chiba University

Bibliographic Information

Other Title
  • 壊疽性膿皮症を合併しSweet症候群として初期治療を行った腸管ベーチェット病の1 例
  • 症例報告 壊疽性膿皮症を合併しSweet症候群として初期治療を行った腸管ベーチェット病の1例
  • ショウレイ ホウコク エソセイ ノウ ヒショウ オ ガッペイ シ Sweet ショウコウグン ト シテ ショキ チリョウ オ オコナッタ チョウカン ベーチェットビョウ ノ 1レイ

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Description

Pyoderma gangrenosum （PG） is a sterile pyoderma that initially forms small pustules and papules predominantly on the lower extremities, and rapidly evolves to ulcers. It is often associated with inflammatory bowel disease, arthritis or vasculitis. A 2-year-old girl presented with a two-week history of fever, tonsillitis, mild abdominal pain and a skin ulcer on her left lower leg. Initially, a small pustule formed on her leg, for which oral antibiotics were not effective. Subsequently, the skin lesion ulcerated. The ulcer improved with cleaning and the application of an ointment （Sulfadiazine silver）, but she showed pathergy and sterile abscesses developed on her finger and lips. Because of the absence of HLA-A26 and B51, major symptoms of Behçet’s disease（ eg. genital ulcer or uveitis）, and vasculitis, we diagnosed Sweet’s syndrome at the first medical examination. After starting prednisolone, all of her symptoms improved. However during the tapering of prednisolone, her symptoms, especially abdominal pain, recurred. Multiple ulcers on her colon were observed during gastrointestinal endoscopy, and we revised the diagnosis to intestinal Behçet’s disease. As the features of skin pathologies of Behçet’s disease, Sweet’s syndrome and PG are similar to each other, attention should be given to the changes in symptoms and diagnosis and treatment should be reviewed. 9：70-75

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