al-Rimawi H S, Abuekteish F M, Daoud A S, Oboosi M M
Department of Paediatrics, Jordan University of Science and Technology, Irbid, Jordan.
Eur J Pediatr. 1996 Sep;155(9):759-62. doi: 10.1007/BF02002902.
Pyoderma gangrenosum (PG) is a rare, poorly understood skin disease that occurs in all age groups. Less than 0.4% of patients are infants and represent a diagnostic challenge as early lesions may resemble other skin disorders. Here we report for the first time three siblings affected with PG all presenting during infancy. Unlike the older age group, the ulcers spared the legs but involved the buttocks, thighs and perianal area in all the infants.
This is the first reported family with PG affecting three siblings suggesting autosomal recessive inheritance. The diagnosis may be more difficult in infants due to absence of underlying associated disorders and the tendency of the lesions to appear in areas where infants frequently have other dermatoses. PG characteristically involves the buttocks, thighs and perianal area and spares the legs.
坏疽性脓皮病（PG）是一种罕见且了解甚少的皮肤病，可发生于所有年龄组。不到0.4%的患者为婴儿，由于早期病变可能类似于其他皮肤病，因此对其诊断具有挑战性。在此，我们首次报告了三名在婴儿期均出现PG的兄弟姐妹。与年龄较大的群体不同，所有婴儿的溃疡均未累及腿部，而是累及臀部、大腿和肛周区域。
这是首次报道的一个患有PG的家庭，有三名兄弟姐妹患病，提示为常染色体隐性遗传。由于婴儿不存在潜在的相关疾病，且病变倾向于出现在婴儿经常出现其他皮肤病的部位，因此婴儿的诊断可能更困难。PG的特征是累及臀部、大腿和肛周区域，不累及腿部。
