Williams Charles A, Merkel Kimberly L
Division of Genetics and Metabolism, Department of Pediatrics, College of Medicine, University of Florida, Gainesville, Florida.
Pediatr Dermatol. 2014 Nov-Dec;31(6):e140-4. doi: 10.1111/pde.12426. Epub 2014 Sep 18.
A 14-year-old boy presented with a chronic history of atypical papular mucinosis consisting of multiple subcutaneous nodules and confluent papular skin lesions. He initially presented at age 2 years with the rapid onset of numerous subcutaneous nodular lesions that completely resolved over a period of years. Clinical and histologic evidence, together with his clinical course, were suggestive of self-healing juvenile cutaneous mucinosis (SHJCM), but a few years later, during childhood, he experienced a recurrence of the subcutaneous nodules involving the limbs, trunk, and face, in addition to new findings of multiple flesh-colored papules coalescing into plaques on his neck and back. Although his early childhood course and histologic picture were suggestive of SHJCM, the progressive nature of his disorder is not like that seen in SHJCM and appears different from other reported disorders involving cutaneous mucinosis.
一名14岁男孩,有非典型丘疹性粘蛋白病的慢性病史,表现为多个皮下结节和融合性丘疹性皮肤病变。他最初在2岁时发病,迅速出现大量皮下结节性病变,这些病变在数年内完全消退。临床和组织学证据以及他的临床病程提示为自愈性青少年皮肤粘蛋白病(SHJCM),但几年后,在儿童期,他的皮下结节复发,累及四肢、躯干和面部,此外,颈部和背部还出现了新的多个肉色丘疹融合成斑块的表现。尽管他幼儿期的病程和组织学表现提示为SHJCM,但他疾病的进行性特征与SHJCM所见不同,且似乎与其他报道的涉及皮肤粘蛋白病的疾病不同。