Cutaneous sclerosis: a previously undescribed manifestation of sclerosing mesenteritis.

BACKGROUND

Sclerosing mesenteritis is a rare disease of unknown etiology that is characterized by self-limited, nonspecific inflammation and fibrosis of the mesenteric adipose tissue. Histologic classification characterizes 3 main stages in the evolution of the fibroinflammatory process: mesenteric lipodystrophy (ML), mesenteric panniculitis (MP), and sclerosing (retractile) mesenteritis (SM).

OBSERVATIONS: A 68-year-old woman with biopsy-proven MP presented with multiple asymptomatic, indurated subcutaneous nodules on both arms, as well as 2 indurated plaques on her abdomen. The cutaneous changes preceded the diagnosis of SM by roughly 3 years. The arm lesions were centrally depressed with a prominent groove and a peau d'orange appearance. Biopsy findings revealed a subcutaneous process with almost total replacement of adipocytes by zones of woody sclerosis and fat necrosis identical to that observed in the mesentery. To our knowledge, this manifestation of sclerosing mesenteritis has not been reported previously.

CONCLUSIONS

Sclerosing mesenteritis has rarely been associated with extra-abdominal idiopathic fibrosclerotic disorders, but a cutaneous component of SM has never been characterized. The fact that the cutaneous lesions were histopathologically identical to the mesenteric changes and their presence prior to the recognition of intra-abdominal disease suggests that future patients with such lesions might be evaluated for this disorder leading to earlier recognition.