Lee Jack, Flowers Richard H, Cocks Margaret M, Noland Mary-Margaret B
Department of Dermatology, University of Virginia, Charlottesville, Virginia.
Department of Pathology, University of Virginia, Charlottesville, Virginia.
J Cutan Pathol. 2018 Dec;45(12):944-948. doi: 10.1111/cup.13357. Epub 2018 Oct 9.
We report the case of a 40-year-old African-American female with biopsy-proven pulmonary sarcoidosis who developed atrophic plaques on her shins, trunk, and scalp that were clinically and histologically consistent with necrobiosis lipoidica (NL). The lesions appeared 3 years after her diagnosis of sarcoidosis, and progressed despite chronic prednisone. Sarcoidosis and NL are granulomatous skin disorders reported to coexist in the same patient only 10 times in the literature. Including the current case, patients have been exclusively females around middle age, and have greater tendencies to develop typical cutaneous sarcoidosis. The incidence of diabetes is rare in this group. Like typical NL, NL associated with sarcoidosis tends to ulcerate, and is difficult to treat. Interestingly, there are six similar cases reported in the literature of patients with sarcoidosis who developed lesions clinically and behaviorally consistent with NL, but received a final histological diagnosis of sarcoidosis. These cases share very similar demographics and clinical features with cases of true NL associated with sarcoidosis, and often have more ambiguous histology containing features of both cutaneous sarcoidosis and NL. Comparing the two sets of cases raises the possibility of a final common disease pathway shared by these two granulomatous skin disorders.
我们报告了一例40岁的非裔美国女性,经活检证实患有肺结节病,其小腿、躯干和头皮出现萎缩性斑块,临床和组织学表现与类脂质渐进性坏死(NL)一致。这些病变在她被诊断为结节病3年后出现,尽管长期使用泼尼松治疗仍有进展。结节病和NL是肉芽肿性皮肤病,据文献报道,二者在同一患者中共存仅有10例。包括本例在内,患者均为中年女性,且更易发生典型的皮肤结节病。该组患者中糖尿病的发病率较低。与典型的NL一样,与结节病相关的NL往往会发生溃疡,且难以治疗。有趣的是,文献中报道了6例类似病例,这些结节病患者出现了临床和表现与NL一致的病变,但最终组织学诊断为结节病。这些病例与与结节病相关的真正NL病例具有非常相似的人口统计学和临床特征,且组织学表现往往更模糊,同时包含皮肤结节病和NL的特征。比较这两组病例,提示这两种肉芽肿性皮肤病可能存在最终的共同疾病途径。