Mullangi Samyukta, Granter Scott R, Laubach Jacob P, Lipworth Adam D
Brigham and Women's Hospital.
Dermatol Online J. 2014 Dec 31;21(3):13030/qt1ps3z09r.
Scleromyxedema is a generalized and progressive fibromucinous disorder associated with substantial cutaneous and systemic morbidity. The diagnosis is often challenging, as is management. We present here a patient with scleromyxedema with atypical, granuloma annulare-like histology, which contributed to delayed diagnosis and management, including a delayed workup for multiple myeloma. Ultimately, the patient did well with appropriate therapy, but his presentation illustrates the importance of more widespread familiarity among dermatologists and dermatopathologists with this variant of scleromyxedema.
硬化性黏液水肿是一种全身性、进行性纤维黏液性疾病,伴有严重的皮肤和全身病变。其诊断往往具有挑战性,治疗也是如此。我们在此介绍一名患有硬化性黏液水肿的患者,其组织学表现不典型,类似环状肉芽肿,这导致了诊断和治疗的延迟,包括对多发性骨髓瘤的延迟检查。最终,患者在接受适当治疗后情况良好,但他的病例表明皮肤科医生和皮肤病理学家更广泛地熟悉这种硬化性黏液水肿变体的重要性。