Mutizwa Misha M, Tang Mark B, Ng See Ket
Division of Dermatology, Washington University School of Medicine, St. Louis, Missouri, USA.
Dermatol Online J. 2010 Jul 15;16(7):5.
Malignant atrophic papulosis, commonly known as Degos disease, is a rare vasculopathy encompassing both benign, cutaneous and lethal systemic variants. We report a case of chronic cutaneous lupus erythematosus in a 41-year-old male presenting with prominent Degos-like skin lesions. Multiple atrophic, porcelain-white, scar-like papules and plaques with dusky, erythematous borders, suggestive of malignant atrophic papulosis, were noted on the patient's back. Additional cutaneous findings included photo-distributed facial erythema and discoid lupus-like plaques on the face, shoulders, and arms. Clinicopathological correlation supported a diagnosis of chronic cutaneous lupus erythematosus; hydroxychloroquine was initiated with good clinical response. No new or active lesions were observed at the sixteen-month follow-up. This case highlights a rare skin finding associated with chronic cutaneous lupus erythematosus and underscores the importance of ruling out primary autoimmune disease, particularly lupus, before a diagnosis of malignant atrophic papulosis can be made.
恶性萎缩性丘疹病，通常称为德戈斯病，是一种罕见的血管病，包括良性皮肤型和致死性全身型。我们报告一例41岁男性慢性皮肤红斑狼疮患者，其出现显著的德戈斯样皮肤病变。在患者背部发现多个萎缩性、瓷白色、瘢痕样丘疹和斑块，边界呈暗红斑状，提示恶性萎缩性丘疹病。其他皮肤表现包括面部光暴露部位红斑以及面部、肩部和手臂的盘状狼疮样斑块。临床病理相关性支持慢性皮肤红斑狼疮的诊断；开始使用羟氯喹治疗，临床反应良好。在16个月的随访中未观察到新的或活动性病变。该病例突出了与慢性皮肤红斑狼疮相关的一种罕见皮肤表现，并强调在诊断恶性萎缩性丘疹病之前排除原发性自身免疫性疾病，尤其是狼疮的重要性。
