Choi Hye-Jin, Chang Sung-Eun, Lee Mi-Woo, Choi Jee-Ho, Moon Kee-Chan, Koh Jai-Kyoung
Department of Dermatology, Asan Medical Center, College of Medicine, University of Ulsan, Seoul, South Korea.
Int J Dermatol. 2006 Apr;45(4):457-9. doi: 10.1111/j.1365-4632.2004.02376.x.
An 80-year-old man presented with multiple erythematous papules on the trunk and extremities of a few weeks' duration. He had no past medical or family history of skin diseases or any other medical diseases. A biopsy showed a perivascular lymphohistiocytic infiltrate and sparse neutrophils with several atypical lymphocytes in the deeper dermis. With an initial diagnosis of T-cell pseudolymphoma or unspecified neutrophilic dermatosis, he showed a brisk response to an intramuscular injection of triamcinolone acetonide (40 mg/mL). After 1 month, his skin lesion recurred. Steroid was given with a good clinical response. One month later, however, his skin lesion relapsed. At this time, he presented with disseminated pustulopapular lesions on the trunk and extremities. Examination revealed multiple, variable-sized, erythematous plaques with central pustules on the extremities (Fig. 1). The mucous membranes were not involved. He had no pain or tenderness. He had no systemic symptoms. Laboratory tests showed a hemoglobin level of 10.3 g/dL, a leukocyte level of 6,900/mm(3), with an increased proportion of segmented nuclear neutrophils (83%), and an elevated C-reactive protein. A skin biopsy revealed a dense perivascular and interstitial infiltrate composed of neutrophils with marked dermal edema (Fig. 2). Sweet's syndrome was the final diagnosis and he was treated with oral prednisolone (30-40 mg/day) and dapsone (50 mg/day) for 2 months. As this 80-year-old patient had a recurrent history of similar skin lesions and anemia, an underlying hematologic malignancy was suspected. A bone marrow biopsy showed typical myelodysplastic syndrome (MDS). The hemoglobin level was decreased to 5.3 g/dL during a follow-up period of 5 months. The skin lesions recurred despite oral steroids and dapsone. The patient received only symptomatic treatment, such as a transfusion, for the underlying malignancy MDS.
一名80岁男性,躯干和四肢出现多处红斑丘疹,持续数周。他既往无皮肤疾病或任何其他内科疾病的病史及家族史。活检显示真皮深层血管周围淋巴细胞组织细胞浸润,伴有稀疏的中性粒细胞及数枚非典型淋巴细胞。初步诊断为T细胞假性淋巴瘤或未明确的嗜中性皮病,他对肌肉注射曲安奈德(40mg/mL)反应迅速。1个月后,皮肤病变复发。给予类固醇治疗,临床反应良好。然而,1个月后,皮肤病变再次复发。此时,他躯干和四肢出现播散性脓疱丘疹性病变。检查发现四肢有多个大小不一的红斑斑块,中央有脓疱(图1)。黏膜未受累。他无疼痛或压痛。无全身症状。实验室检查显示血红蛋白水平为10.3g/dL,白细胞水平为6900/mm³,分叶核中性粒细胞比例增加(83%),C反应蛋白升高。皮肤活检显示由中性粒细胞组成的密集血管周围和间质浸润,伴有明显的真皮水肿(图2)。最终诊断为Sweet综合征,给予口服泼尼松龙(30 - 40mg/天)和氨苯砜(50mg/天)治疗2个月。由于这位80岁患者有类似皮肤病变和贫血的复发史,怀疑存在潜在的血液系统恶性肿瘤。骨髓活检显示为典型的骨髓增生异常综合征(MDS)。在5个月的随访期间,血红蛋白水平降至5.3g/dL。尽管使用了口服类固醇和氨苯砜,皮肤病变仍复发。患者仅接受了针对潜在恶性肿瘤MDS的对症治疗,如输血。