Abstract：
UNASSIGNED: Mucha-Habermann disease (MHD) is an inflammatory skin disease characterized by polymorphous eruptions of erythematous, necrotic macules that have been reported for similarities to cutaneous T-cell lymphoma. Febrile ulceronecrotic MHD (FUMHD) represents a severe variant of MHD, marked by ulcers, hemorrhagic bullae, and systemic symptoms. Herein, we report a case of a severely atypical lymphomatoid expression of FUMHD associated with hemophagocytic lymphohistiocytosis (HLH). A previously healthy 21-year-old woman was admitted to the hospital with a rapidly progressive necrotic papular rash. Physical examination revealed right orbital swelling, bilateral hemorrhagic auricular bullae, and multiple ulcerative purpuric papulonodules on the trunk, face, and extremities. Biopsy indicated a dermal and subcutaneous infiltrate of atypical CD8 + lymphocytes with loss of CD5 and reduction in CD7 expression, along with features of lymphomatoid vasculitis. A diagnosis of a severely atypical lymphomatoid expression of FUMHD was made. The patient also met 7 of 9 HLH-2004 criteria, leading to a diagnosis of HLH. Positron emission tomography/computed tomography, flow cytometry, and rheumatologic workup were unremarkable. Treatment with an eight-week course of etoposide and dexamethasone for HLH led to rapid clinical improvement. Over time, her skin lesions regressed and eventually scabbed over to leave hyperpigmented scars, confirming the diagnosis of MHD. She has remained stable, off therapy for 4 years. Although potentially fatal, FUMHD often exhibits favorable outcomes and may resolve without recurrence, as in our patient. FUMHD should be considered in the differential diagnosis for patients presenting with cutaneous CD8 + necrotizing angiocentric lymphoproliferative disease complicated by HLH.
摘要：
■Mucha-Habermann病（MHD）是一种炎症性皮肤病，其特征是红斑的多形性爆发，据报道与皮肤T细胞淋巴瘤相似的坏死斑。发热性溃疡性MHD（FUMHD）代表MHD的严重变异，以溃疡为标志，出血性大疱,和全身症状。在这里，我们报告一例FUMHD的严重非典型淋巴瘤样表达与噬血细胞性淋巴组织细胞增多症（HLH）相关。一名先前健康的21岁妇女因快速进行性坏死性丘疹入院。体格检查发现右眶肿胀,双侧出血性耳大疱，躯干上有多个溃疡性紫癜性丘疹，脸,和四肢。活检显示皮肤和皮下有非典型CD8+淋巴细胞浸润，CD5缺失，CD7表达减少，以及淋巴瘤样血管炎的特征。诊断为FUMHD的严重非典型淋巴瘤样表达。患者还符合9项HLH-2004标准中的7项，导致HLH的诊断。正电子发射断层扫描/计算机断层扫描，流式细胞术,风湿病检查并不明显。用依托泊苷和地塞米松治疗HLH的八周疗程导致快速的临床改善。随着时间的推移,她的皮肤损伤消退，最终结痂留下色素沉着的疤痕，确认MHD的诊断。她一直保持稳定,停止治疗4年。虽然可能致命，FUMHD通常表现出良好的结果，并且可能在不复发的情况下消退，就像我们的病人一样。对于患有皮肤CD8坏死性血管中心性淋巴增生性疾病并伴有HLH的患者，应在鉴别诊断中考虑FUMHD。
