Abstract:
Neutrophilic dermatosis of the dorsal hands (NDDH) is a variant of Sweet syndrome that presents with erythematous bullae, papules/plaques, or pustules on the dorsal hands. It is most commonly associated with hematologic and solid organ malignancies, though cases of NDDH associated with inflammatory bowel disease, rheumatologic disorders, and medication exposure have also been described in the literature. Felty syndrome is a rare complication of long-standing rheumatoid arthritis characterized by neuropathy, splenomegaly, and neutropenia. Granulocyte colony stimulating factors (e.g., filgrastim) can be utilized to rescue the neutropenia observed in Felty syndrome, but this treatment may subsequently cause Sweet syndrome. Herein, we present a 64-year-old man with Felty syndrome and a complex medical history who presented with sudden onset, painful blisters located on the dorsal and palmar aspects of his bilateral hands. Given the patient\'s past medical history, a broad differential diagnosis, including disseminated fungal and viral infection was initially considered. A punch biopsy of the skin lesion disclosed neutrophilic dermatosis, which together with laboratory data satisfied the von den Driesch criteria for Sweet syndrome. As the lesions were localized exclusively on the patient\'s hands, the qualification of NDDH was also endorsed.
摘要:
背手嗜中性粒细胞性皮肤病(NDDH)是Sweet综合征的一种变体,表现为红斑性大疱,丘疹/斑块,或背侧的脓疱。它最常见的与血液和实体器官恶性肿瘤有关,虽然与炎症性肠病相关的NDDH病例,风湿病,和药物暴露在文献中也有描述。Felty综合征是以神经病为特征的长期类风湿性关节炎的罕见并发症,脾肿大,和中性粒细胞减少症.粒细胞集落刺激因子(例如,非格司亭)可用于挽救在Felty综合征中观察到的中性粒细胞减少症,但这种治疗可能会导致Sweet综合征。在这里,我们介绍了一个64岁的男性,患有Felty综合征和复杂的病史,他突然发作,疼痛的水泡位于他的双侧手的背侧和手掌方面。鉴于患者的既往病史,广泛的鉴别诊断,最初考虑了包括播散性真菌和病毒感染。皮肤病变的穿刺活检显示嗜中性皮肤病,与实验室数据一起满足了斯威特综合征的vondenDriesch标准。由于病变仅位于患者的手上,NDDH的资格也得到认可。