Matsuo Toshihiko, Tanaka Takehiro, Asada Noboru, Obika Mikako, Omichi Ryotaro, Iwatsuki Keiji
Department of Ophthalmology, Graduate School of Interdisciplinary Science and Engineering in Health Systems, Okayama University, and Okayama University Hospital, Okayama, JPN.
Department of Pathology, Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama University, Okayama, JPN.
Cureus. 2025 Apr 16;17(4):e82348. doi: 10.7759/cureus.82348. eCollection 2025 Apr.
Pyoderma gangrenosum is a non-infectious autoimmune disease with skin plaques and ulcers in the entity of neutrophilic dermatosis and may have a background of myelodysplastic syndromes. This study reported a 20-year follow-up of a patient with pyoderma gangrenosum and scleritis who showed chromosomal aberrancy from the initial phase and later in the course developed thrombocythemia. A 51-year-old man presented with widespread indurated erythematous plaques with scaling and pustules on the forehead, bilateral eyelids, and nasal bridge, in addition to nodular scleritis in the left eye and ulcer formation of the plaques in the lower legs. Skin biopsy revealed massive dermal infiltration mainly with neutrophils in the absence of neutrophilic vasculitis. Suspected of myelodysplastic syndromes, bone marrow biopsy was normal, while chromosomal aberrancy, 46, XY, del (20) (q11q13.3), was detected. In the diagnosis of neutrophilic dermatosis, probably of pyoderma gangrenosum, he began to have oral prednisolone 20 mg daily and colchicine 1 mg daily, leading to the subsidence of skin lesions. Four months later, he developed nodular scleritis in the right eye and began to use topical 0.1% betamethasone in both eyes. He was stable with only prednisolone 12.5 mg daily until the age of 55.5 years, when he showed an increase of serum lactate dehydrogenase. The bone marrow aspirate disclosed neither blast cell increase nor atypical cells. The same chromosomal aberrancy was repeatedly detected. One year later, he developed breathing difficulty and underwent tracheostomy. Laryngeal lesion biopsy disclosed squamous cell papilloma with human papillomavirus-6. At 60 years old, he showed marginal corneal infiltration in the left eye, and at 61 years old, hypopyon in the right eye. Platelets tended to increase up to 1000 × 10/µL, and bone marrow examinations were recommended but refused by the patient. At the latest follow-up at 71 years old, he was ambulatory in health and stable with a tracheostomy cannula. In conclusion, pyoderma gangrenosum with scleritis occurred in an undetermined hematological malignancy with chromosomal aberrancy.
坏疽性脓皮病是一种非感染性自身免疫性疾病，属于嗜中性皮病范畴，表现为皮肤斑块和溃疡，可能伴有骨髓增生异常综合征背景。本研究报告了一名患有坏疽性脓皮病和巩膜炎的患者长达20年的随访情况，该患者从疾病初期就出现染色体异常，随后病程中发展为血小板增多症。一名51岁男性，前额、双侧眼睑和鼻梁出现广泛的硬结性红斑斑块，伴有鳞屑和脓疱，此外左眼患有结节性巩膜炎，小腿斑块形成溃疡。皮肤活检显示真皮大量浸润，主要为中性粒细胞浸润，无嗜中性血管炎。怀疑患有骨髓增生异常综合征，骨髓活检正常，但检测到染色体异常，核型为46, XY, del(20)(q11q13.3)。在诊断为可能是坏疽性脓皮病的嗜中性皮病后，他开始每日口服泼尼松龙20 mg和秋水仙碱1 mg，皮肤病变逐渐消退。四个月后，他右眼出现结节性巩膜炎，开始双眼使用0.1%倍他米松滴眼液。在55.5岁之前，他仅每日服用12.5 mg泼尼松龙病情稳定，此时他血清乳酸脱氢酶升高。骨髓穿刺未发现原始细胞增多或非典型细胞。反复检测到相同的染色体异常。一年后，他出现呼吸困难并接受了气管切开术。喉部病变活检显示为伴有6型人乳头瘤病毒的鳞状细胞乳头瘤。60岁时，他左眼出现角膜边缘浸润，61岁时右眼出现前房积脓。血小板计数逐渐升高至1000×10⁹/L，建议进行骨髓检查，但患者拒绝。在71岁的最后一次随访中，他健康状况尚可，带着气管切开套管病情稳定。总之，坏疽性脓皮病合并巩膜炎发生于一种伴有染色体异常的未明确血液系统恶性肿瘤患者。
