Department of Hematology, The First People's Hospital of Yunnan Province, Yunnan Province Clinical Research Center for Hematologic Disease, Yunnan Province Clinical Center for Hematologic Disease, Kunming, 650032 Yunnan, China.
Department of Hematology, The Affiliated Hospital of Kunming University of Science and Technology, Kunming, 650032 Yunnan, China.
Medicine (Baltimore). 2024 Mar 8;103(10):e37159. doi: 10.1097/MD.0000000000037159.
Pyoderma gangrenosum (PG) is a rare noninfectious neutrophilic skin disease. The diagnosis of PG is mainly based on clinical manifestations. Therefore, the clinical features of PG are important for confirming the diagnosis of this disease. Herein, the clinical data of 2 young males with PG complicated with hematological malignancies were reported, and the literature were reviewed.
The first case was a 22-year-old male who was admitted due to a systemic rash, headache, and fever. Physical examination showed black scabs on the skins of the extremities, trunk, scalp, and face. Biopsy of the skin lesion showed epidermal edema, spongy formation, neutrophil infiltration, acute and chronic inflammatory cell infiltration in the dermis, showing purulent inflammation with epidermal erosion. The bone marrow biopsy showed obviously active proliferation of nucleated cells, granulocytes at various stages, abnormal morphological neutrophils, and occasionally observed young red blood cells. The diagnosis of PG and chronic myelomonocytic leukemia (CMML-0) was made. The second case was a 28-year-old male who presented a swollen, painful right calf following injury and then developed ulcers on skin and soft tissues. Bone marrow biopsy showed obviously active nucleated cell proliferation, suggesting a myeloid tumor. He was also diagnosed with PG and hematological malignancies. They both received hormone and antiinfection therapy. After treatment, their body temperature, infection, and skin lesions were improved. However, both of them were readmitted and had a poor prognosis.
PG may be associated with hematological malignancies. For patients with typical skin lesions and obvious abnormal blood routines, it is necessary to investigate the possibility of PG with hematological malignancies.
坏疽性脓皮病(PG)是一种罕见的非传染性中性粒细胞皮肤病。PG 的诊断主要基于临床表现。因此,PG 的临床特征对于确认该疾病的诊断很重要。本文报告了 2 例伴有血液系统恶性肿瘤的 PG 年轻男性患者的临床资料,并复习了相关文献。
第 1 例患者为 22 岁男性,因全身皮疹、头痛和发热入院。体格检查显示四肢、躯干、头皮和面部有黑色痂皮。皮肤病变活检显示表皮水肿、海绵形成、中性粒细胞浸润,真皮内急性和慢性炎症细胞浸润,表现为表皮侵蚀的脓性炎症。骨髓活检显示核细胞明显活跃增殖,各阶段粒细胞、形态异常的中性粒细胞,偶尔观察到年轻红细胞。诊断为 PG 和慢性粒单核细胞白血病(CMML-0)。第 2 例患者为 28 岁男性,受伤后右小腿肿胀、疼痛,随后皮肤和软组织出现溃疡。骨髓活检显示核细胞明显活跃增殖,提示髓系肿瘤。他还被诊断为 PG 和血液系统恶性肿瘤。他们都接受了激素和抗感染治疗。治疗后,他们的体温、感染和皮肤病变得到改善。然而,他们都再次入院,预后不良。
PG 可能与血液系统恶性肿瘤有关。对于具有典型皮肤病变和明显异常血常规的患者,有必要调查是否存在伴有血液系统恶性肿瘤的 PG 的可能性。