Chadli Sarra, Maamar Mouna, Khibri Hajar, Harmouche Hicham, Tazi Mezalek Zoubida
Internal Medicine, Ibn Sina University Hospital, Mohammed V University, Rabat, MAR.
Hematology, Ibn Sina University Hospital, Mohammed V University, Rabat, MAR.
Cureus. 2024 Apr 23;16(4):e58838. doi: 10.7759/cureus.58838. eCollection 2024 Apr.
While Pyoderma gangrenosum (PG) is commonly associated with hematological disorders such as acute myeloid leukemia (AML), it typically presents concurrently with the hemopathy, mostly in its bullous form, among middle-aged individuals. Here, we report the unusual case of a young female patient who presented with PG in its ulcerative form, three weeks before the onset of AML. A 31-year-old female presented with a one-week history of painful perianal papulopustule that evolved into an irregular ulceration with violaceous borders, mucopurulent serosity, and erythematous surrounding skin. Laboratory work-up demonstrated elevated inflammatory markers and hyperleukocytosis, with no cytopenia, and normal peripheral blood smear. Two weeks later, the ulcer growth was noted with a similar ulceration at a venipuncture site. A complete blood count revealed pancytopenia, with 45% blasts on the peripheral blood smear. Skin biopsies showed an aseptic neutrophilic infiltrate in favor of PG. Intravenous methylprednisolone was administered with rapid resolution of the lesions. However, the patient died shortly after. The post-mortem results of bone marrow aspirate revealed AML, with immunohistochemistry of the skin lesions confirming the clonality of neutrophils derived from the leukemic clone. This case highlights a distinctive clinical presentation, illustrating the manifestation of PG three weeks before the onset of AML in its ulcerative rather than bullous form, in a young female patient.
坏疽性脓皮病(PG)通常与血液系统疾病如急性髓系白血病(AML)相关,在中年个体中,它通常与血液病同时出现,大多表现为大疱性。在此,我们报告一例年轻女性患者的罕见病例,该患者在AML发病前三周出现溃疡性PG。一名31岁女性,有一周肛周疼痛性丘疹脓疱病史,后发展为边界呈紫罗兰色、有黏液脓性浆液及周围皮肤红斑的不规则溃疡。实验室检查显示炎症标志物升高和白细胞增多,无血细胞减少,外周血涂片正常。两周后,发现溃疡扩大,静脉穿刺部位出现类似溃疡。全血细胞计数显示全血细胞减少,外周血涂片可见45%的原始细胞。皮肤活检显示无菌性中性粒细胞浸润,支持PG诊断。给予静脉注射甲泼尼龙后,病变迅速消退。然而,患者不久后死亡。骨髓穿刺的尸检结果显示为AML,皮肤病变的免疫组化证实了源自白血病克隆的中性粒细胞的克隆性。该病例突出了一种独特的临床表现,即在一名年轻女性患者中,PG以溃疡性而非大疱性形式在AML发病前三周出现。