关键词: Stevens–Johnson syndrome allopurinol hypersensitivity pancytopenia severe cutaneous adverse reaction

来  源:   DOI:10.1097/MS9.0000000000002229   PDF(Pubmed)

Abstract:
UNASSIGNED: Stevens-Johnson syndrome (SJS) is a rare and unusual hypersensitivity reaction to certain drugs like allopurinol, commonly used for treating gout. SJS is recognized by extensive necrosis and detachment of skin and mucus membranes. Pancytopenia, characterized by decreased levels of red blood cells, white blood cells and platelets, is an exceedingly rare occurrence in the rare disorder SJS.
UNASSIGNED: The authors present a 61-year-old male who exhibited symptoms of fever and rash for 5 days accompanied by pancytopenia and liver injury.
UNASSIGNED: The abdomen and bilateral lower extremities exhibited several well-defined dusky-colored hyperpigmented macular lesions. Initially, these lesions were small, tender, erythematous, and raised, later transitioning to a dark red. Multiple distinct ulcerations were present on the lips and buccal cavity. Additionally, there was denudation of the skin with bleeding observed between the toes of both legs. The causality was assessed as a definite adverse drug reaction according to the Naranjo and ALDEN algorithm. The patient received treatment consisting of intravenous steroid along with prophylactics antibiotics. The individual\'s pancytopenia was resolved without requiring any blood cells or plasma or platelet concentrate transfusion.
UNASSIGNED: The exact pathophysiology of SJS associated with pancytopenia has not yet been fully elucidated. The authors\' study hypothesized that the cause of pancytopenia in SJS could be either the direct cytotoxicity of drugs or immune-mediated damage to the bone marrow cells. Additional studies are necessary to establish the precise pathophysiology of the condition. Moreover, our study also indicates that pancytopenia can resolve in SJS without the need for blood cells or plasma or platelet concentrate transfusion. Once more, further studies are required to establish precise management strategies for managing SJS associated with pancytopenia.
摘要:
Stevens-Johnson综合征(SJS)是对某些药物如别嘌呤醇的一种罕见且不寻常的超敏反应,常用于治疗痛风。SJS通过皮肤和粘膜的广泛坏死和脱离来识别。全血细胞减少症,以红细胞水平下降为特征,白细胞和血小板,在罕见的疾病SJS中非常罕见。
作者介绍了一名61岁的男性,他表现出发烧和皮疹症状5天,并伴有全血细胞减少和肝损伤。
腹部和双侧下肢表现出一些清晰的暗色色素沉着黄斑病变。最初,这些病变很小,tender,红斑,和提高,后来过渡到深红色。嘴唇和颊腔上存在多个明显的溃疡。此外,两腿脚趾之间有皮肤剥脱和出血。根据Naranjo和ALDEN算法将因果关系评估为确定的药物不良反应。患者接受了由静脉类固醇和预防性抗生素组成的治疗。该个体的全血细胞减少症得以解决,无需输注任何血细胞或血浆或血小板浓缩物。
SJS与全血细胞减少症相关的确切病理生理学尚未完全阐明。作者的研究假设SJS中全血细胞减少症的原因可能是药物的直接细胞毒性或免疫介导的骨髓细胞损伤。需要进行其他研究以建立该病症的精确病理生理学。此外,我们的研究还表明,全血细胞减少症可以在SJS中缓解,而无需输注血细胞或血浆或血小板浓缩物.再一次,需要进一步的研究来建立管理与全血细胞减少相关的SJS的精确管理策略.
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