关键词: morphea syndromic albinism vitiligo

来  源:   DOI:10.3390/hematolrep16020036   PDF(Pubmed)

Abstract:
Hypopigmentation disorders pose significant diagnostic challenges in dermatology, sometimes reflecting underlying hematological conditions. This review explores the clinical presentations related to hypopigmentation in hematological disorders, focusing on vitiligo, morphea, and syndromic albinism. Vitiligo, an autoimmune disorder targeting melanocytes, involves interactions between genetic polymorphisms and immune responses, particularly regarding CD8+ T cells and IFN-γ. Drug-induced vitiligo, notably by immune checkpoint inhibitors and small-molecule targeted anticancer therapies, underscores the importance of immune dysregulation. Morphea, an inflammatory skin disorder, may signal hematological involvement, as seen in deep morphea and post-radiotherapy lesions. Syndromic albinism, linked to various genetic mutations affecting melanin production, often presents with hematologic abnormalities. Treatment approaches focus on targeting the immune pathways specific to the condition, and when that is not possible, managing symptoms. Understanding these dermatological manifestations is crucial for the timely diagnosis and management of hematological disorders.
摘要:
色素沉着障碍在皮肤病学中提出了重大的诊断挑战,有时反映潜在的血液学状况。这篇综述探讨了血液病中色素沉着不足的临床表现,专注于白癜风,疣,和综合征白化病。白癜风,针对黑素细胞的自身免疫性疾病,涉及遗传多态性和免疫反应之间的相互作用,特别是关于CD8+T细胞和IFN-γ。药源性白癜风,特别是通过免疫检查点抑制剂和小分子靶向抗癌疗法,强调了免疫失调的重要性。Morphea,炎症性皮肤病,可能是血液学受累的信号,如在深层和放疗后的病变中所见。综合征白化病,与影响黑色素产生的各种基因突变有关,常表现为血液学异常。治疗方法侧重于针对特定疾病的免疫途径,当这是不可能的时候,管理症状。了解这些皮肤病表现对于及时诊断和治疗血液病至关重要。
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