Department of Dermatology, Mayo Clinic, Jacksonville, Florida, USA.
Department of Dermatology, Mayo Clinic, Rochester, Minnesota, USA.
J Cutan Pathol. 2024 Nov;51(11):834-839. doi: 10.1111/cup.14678. Epub 2024 Jul 12.
Histiocytoid Sweet syndrome (H-SS) is a histopathological variant of Sweet syndrome (SS) defined by cutaneous infiltration of immature myeloid cells morphologically resembling histiocytes. The association of H-SS with underlying malignancy, particularly myelodysplastic syndromes, is well-established. Myelodysplasia cutis (MDS-cutis) has been proposed to describe cases historically diagnosed as H-SS but characterized by shared clonality of the myeloid infiltrate in skin and bone marrow. Therefore, identifying patients who might have MDS-cutis is critical for the management of the associated hematologic malignancy. VEXAS syndrome, an adult-onset autoinflammatory disease, should also be included in the histopathologic differential diagnosis of H-SS, as it shares clinical and pathologic features with MDS-cutis. Through the presentation of two cases, we aim to highlight the defining features and key clinical implications of MDS-cutis and VEXAS syndrome.
组织细胞样嗜中性粒细胞皮肤病（H-SS）是Sweet 综合征（SS）的一种组织病理学变异型，其特征为皮肤浸润不成熟的髓系细胞，形态上类似于组织细胞。H-SS 与潜在恶性肿瘤，特别是骨髓增生异常综合征（MDS）的相关性已得到充分证实。髓系皮肤病（MDS-cutis）曾被用来描述既往诊断为 H-SS 的病例，但这些病例的特征是皮肤和骨髓中的髓系浸润具有共同的克隆性。因此，识别可能患有 MDS-cutis 的患者对于相关血液恶性肿瘤的治疗至关重要。VEXAS 综合征，一种成人发病的自身炎症性疾病，也应包含在 H-SS 的组织病理学鉴别诊断中，因为它与 MDS-cutis 具有相似的临床和病理特征。通过介绍两个病例，我们旨在强调 MDS-cutis 和 VEXAS 综合征的特征性表现和关键临床意义。
