Federmann Birgit, Bonzheim Irina, Yazdi Amir S, Schmidt Janine, Fend Falko, Metzler Gisela
Institute of Pathology and Neuropathology, University Hospital and Comprehensive Cancer Center Tuebingen, University of Tuebingen, 72076 Tuebingen.
Department of Dermatology, University Hospital and Comprehensive Cancer Center Tuebingen, University of Tuebingen, 72076 Tuebingen.
Hum Pathol. 2017 Jun;64:198-206. doi: 10.1016/j.humpath.2017.04.010. Epub 2017 Apr 22.
Palisaded neutrophilic and granulomatous dermatitis (PNGD) is characterized by erythematous papules or plaques on trunk or limbs and is frequently associated with rheumatologic, autoimmune, or hematologic malignancies. Histopathology shows interstitial granulomas composed of epitheloid histiocytes in the reticular dermis with surrounding foci of collagen degeneration and variable neutrophilic inflammation. We report 3 cases of generalized PNGD associated with chronic myelomonocytic leukemia (CMML), a myelodysplastic/myeloproliferative neoplasm, which may show a variety of cutaneous manifestations. SRSF2 P95 hotspot mutations, found in 40%-50% of CMML cases, were retrospectively detected in skin and bone marrow biopsies of all 3 patients, in 1 of them already 5 years before CMML diagnosis. Generalized PNGD may represent a type of cutaneous manifestation of CMML. Because diagnosis of CMML is frequently difficult in cases with isolated persistent monocytosis and minimal dysplasia in the bone marrow, patients with a generalized PNGD should be evaluated for the presence of hematologic disorders including CMML, ideally supported by mutational analyses.
栅栏状中性粒细胞和肉芽肿性皮炎（PNGD）的特征是躯干或四肢出现红斑丘疹或斑块，常与风湿性、自身免疫性或血液系统恶性肿瘤相关。组织病理学显示，在网状真皮层有由上皮样组织细胞组成的间质肉芽肿，周围有胶原变性灶和不同程度的中性粒细胞炎症。我们报告3例与慢性粒单核细胞白血病（CMML）相关的泛发性PNGD，CMML是一种骨髓增生异常/骨髓增殖性肿瘤，可能表现出多种皮肤表现。在40%-50%的CMML病例中发现的SRSF2 P95热点突变，在所有3例患者的皮肤和骨髓活检中均被回顾性检测到，其中1例在CMML诊断前5年就已检测到。泛发性PNGD可能是CMML的一种皮肤表现类型。由于在骨髓中仅有持续性单核细胞增多和轻度发育异常的病例中，CMML的诊断通常很困难，因此对于泛发性PNGD患者，应评估是否存在包括CMML在内的血液系统疾病，理想情况下可通过突变分析来辅助诊断。
