Morrell Dean S, Pepping Marie A, Scott J Paul, Esterly Nancy B, Drolet Beth A
University of North Carolina, Department of Dermatology, 3100 Thurston-Bowles CB#7287, Chapel Hill, NC 27514, USA.
Arch Dermatol. 2002 Sep;138(9):1208-12. doi: 10.1001/archderm.138.9.1208.
Hemophagocytic lymphohistiocytosis is a rare, rapidly progressive, and potentially fatal disorder of activated histiocytes. The initial clinical presentation commonly includes fever, hepatosplenomegaly, and pancytopenia. Skin eruptions are described in up to 65% of patients. Information regarding the morphological features, configuration, and distribution of these eruptions is lacking and is typically reported as nonspecific and "maculopapular." The aim of this report is to better delineate the cutaneous manifestations of the disorder to assist in differentiating the process from other systemic diseases.
A case report of a neonate with hemophagocytic lymphohistiocytosis with generalized purpuric macules is described. The clinical features of 5 other patients with hemophagocytic lymphohistiocytosis at Children's Hospital of Wisconsin, Milwaukee, are summarized. Clinical images of 1 additional neonatal patient with hemophagocytic lymphohistiocytosis are presented as well. These observations demonstrate the varied cutaneous manifestations of hemophagocytic lymphohistiocytosis: erythroderma, generalized purpuric macules and papules, and morbilliform eruptions.
Awareness of cutaneous involvement can assist in the initial diagnosis of hemophagocytic lymphohistiocytosis and potentially signify recurrences.
噬血细胞性淋巴组织细胞增生症是一种罕见的、进展迅速且可能致命的活化组织细胞疾病。其初始临床表现通常包括发热、肝脾肿大和全血细胞减少。高达65%的患者有皮肤疹。关于这些皮疹的形态特征、形态及分布的信息匮乏，通常被报道为非特异性的“斑丘疹”。本报告旨在更好地描述该疾病的皮肤表现，以协助将其与其他全身性疾病相鉴别。
描述了1例患有噬血细胞性淋巴组织细胞增生症并伴有全身性紫癜性斑疹的新生儿病例报告。总结了威斯康星州密尔沃基市儿童医院另外5例噬血细胞性淋巴组织细胞增生症患者的临床特征。还展示了另外1例患有噬血细胞性淋巴组织细胞增生症的新生儿患者的临床图片。这些观察结果显示了噬血细胞性淋巴组织细胞增生症多样的皮肤表现：红皮病、全身性紫癜性斑疹和丘疹以及麻疹样皮疹。
认识到皮肤受累有助于噬血细胞性淋巴组织细胞增生症的初步诊断，并可能提示复发。
