Marcoval J, Moreno A, Bordas X, Gallardo F, Peyrí J
Department of Dermatology, Hospital Prínceps D'Espanya, Barcelona, Spain.
J Am Acad Dermatol. 1998 Sep;39(3):439-42. doi: 10.1016/s0190-9622(98)70321-4.
Diffuse plane xanthoma (DPX) usually has been described in association with a reticuloendothelial disease. However, the incidence of an underlying disease and its relation to clinical and histopathologic features have not been previously described.
We describe the clinicopathologic features of a series of patients with DPX.
Eight patients with DPX were diagnosed and studied between 1987 and 1996.
Three of the 8 patients had a reticuloendothelial disease (benign monoclonal gammopathy in 2 and chronic myelomonocytic leukemia in 1). These 3 patients had larger cutaneous lesions and involvement of trunk and extremities. Histologic examination showed only foamy macrophages in 2 of the 8 patients. The remainder exhibited macrophages, foamy histiocytes, lymphocytes, and Touton cells.
The incidence of underlying disease associated with DPX seems to be lower than expected. However, patients with DPX should be observed for the potential occurrence of an associated condition.
弥漫性扁平黄瘤(DPX)通常被描述为与一种网状内皮系统疾病相关。然而,潜在疾病的发生率及其与临床和组织病理学特征的关系此前尚未见报道。
我们描述了一系列DPX患者的临床病理特征。
1987年至1996年间诊断并研究了8例DPX患者。
8例患者中有3例患有网状内皮系统疾病(2例为良性单克隆丙种球蛋白病,1例为慢性粒单核细胞白血病)。这3例患者的皮肤损害较大,累及躯干和四肢。组织学检查显示,8例患者中有2例仅见泡沫状巨噬细胞。其余患者表现为巨噬细胞、泡沫状组织细胞、淋巴细胞和图顿巨细胞。
与DPX相关的潜在疾病发生率似乎低于预期。然而,应对DPX患者观察是否可能出现相关疾病。