Abstract：
Crystal-storing histiocytosis (CSH) is a rare condition in which crystals accumulate in the cytoplasm of histiocytes and is usually associated with a lymphoplasmacytic neoplasm. Cutaneous CSH is extraordinarily rare and limited to case reports in the literature. We report two cases of this disease with cutaneous involvement. Case 1 was a 65-year-old male with a 4-month history of a pruritic eruption that started as a solitary pink to skin-colored indurated plaque on the anterior neck before progressing to involve the whole neck, chest wall, and face. Case 2 was a 54-year-old woman with a history of unspecified \"lymphoma\" who presented with a soft nodule on the forearm. Biopsies from both cases had similar findings and showed a proliferation of epithelioid cells with pink cytoplasm and intracellular crystalline structures infiltrating the dermis and subcutaneous fat. In the first case, the cells were positive for CD43, CD45, CD68, and IgG kappa, and in the second case, the crystals were positive for IgG lambda. Based on these findings, the patients were diagnosed with cutaneous CSH. We highlight this rare diagnosis and the importance of investigating an underlying lymphoplasmacytic neoplasm.
摘要：
储存晶体的组织细胞增多症（CSH）是一种罕见的疾病，其中晶体在组织细胞的细胞质中积累，通常与淋巴浆细胞瘤有关。皮肤CSH非常罕见，文献中仅限于病例报告。我们报告了2例皮肤受累的这种疾病。病例1是一名65岁的男性，有4个月的瘙痒性皮疹病史，最初是前颈上的单发粉红色至肤色的饱和斑块，然后累及整个颈部。胸壁,和脸。病例2是一名54岁的女性，有未指明的“淋巴瘤”病史，前臂上有一个软结节。两种病例的活检结果相似，并显示上皮样细胞增殖，粉红色细胞质和细胞内结晶结构浸润真皮和皮下脂肪。在第一种情况下,细胞CD43、CD45、CD68和IgGκ呈阳性，在第二种情况下，晶体对IgGλ呈阳性。基于这些发现,患者被诊断为皮肤CSH。我们强调了这种罕见的诊断以及研究潜在的淋巴浆细胞瘤的重要性。
