Department of Internal Medicine , An-Najah National University Hospital , 7707, Asira Street, Nablus, Palestine.
Department of Dermatology , An-Najah National University Hospital , Nablus, Palestine.
BMC Dermatol. 2020 Dec 4;20(1):18. doi: 10.1186/s12895-020-00118-7.
Scleromyxedema is a rare, para-neoplastic, chronic, progressive condition of the Lichen myxedematosus (LM) family. The clinical picture consists of generalized confluent papular eruptions with possible systemic manifestations, which may be fatal as it still constitutes a therapeutic dilemma. Histologically, it is characterized by dermal mucin deposition, fibroblast proliferation with fibrosis, with monoclonal gammopathy in the absence of thyroid disease. Some atypical forms of the disease were reported in the literature, but none were reported in acute leukemia.
Herein, we report a case of a 21 years old female patient, known case of acute lymphoblastic leukemia (ALL), who developed numerous hyper-pigmented erythematous papules and plaques, mainly over her thighs, lower abdomen, and sub-mammary flexures. Histopathology of skin lesions confirmed the diagnosis of atypical scleromyxedema. Her symptoms significantly improved with the use of high dose intravenous immunoglobulin (IVIG).
Despite that scleromyxedema is associated with many hematologic disorders, it is very rarely associated with acute lymphoblastic leukemia, and a high index of suspicion is needed for diagnosis. IVIG remains a reasonable management of such a disabling disease.
硬肿性黏液水肿是一种罕见的、副瘤性的、慢性的、进行性的黏蛋白样黏液水肿（LM）家族疾病。临床表现为全身性融合性丘疹性皮疹，可能伴有全身表现，由于其仍然是一个治疗难题，因此可能致命。组织学上，其特征是皮肤粘蛋白沉积、成纤维细胞增殖伴纤维化，伴单克隆丙种球蛋白血症而无甲状腺疾病。文献中报道了一些该病的非典型形式，但在急性白血病中尚未报道。
在此，我们报告一例 21 岁女性患者，已知患有急性淋巴细胞白血病（ALL），她出现了许多色素沉着性红斑性丘疹和斑块，主要分布在大腿、下腹部和乳房下皱褶处。皮肤病变的组织病理学检查证实了非典型硬肿性黏液水肿的诊断。她的症状在使用大剂量静脉注射免疫球蛋白（IVIG）后显著改善。
尽管硬肿性黏液水肿与许多血液疾病有关，但与急性淋巴细胞白血病的相关性非常罕见，因此需要高度怀疑以做出诊断。IVIG 仍然是治疗这种致残性疾病的合理方法。
