Yoshimura T, Aiba S, Tadaki T, Tagami H
Tohoku University School of Medicine, Department of Dermatology, Sendai, Japan.
Acta Derm Venereol. 1994 May;74(3):221-3. doi: 10.2340/0001555574221223.
Generalized normolipemic plane xanthomatosis is a rare cutaneous disorder, frequently associated with reticuloendothelial diseases and some disorders with inflammation. Relapsing polychondritis is also a rare disease that shows an association with various immune-mediated diseases. We report a case of generalized normolipemic plane xanthomatosis associated with relapsing polychondritis in a 56-year-old Japanese man. We have already reported the clinical picture of relapsing polychondritis as well as an increase in urinary glycosaminoglycans excretion in this patient. During subsequent treatment with various immunosuppressive therapy, including prednisone, methotrexate, azathioprine, or aurothiomalate, multiple elevated yellowish erythematous plaques appeared on his neck approximately 32 months after the onset of relapsing polychondritis. Histologically, these eruptions consisted of perivascular neutrophilic infiltrate with nuclear dust and multiple foam cells among collagen bundles, compatible with those of generalized normolipemic plane xanthomatosis. This combination of two rare diseases has not been reported in the literature to our knowledge.
泛发性正常血脂性扁平黄瘤病是一种罕见的皮肤疾病，常与网状内皮系统疾病及一些炎症性疾病相关。复发性多软骨炎也是一种罕见疾病，与多种免疫介导性疾病有关。我们报告一例56岁日本男性，患有泛发性正常血脂性扁平黄瘤病并伴有复发性多软骨炎。我们已经报道过该患者复发性多软骨炎的临床表现以及尿糖胺聚糖排泄增加的情况。在随后使用包括泼尼松、甲氨蝶呤、硫唑嘌呤或金硫代苹果酸钠等多种免疫抑制疗法进行治疗期间，复发性多软骨炎发病约32个月后，患者颈部出现多个隆起的淡黄色红斑斑块。组织学检查显示，这些皮疹由血管周围嗜中性粒细胞浸润伴核尘以及胶原束间多个泡沫细胞组成，与泛发性正常血脂性扁平黄瘤病相符。据我们所知，这两种罕见疾病的这种组合在文献中尚未见报道。
