Myelodysplastic Syndrome Presenting as Generalized Granulomatous Dermatitis
Myelodysplastic Syndrome Presenting as Generalized Granulomatous Dermatitis
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DOI：
10.1001/archdermatol.2011.39
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发表时间：
2011-03-01
影响因子：
--
通讯作者：
Pittelkow, Mark R.
中科院分区：
文献类型：
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作者：
Balin, Samuel J.;Wetter, David A.;Pittelkow, Mark R.
背景：肉芽肿性皮炎很少被报道为白血病或骨髓增生异常综合征(MDS)的表现。我们描述了一例广泛的肉芽肿性皮炎，比MDS诊断早了两年。观察：一名71岁的男性患者出现了一种全身轻度瘙痒的皮疹，在两年内缓慢进展。穿孔活检标本显示真皮间质肉芽肿性炎症。有差别的完整血细胞计数显示明显的单核细胞增多症，随后的骨髓活检结果证实了MDS。来那度胺开始治疗后，患者的皮肤状况在治疗6周后有所改善；然而，他的MDS进展为急性髓系白血病，并在随后不久死亡。结论：很少有文献记录肉芽肿性皮炎的发生，作为潜在的血液系统疾病的表现。这一病例说明了广泛存在的肉芽肿性皮炎预示着MDS的发病。皮肤病界必须认识到肉芽肿性皮炎与骨髓发育不良的罕见关联，因为皮肤表现可能是目前的主要发现，并且可以比白血病的发展早几年。
Background: Granulomatous dermatitis has rarely been reported as a manifestation of leukemia or myelodysplastic syndrome (MDS). We describe a case of widespread granulomatous dermatitis that preceded the diagnosis of MDS by 2 years.Observations: A 71-year-old man developed a generalized, mildly pruritic eruption that slowly progressed over a 2-year period. Punch biopsy specimens demonstrated interstitial dermal granulomatous inflammation. A complete blood cell count with differential showed marked monocytosis, and the findings of a subsequent biopsy of the bone marrow confirmed MDS. Lenalidomide therapy was initiated, and the patient's skin condition improved after 6 weeks of treatment; however, his MDS progressed to acute myeloid leukemia, and he died shortly thereafter.Conclusions: There is a paucity of literature documenting the occurrence of granulomatous dermatitis as a manifestation of an underlying hematologic disorder. This case illustrates a striking example of widespread granulomatous dermatitis heralding the onset of MDS. It is imperative that the dermatologic community recognize the rare association of granulomatous dermatitis with myelodysplasia, because the cutaneous manifestations may be the presenting finding and can precede the development of leukemia by several years.