Doffoël-Hantz V, Fauchais A-L, Sparsa A, Roux C, Ly K, Loustaud-Ratti V, Bonnetblanc J-M, Vidal E, Bédane C
Service de dermatologie, CHU Dupuytren, 2, avenue Martin-Luther-King, 87042 cedex Limoges, France.
Rev Med Interne. 2007 Feb;28(2):127-30. doi: 10.1016/j.revmed.2006.10.330. Epub 2006 Nov 10.
Abrupt onset of papular lesions, with ulcero necrotic evolution could refers to many dermatitis. Febrile ulceronecrotic Mucha-Habermann disease is an unusual severe form of pityriasis lichenoides and varioliform acuta (PLEVA) characterized with ulceronecrotic eruption potentially associated with high fever and systemic symptoms leading to death. Lymphomatoid papulosis is a recurrent papulonodular eruption with an initial presentation close to PLEVA. While this disorder usually has a benign course, about 10% of the patients develop lymphoproliferative disorders such as CD30+ lymphoma.
The authors reported an acute form of PLEVA in a young man hospitalized in internal medicine unit with fever and ulcero-necrotic papulo-vesicular lesions. Treatment with tetracycline was successful. They also report a case of lymphomatoïd pustulosis that occurred in a 34 years old woman with an extensive nodulo-pustular eruption characterized by central necrosis and ulceration. Histopathological examination revealed CD30 lymphocytic infiltration leading to diagnosis.
Theses two dermatosis, uncommon and clinically similar, are both characterized with an unpredictable evolution that to be known by internist.
丘疹性皮损突然出现,并伴有溃疡坏死性演变,可能提示多种皮炎。发热性溃疡坏死性穆查-哈伯曼病是急性痘疮样苔藓样糠疹(PLEVA)的一种罕见严重形式,其特征为溃疡坏死性皮疹,可能伴有高热和全身症状,甚至导致死亡。淋巴瘤样丘疹病是一种复发性丘疹结节性皮疹,最初表现与PLEVA相似。虽然这种疾病通常病程良性,但约10%的患者会发展为淋巴增殖性疾病,如CD30+淋巴瘤。
作者报告了一名在内科住院的年轻男性,患有急性PLEVA,伴有发热和溃疡坏死性丘疹水疱性皮损。四环素治疗成功。他们还报告了一例发生在一名34岁女性身上的淋巴瘤样脓疱病,表现为广泛的结节脓疱性皮疹,中央有坏死和溃疡。组织病理学检查显示CD30淋巴细胞浸润,从而确诊。
这两种皮肤病并不常见,临床症状相似,其演变均不可预测,内科医生应有所了解。