Petit A, Lemarchand-Venencie F, Pinquier L, Lebbe C, Bourrat E
Service de dermatologie, hôpital Saint-Louis, 1, avenue Claude-Vellefaux, 75010 Paris, France.
Ann Dermatol Venereol. 2012 Mar;139(3):183-8. doi: 10.1016/j.annder.2011.10.411. Epub 2011 Dec 16.
We report three cases of a peculiar rash with mixed clinical features of both epidermal nevus and acanthosis nigricans. Their characteristics have been compared to those of very rare but similar cases found in the medical literature.
Two young adults (one male, one female) and a 7-year-old boy consulted for hyperchromic asymptomatic plaques located respectively on the right scapula, the left upper arm and the right frontotemporal area of the face. None had any noticeable familial medical history. None were overweight or had a personal history of diabetes or endocrine dysfunction. The plaques had appeared soon after puberty in the adults and at the age of 4 years in the child and they had remained stable for years. They were polycyclic in shape, sharply demarcated, and with a linear distribution. Their surface was slightly papular, with a velvety appearance and texture. No other skin or mucous membrane lesions were observed elsewhere. The physical examination was otherwise normal. Skin biopsy specimens showed mild acanthosis with papillomatosis, hyperorthokeratosis with elongated rete ridges and slight thinning of the roof of the dermal papilla.
The striking clinical features of this rash rule out confusion with any other epidermal nevus (because of age of onset, shape and texture) or with acanthosis nigricans (because of its topography and the lack of associated neoplastic or endocrine disease). A dozen similar case reports can be found in the medical literature, mostly under the term "nevoid acanthosis nigricans". Such a rash may be linked to postzygotic mosaicism with the same mutations of the FGFR3 gene. However, since the physiopathology of this rash remains hypothetical, we propose to name it "RAVEN", for "rounded and velvety epidermal nevus", which is simply a descriptive acronym and nothing more.
我们报告了三例具有表皮痣和黑棘皮病混合临床特征的特殊皮疹病例。已将它们的特征与医学文献中发现的非常罕见但相似的病例进行了比较。
两名年轻人（一名男性，一名女性）和一名7岁男孩因分别位于右肩胛骨、左上臂和面部右额颞区的色素沉着无症状斑块前来就诊。他们均无明显的家族病史。均无超重情况，也无糖尿病或内分泌功能障碍的个人史。这些斑块在成年人青春期后不久出现，在儿童4岁时出现，并且多年来一直保持稳定。它们形状为多环状，边界清晰，呈线状分布。其表面略有丘疹，外观和质地呈天鹅绒状。在其他部位未观察到其他皮肤或黏膜病变。体格检查其他方面正常。皮肤活检标本显示轻度棘层肥厚伴乳头瘤样增生、过度正角化伴延长的 rete 嵴以及真皮乳头顶部轻微变薄。
这种皮疹显著的临床特征排除了与任何其他表皮痣（因其发病年龄、形状和质地）或黑棘皮病（因其部位以及缺乏相关肿瘤或内分泌疾病）混淆的可能性。在医学文献中可以找到十几例类似的病例报告，大多归类为“痣样黑棘皮病”。这种皮疹可能与 FGFR3 基因相同突变的合子后镶嵌现象有关。然而，由于这种皮疹的病理生理学仍然是假设性的，我们提议将其命名为“RAVEN”，即“圆形天鹅绒状表皮痣”，这只是一个描述性首字母缩写词，仅此而已。
