Donati Pietro, Muscardin Luca, Amantea Ada, Paolini Francesca, Venuti Aldo
Laboratory of Cutaneous Histopathology, Institute of Dermatology S. Maria and San Gallicano, Via Chianesi, 53, 00144 Rome, Italy.
Eur J Dermatol. 2009 May-Jun;19(3):243-7. doi: 10.1684/ejd.2009.0629. Epub 2009 Feb 12.
Incontinentia pigmenti (IP) is an X-linked dominant disorder, which occurs in female patients. We present a typical case of IP with subungual tumors (STIP) together with a short review on subungual tumors in IP. The diagnosis was achieved on the basis of the onset in adult life of STIP together with the other specific symptoms like ocular and dental abnormalities and achromic lesions of the legs. In the STIP lesions the presence and, in one of them, the expression, of HPV type 15 were detected. Topical therapy with retinoic acid cured the tumoral lesions. To the best of our knowledge this is the first report of HPV in STIP, opening a new scenario in the pathogenesis and the treatment of STIP. In conclusion, in our opinion, all painful subungual tumors should be considered as a possible late manifestation of IP.
色素失禁症(IP)是一种X连锁显性疾病,发生于女性患者。我们报告一例典型的伴有甲下肿瘤的色素失禁症(STIP)病例,并对色素失禁症中的甲下肿瘤进行简要综述。诊断基于成年期出现的甲下肿瘤以及眼部和牙齿异常、腿部色素脱失性病变等其他特定症状。在甲下肿瘤病变中检测到15型人乳头瘤病毒(HPV)的存在,其中一例还检测到其表达。外用维甲酸治愈了肿瘤性病变。据我们所知,这是关于甲下肿瘤中HPV的首次报道,为甲下肿瘤的发病机制和治疗开辟了新的局面。总之,我们认为,所有疼痛性甲下肿瘤都应被视为色素失禁症可能的晚期表现。