Martínez-Menchón Teresa, Mahiques Santos L, Vilata Corell J J, Febrer Bosch I, Fortea Baixauli J M
Service of Dermatology, Valencia University General Hospital, Valencia, Spain.
Pediatr Dermatol. 2005 Jan-Feb;22(1):44-7. doi: 10.1111/j.1525-1470.2005.22110.x.
Phacomatosis pigmentokeratotica is a rare syndrome defined by the association of an organoid nevus occasionally with sebaceous differentiation, a speckled lentiginous nevus, and other extracutaneous anomalies. The disorder is a consequence of the so-called twin spot genetic mechanism. We describe the first occurrence involving malignant degeneration of both nevus components, giving rise to three basal cell carcinomas over the sebaceous nevus and a malignant melanoma of the superficial spreading type over the speckled lentiginous nevus. This observation, in concert with the other instances reported in the literature, points to the need for adequate patient follow-up to ensure early detection and treatment of any possible associated malignant degeneration.
色素沉着性角化性错构瘤是一种罕见的综合征，其特征为偶尔伴有皮脂腺分化的器官样痣、斑状雀斑样痣以及其他皮肤外异常同时存在。该疾病是所谓的双斑遗传机制的结果。我们描述了首例两种痣成分均发生恶性变的病例，皮脂腺痣上出现了3例基底细胞癌，斑状雀斑样痣上出现了1例浅表扩散型恶性黑色素瘤。这一观察结果，结合文献中报道的其他病例，表明需要对患者进行充分随访，以确保早期发现并治疗任何可能相关的恶性变。
