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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. [ABSTRACT FROM AUTHOR]

Lymphangioma circumscriptum is an uncommon hamartomatous malformation that is not only localized to an area of skin, but also spreads to subcutaneous tissue and muscle (Whimster's hypothesis). We describe a 7-year-old boy with a bluish plaque on the left side of the back and abdomen with clusters of translucent vesicles (frog spawn). Magnetic resonance imaging showed the lymphangioma had spread to muscles and even fat adjacent to the left kidney. We decided against radical surgery as it would have been too aggressive, and limited excision can produce new surface lymphangiomas. [ABSTRACT FROM AUTHOR]