Multifocal lymphangioendotheliomatosis with thrombocytopenia (MLT) is a rare disease characterized by congenital and progressive vascular lesions of the skin and gastrointestinal tract that may be associated with thrombocytopenia and possibly life-threatening gastrointestinal bleeding. Reports published on the disease and treatment strategies are scarce. We present two cases of MLT treated with sirolimus.

A 13-year-old female with Crouzon syndrome and hydrosyringomyelia was referred for assessment of a tumor located on her back adjacent to the right scapula. The lesion had appeared 2 months previously.The mother reported that the tumor developed on top of a congenital macule. The lesion was reported to have exophytic growth, was asymptomatic, and did not bleed.Physical examination revealed a sessile, firm, and dark blue-purple tumor with well-defined borders, a lobulated surface, and a depressed center, measuring 1 Â 0.5 cm (Figure 1); there was no macule visible under the tumor. Dermoscopy showed blue-gray ovoid nests with bright whitish areas (chrysalis) and a central depression with small crusts (Figure 2). An excisional biopsy was performed (Figures 3 and 4).

Orbital vascular anomalies (OVAs) are a heterogeneous group of disorders frequently found in the orbital cone, the periorbital region, or within the orbit itself. OVAs are divided into tumors and malformations. The most frequent clinical presentation is exophthalmos, associated or not with an alteration of the visual axis. They may also cause acute complications, being intralesional bleeding or cellulitis the most frequent, and chronic complications, such as amblyopia and long-term visual acuity impairment.The development of imaging techniques, the use of new drugs, and the implementation of innovative procedures in interventional radiology have resulted in a significant improvement in the diagnostic and therapeutic approaches to these patients, essential to an accurate diagnosis and management.

Basaloid follicular hamartoma is a benign, superficial malformation of hair follicles that can be mistaken both clinical and histopathologically for basal cell carcinoma. Basaloid follicular hamartoma has been linked to a mutation in the PTCH-1 gene, which is part of the same pathway involved in Gorlin-Goltz syndrome. Here we present a 9-year-old patient with an asymptomatic congenital lesion on the forehead, which increased in size over the years. Histopathology showed a basaloid follicular hamartoma associated with follicular mucinosis and inflammation. Gorlin-Goltz syndrome was ruled out by clinical examination.