A number of other multifocal vascular skin disorders presenting at birth, in infancy, or in childhood have been described (Table 2).1- 8 The skin lesions in our 3 patients, however, are unique in their clinical and histological appearance, and far more numerous than would be typical in any of these conditions except for diffuse infantile hemangiomatosis. The morphologic characteristics of cutaneous lesions in multifocal lymphangioendotheliomatosis with thrombocytopenia are quite distinctive, characterized by flat or indurated papules and plaques with a red-brown to burgundy color. Many have central pallor, and in some cases central scarlike areas. Slowly progressive onset of new lesions was observed in all cases, without clinical evidence of regression. Multifocal infantile hemangiomas, by contrast, are typically bright red and dome-shaped or nodular in clinical appearance. They typically present in the first few weeks of life and disappear or involute signficantly by 5 years of age. Blue rubber bleb nevus syndrome is characterized by multiple venous malformations involving the skin and GI tract, blue in color, and usually very compressible, with clinical appearances including classic nipplelike lesions, small punctate papules, and larger disfiguring lesions. These lesions may slowly increase in number over time (as in our patients), but remain far fewer in total number. Multifocal venous malformations with glomus cells (eg, glomuvenous malformations and glomangiomas) are clinically similar to but distinguishable from venous malformations, typically involving a large cutaneous area as multiple, soft, red-to-blue nodules that may be widely distributed or confluent, or as pink to deep blue, cobblestonelike plaques that typically thicken with time.25 Maffucci syndrome is usually not congenitally evident, but develops during childhood as multiple bulging, firm, blue-to-purple vascular lesions that are most prominent on the distal extremities. These lie deeper in the dermis and subcutis than the lesions in our patients; patients with Maffucci syndrome differ further in showing lytic bone lesions, whereas GI tract involvement is absent. Hereditary hemorrhagic telangiectasia only rarely presents in early infancy; mucocutaneous lesions typically present first on the tongue and oral mucosa, and later involve the face and extremities during adulthood, most often as small red-to-purple papules with radiating peripheral telangiectasias.