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A Newly Recognized Clinicopathological Entity

Paula E. North, MD, PhD; Teri Kahn, MD; Maria R. Cordisco, MD; Soheil S. Dadras, MD, PhD; Michael Detmar, MD; Ilona J. Frieden, MD

Arch Dermatol. 2004;140:599-606.

Background  Severe thrombocytopenic coagulopathy may complicate platelet-trapping vascular tumors such as kaposiform hemangioendothelioma and tufted angioma. Low-grade, chronic consumptive coagulopathy may occur with extensive venous and lymphatic malformations. We have also observed patients with rare multifocal, congenital skin and gastrointestinal (GI) tract vascular anomalies of distinctive and remarkably similar appearance, all associated with coagulopathy. We studied the clinical and histopathologic features of 3 patients demonstrating this previously uninvestigated phenomenon.

Observations  All 3 patients presented with hundreds of congenital red-brown skin plaques as large as a few centimeters, with similar lesions throughout the GI tract and severe GI tract bleeding. One patient had synovial involvement. All had significant thrombocytopenia, with prothrombin and partial thromboplastin times and fibrinogen levels near the reference range. Corticosteroids and/or interferon alfa treatment resulted in equivocal or no improvement. Skin lesions from all 3 patients were histologically distinctive and similar, including dilated, thin-walled vessels in the dermis and subcutis lined by hobnailed, proliferative endothelial cells (10%-15% immunoreactive for Ki-67), most displaying intraluminal papillary projections. Immunoreaction for the lymphatic marker LYVE-1 was uniformly present.

Conclusions  We propose the term multifocal lymphangioendotheliomatosis with thrombocytopenia to distinguish this newly recognized clinicopathological entity. These congenital lesions, like tufted angioma and kaposiform hemangioendothelioma, show lymphatic differentiation, strengthening the association between abnormal lymphatic endothelium and coagulopathy.

From the Departments of Pathology and Otolaryngology, the University of Arkansas for Medical Sciences and Arkansas Children's Hospital, Little Rock (Dr North); the Department of Dermatology, The Cleveland Clinic, Cleveland, Ohio (Dr Kahn); the Hospital Nacional de Pediatria, Buenos Aires, Argentina (Dr Cordisco); the Cutaneous Biology Research Center, Massachusetts General Hospital and Harvard Medical School, Boston (Drs Dadras and Detmar); and the Departments of Pediatrics and Dermatology, University of California–San Francisco Medical Center (Dr Frieden). The authors have no relevant financial interest in this article.

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