Alcántar AMM, Rojas CJ, Rodríguez RA, Hierro OS, Díaz MV

Language: Spanish
References: 5
Page: 311-313
PDF size: 382.15 Kb.

ABSTRACT

Kasabach-Merritt phenomenon is a rare disorder that occurs in less than 1% of the population. Characterized by vascular tumors, among which the most frequently found are kaposiform hemangioendothelioma and tufted angiomas, associated with severe thrombocytopenia, microangiopathic hemolytic anemia, and consumption coagulopathy. Prompt diagnosis and treatment improve patient survival. The first-line treatment option should be systemic corticosteroids and vincristine.
We report a patient with less than three months of age with a localized dermatosis on the right pelvic limb that affected the anterior and posterior area of the thigh, the leg, and the external genitals. The lesion had evolved during the last 15 days and was characterized by a plaque-like tumor of 12 cm wide in its long axis, irregular shape, red-violet color, smooth surface, welldefined borders, indurated, with increase local temperature, without palpable vibration, with a subcutaneous component.

REFERENCES

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3. Vivas-Colmenares GV, Ramírez-Villar GL, Bernabeu-Wittel J, Matutede Cárdenas JA y Fernández-Pineda I, The importance of early diagnosisand treatment of kaposiform hemangioendothelioma complicatedby Kasabach-Merritt phenomenon, Dermatol Pract Concept 2015;5(1):91-3.

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5. Gruman A, Liang MG, Mulliken JB, Fishman SJ, Burrows PE, KozakewichHP, Blei F y Frieden IJ, Kaposiform hemangioendothelioma without Kasabach-Merritt phenomenon, J Am Acad Dermatol 2005; 52(4):616-22.