Ann Dermatol.  2010 Nov;22(4):426-430. 10.5021/ad.2010.22.4.426.

Kasabach-Merritt Syndrome Arising from Tufted Angioma Successfully Treated with Systemic Corticosteroid

Affiliations
  • 1Department of Dermatology & Cutaneous Biology Research Institute, Yonsei University College of Medicine, Seoul, Korea. kychung@yuhs.ac

Abstract

We report a case of Kasabach-Merritt syndrome arising from a tufted angioma successfully treated with systemic corticosteroid. A 2-month-old male infant presented with a palm-sized, erythematous induration on his left pubis. The lesion was diagnosed as tufted angioma histopathologically. After 1 month, the lesion suddenly expanded to the abdomen and scrotum. Initial laboratory tests were consistent with consumptive coagulopathy. He was diagnosed with Kasabach-Merritt syndrome and treated with intravenous dexamethasone at 0.32 mg/kg/day (equivalent to prednisolone 2.0 mg/kg/day). Two days after initiating the treatment, his platelet counts recovered and the lesion ceased to expand. Steroid therapy was converted to oral prednisolone and the dosage was subsequently tapered, and the lesion gradually involuted with no signs of recurrence for a year.

Keyword

Kasabach-Merritt syndrome; Systemic corticosteroid; Tufted angioma

MeSH Terms

Abdomen
Dexamethasone
Hemangioma
Humans
Infant
Kasabach-Merritt Syndrome
Male
Platelet Count
Prednisolone
Recurrence
Scrotum
Skin Neoplasms
Dexamethasone
Hemangioma
Prednisolone
Skin Neoplasms

Figure

  • Fig. 1 (A) A solitary 2.5×2.5 cm-sized bluish, growing indurated subcutaneous nodule on the left pubis. (B) A large, well-defined purpuric induration expanded to the abdomen, scrotum and left thigh.

  • Fig. 2 (A) Cannon ball distribution of small discrete vascular tufts are found throughout the dermis (H&E, ×40). (B) A lobule composed of bloodless aggregates of round dilated capillaries surrounded by crescent-shaped vascular structures (H&E, ×200).

  • Fig. 3 Slightly bluish and erythematous induration of involuted lesion 3 months after treatment.


Cited by  1 articles

Intractable Tufted Angioma Associated with Kasabach-Merritt Syndrome
Jae Woo Choi, Jung Im Na, Jong Soo Hong, Soon Hyo Kwon, Sang Young Byun, Kwang Hyun Cho, Sang Woong Youn, Hyung Soo Choi, Kyoung-Duk Park, Kyoung Chan Park
Ann Dermatol. 2013;25(1):129-130.    doi: 10.5021/ad.2013.25.1.129.


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