Ann Dermatol. 2013 Feb;25(1):129-130. English.
Published online Feb 14, 2013.
Copyright © 2013 The Korean Dermatological Association and The Korean Society for Investigative Dermatology
letter

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,1 Sang Woong Youn, Hyung Soo Choi,2 Kyoung-Duk Park,3 and Kyoung Chan Park
    • Department of Dermatology, Seoul National University Bundang Hospital, Seongnam, Korea.
    • 1Department of Dermatology, Seoul National University Hospital, Seoul, Korea.
    • 2Department of Pediatrics, Seoul National University Bundang Hospital, Seongnam, Korea.
    • 3Department of Pediatrics, Seoul National University Hospital, Seoul, Korea.
Received May 11, 2012; Revised June 14, 2012; Accepted July 12, 2012.

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Dear Editor:

A 15-day-old boy visited our clinic presenting a palm sized erythematous patch located on his left cheek from birth, which was aggravated soon after his 1st vaccination against the hepatitis B virus. A skin biopsy revealed an oval shaped vascular lobules scattered in the dermis. It was a typical 'Cannonball' appearance (Fig. 1A). In high power field, the lobules were groups of ducts composed of proliferated endothelial cells. Tufted angioma (TA) was the most consistent diagnosis. The patient began receiving interferon a2b injection of 150 MU four times a week. However, the injection was ceased because 18 injections during a course of five weeks only brought intermittent fever with minimal effect. As an alternative option, propranolol (15 mg/d) and prednisolone (3 mg/kg/d) were given to the patient. Although a short-term tumor shrinkage was achieved, intermittent flare-ups of swelling, pain and tenderness persisted (Fig. 1B). Even vincristine (0.05 mg/kg/wk) failed to downsize the tumor.

Fig. 1
(A) Discrete and oval shaped scattered lobules composed of capillaries with obstructed lumen (H&E, ×100). (B) At the age of 2 months, the patch is swollen to a plaque, hindering his eye-opening.

The laboratory result at the four-month-follow up revealed a decreased platelet (107,000/ul), elevated D-dimer (4.66 ug/ml) and decreased fibrinogen (136 mg/dl), which implied the advent of Kasabach-Merritt syndrome (KMS). On this, he underwent nine sessions of combined chemotherapy of vincristine 0.05 mg/kg, actinomycin-D 1.5 mg/m2, cyclophosphamide 500 mg/m2 until now. For the one-year follow up since his first visit, neither the size of the tumor nor the KMS satisfactorily responded to the various treatment modalities. He still suffers from outbreaks of edema, tenderness and thrombocytopenia. For acute aggravation, methylprednisolone pulse therapy (30 mg/kg/day for 3 consequent days) and platelet transfusion were conducted for edema and thrombocytopenia, respectively (Fig. 2).

Fig. 2
Treatment history is summarized. Each bar stands for the duration of corresponding medication, the upward arrow for methylprednisolone pulse therapy, and the cross for platelet transfusion.

TA is a rare benign vascular tumor, which originates from the abnormal proliferation of endothelial cells1. Another vascular tumor, Kaposiform Hemangioendothelioma (KHE), shares many common properties with TA. Both have similar histopathology and can cause KMS. Although the immunohistochemistry for D2-40 differentiates KHE (positive for this marker) and TA (negative), it has currently been suggested that both are spectrums of the same vascular tumor. Unfortunately, there are no established treatments. Only several cases of using systemic agents or laser have been reported. It was suggested that physical trauma possibly aggravates the tumor by introducing various growth factors2. This hypothesis partly explains several cases of aggravated TA after skin punch biopsy at the authors' clinic (unpublished observation). We also experienced an aggravation of TA followed by a long pulsed Nd:YAG laser3. Interestingly in this case, the aggravation took place along with fever after vaccination. If the aggravation was correlated with mild inflammatory response caused by vaccination, we could hypothesize that even a mild inflammation triggers the proliferation of vascular tumor cells. Our experiences imply that TA is also associated with Koebner phenomenon (KP), which is similar to another vascular tumor, Kaposi's sarcoma, which is known to be developed as a manifestation of KP4.

KMS is a rare condition characterized by thrombocytopenia and consumptive coagulopathy, arising in patients with vascular tumor. Treatment experiences using corticosteroid and chemotherapeutic agents have been reported5.

References

    1. Park KC, Ahn PS, Lee YS, Kim KH, Cho KH. Treatment of angioblastoma with recombinant interferon-alpha 2. Pediatr Dermatol 1995;12:184–186.
    1. Weiss G, Shemer A, Trau H. The Koebner phenomenon: review of the literature. J Eur Acad Dermatol Venereol 2002;16:241–248.
    1. Na JI, Cho KH, Kim YG, Park KC. Angioblastoma showing aggravation after treatment with long-pulsed Nd:YAG laser (1064 nm). Pediatr Dermatol 2007;24:397–400.
    1. Stavrianeas NG, Katoulis AC, Kanelleas AI, Toumbis-Ioannou E, Bozi E, Anyfantakis V, et al. Kaposi's sarcoma as a Köbner phenomenon in a patient with bullous pemphigoid and sarcoidosis. Clin Exp Dermatol 2007;32:579–581.
    1. Kim T, Roh MR, Cho S, Chung KY. Kasabach-merritt syndrome arising from tufted angioma successfully treated with systemic corticosteroid. Ann Dermatol 2010;22:426–430.

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