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To the Editor:

We read with great interest the report by Javvaji and Frieden, “Response of Tufted Angiomas to Low-Dose Aspirin”[1]. The authors described two cases of tufted angiomas without Kasabach-Merritt phenomenon (KMP) that responded dramatically to treatment with 5 mg/kg/day of aspirin [1]. Both cases had improved appearance, decreased bulk, and resolution of symptoms [1]. Inspired by these cases, we decided to treat our patient in a similar fashion.

A 2-year-old boy with biopsy-proven tufted angioma presented to our pediatric dermatology clinic for treatment options. The lesion first appeared shortly after birth as a small red papule but began to enlarge. It became extremely tender to palpation. Examination revealed multiple red to violaceous papules and ill-defined tender plaques in a segmental distribution on the left posterior scalp, neck, upper back and shoulder, and upper chest with some overlying hypertrichosis (Fig. 1A). Laboratory results were significant for a hemoglobin of 11g/dL, d-dimer of 416 ng/mL, and normal platelets.

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Figure 1. Tufted angioma (A) before and (B) after 2 weeks of treatment with low-dose aspirin.

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A decision was made to initiate treatment because of the patient's symptoms. Because reported results to propranolol have been variable [2], other options were explored. After reading the impressive response to aspirin that Javvaji and Frieden reported [1] and with much-appreciated guidance through personal communication with Ilona Frieden, M.D., about this case, a decision was made to pursue treatment with aspirin. Our patient's mother has a known severe allergy to aspirin and was concerned about the risk of Reye's syndrome, so the family was hesitant to start treatment at high doses. Javvaji and Frieden described treatment with low-dose aspirin at 5 mg/kg/day [1]. Low-dose aspirin treatment in adults with 81 mg/day is anywhere from 0.9 to 1.6 mg/kg/day, assuming that an average adult patient weighs 50 to 90 kg. Taking these concerns and facts into consideration, we decided to treat our patient at a conservative dose of 5 mg/kg every other day (actual dose 3/4 of an 81-mg tablet, which is 60.75 mg every other day, which is equivalent to 30.4 mg/day = 2.2 mg/kg/day in this patient). This treatment dose is less than half of what was previously reported to be effective.

After the boy had taken only two doses of aspirin, the lesion started decreasing in fullness. After three doses, the family reported that it was significantly improved in appearance and symptoms. In less than 2 weeks the tufted angioma flattened dramatically, lightened in color, and was no longer tender (Fig. 1B).

Because the patient was tolerating the treatment well without side effects, in an attempt to simplify dosing, the regimen was changed to half an 81 mg tablet every day (40.5 mg/day = 2.9 mg/kg/day). Given his excellent response, we plan to continue treatment for several months and then consider decreasing the dose. Lower doses of aspirin may be important for minimizing side effects and to decrease the risk of Reye's syndrome.

We present this case to add to the literature another tufted angioma without KMP that responded dramatically and quickly to low-dose aspirin. This case also demonstrates that even lower doses of aspirin than what had previously been reported may be effective in the treatment of tufted angiomas. Although further reports and studies are needed to research the optimal treatment for these challenging cases, we are hopeful that low-dose aspirin may provide clinical improvement and symptomatic relief and may prevent progression or complications associated with tufted angiomas.

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