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An 8-year-old boy was born with a vascular mass involving the left axilla. The affected skin showed a violaceous purpuric plaquelike lesion with induration and telangiectasia (Figure). His blood cell count was notable for severe thrombocytopenia (platelet count of 17 × 103/μL [to convert to ×109/L, multiply by 1]). Hemoglobin level, white blood cell count, prothrombin time/international normalized ratio, partial thromboplastin time, fibrinogen level, and D-dimer level were normal.
Clinical photographs of the left axilla at 4 months, 2.5 years, 5.5 years, and 8 years of age (A, B, C, and D, respectively).
Treatment with oral corticosteroids for 1 year showed variable response. At the age of 14 months, treatment with vincristine was initiated. This resulted in normalization of the platelet count and a decrease in the mass size. On the last clinic visit 6 years after discontinuing vincristine treatment, the platelet count was 343 × 103/μL and the lesion continued to shrink (Figure). However, clinical examination showed atrophy of the muscles and soft tissue of the left axilla and chest wall with some limitation of the range of motion of the left shoulder. The family and prenatal histories were noncontributory.
What is your diagnosis?
Alomari AK, Alomari AI. Picture of the Month—Quiz Case. Arch Pediatr Adolesc Med. 2010;164(4):387. doi:10.1001/archpediatrics.2010.39-a