ABSTRACT
Acral pseudolymphomatous angiokeratoma of children (APACHE) is a recently recognized, rare clinical entity with only 20 reported cases worldwide. It is characterized by unilateral eruptions of multiple angiomatous papules, mostly on acral areas and mainly in children, although some cases have also been reported with involvement of non-acral areas and occurring in adults. The histopathologic examination revealed diffuse dermal infiltrates of lymphohistiocytes and thick-walled vessels lined with prominent plump endothelial cells. APACHE is a cutaneous pseudolymphoma, rather than a vascular neoplasm, because of distinct histopathologic and immunohistochemical findings. We report a case of a 9-year-old girl who presented with multiple angiomatous papules on her right upper arm. The histopathologic and immunohistochemical findings in our patient were consistent with a diagnosis of APACHE.