Acral pseudolymphomatous angiokeratoma: case report and literature review

Abstract

The authors describe a case of a female patient with Acral Pseudolymphomatous Angiokeratoma of Children, known as APACHE. It is a rare benign cutaneous disease, of unknown etiology, characterized by multiple, asymptomatic erythematous-violaceous papules and nodules, usually located unilaterally with acral distribution. Today, this denomination is questionable, since there are published reports of this disease in adults and in different locations. Clinically, it is similar to an angiokeratoma, whereas hystologically, it corresponds to a distinct type of pseudolymphoma. The immunohistochemical study is required to distinguish APACHE from cutaneous lymphoma.

Linear acral pseudolymphomatous angiokeratoma of children with associated nail dystrophy

“…As the name suggests, most people diagnosed with this entity are children, but this diagnosis has been made in adults as well. The majority of patients are females (81%) and most, but not all, lesions are located on the upper or lower extremities [1][2][3][4][5]. In our case, the heavy infiltrate of benign lymphocytes, proliferation of blood vessels, and acral location in a young female were consistent with the diagnosis of acral pseudolymphomatous angiokeratoma of children (APACHE).…”

“…Previous cases have demonstrated a female preponderance with 81% of cases occurring in young girls between the ages of 2 and 13. Clinically, lesions can be variable in appearance but often have an angiomatous quality with erythematous to violaceous color [1]. We report a 10-year-old girl with linearly-arrayed pseudolymphomatous papules on the index finger with accompanying dystrophy of the index nail plate.…”

Use of topical rapamycin in acral pseudolymphomatous angiokeratoma of children (APACHE): A report of two cases and review of the literature

“…Total surgical excision or curettage appeared to be the most successful treatment in previously reported cases: six patients were found to have complete resolution of the lesions without recurrence 2‐7 . In contrast, all seven patients who received steroids (topical or intralesional) had either incomplete resolution of the lesions or recurrence, despite experiencing initial improvement 7‐13 .…”

“…One patient experienced improvement without any treatment 2 . Cryotherapy and radiotherapy are other potential treatment options 5,20 …”

“…Histopathology shows a dense subepidermal lymphohistiocytic infiltrate, and immunohistochemistry shows a mixed population of B and T cells. Total surgical excision is the most successful treatment in preventing recurrence, 2‐7 while steroids (topical or intralesional) are associated with either incomplete resolution or recurrence of the lesions 7‐13 . While topical rapamycin has been used successfully in vascular proliferations such as facial angiofibromas due to its inhibitory effects on mTOR and angiogenesis, 14‐18 it has not been explored for APACHE or other pseudolymphomas, although one study explored the role of topical tacrolimus in lymphocytoma cutis, another type of pseudolymphoma 19 .…”