Acral lymphomatoid papulosis (a-LyP) is a rare clinical variant of LyP whose diagnosis may be challenging. A case series of a-LyP was studied clinically, histopathologically, immunohistochemically, and from molecular point of view. Including ours, 25 cases of a-LyP have so far been reported. Clinically, a-LyP may present as acral involvement exclusively, in combination with mucosal lesions, (in itself a rare presentation), or in association with conventional LyP. The age of presentation was slightly higher than that of conventional LyP (55 vs 45 years) and a male predominance has been observed, as usually reported. Histopathologically, no morphological differences exclusively from conventional LyP were observed. LyP types A and E were the main variants. We describe for the first time one case of type D a-LyP. Acral LyP is a rare entity and correct diagnosis can only be reached with clinical and histopathological correlation, to avoid aggressive treatment of this indolent lymphoproliferative disorder.
Keywords: CD30+ lymphoproliferative disorder; localized lymphomatoid papulosis; lymphomatoid papulosis; mucosal lymphomatoid papulosis; primary cutaneous lymphoma.
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