Primary cutaneous acral CD8-positive T-cell lymphoproliferative disorder: A clinical and histologic retrospective...

Clonally restricted, non-epidermotropic, low-grade, CD8-positive T-cell infiltrates of the skin was recognized as a unique form of indolent CD8-positive lymphoproliferative disease in 2007 when it was first called primary cutaneous indolent CD8-positive lymphoid proliferation. More recently, the designation of primary cutaneous acral CD8-positive T-cell lymphoproliferative disorder has been used. It is unique as a cutaneous lymphoproliferative disorder because of relative uniformity in its clinical presentation and histomorphology. It has been recognized as having an interesting predilection for the ear and acral sites, characteristically presenting as a solitary lesion. The basic morphology is one characterized by a non-epidermotropic, tumefactive infiltrate of well-differentiated, noncerebriform, atypical, small- to intermediate-sized lymphocytes that exhibit a specific phenotype characterized by CD8 and TIA positivity in concert with a distinct perinuclear Golgi staining pattern for CD68. The typical presentation is in the context of a solitary lesion, which can be treated surgically or with local irradiation. We describe in detail two very unusual cases that expand the clinical spectrum of this condition given the non-acral localization, the multiplicity of lesions to involve the trunk and extremities, and, in one case, the stable but recalcitrant course over 30 years. In addition, the second patient developed paraneoplastic dermatomyositis. We also retrospectively review our database for other cases that represent the entity of primary cutaneous acral CD8-positive T-cell lymphoproliferative disorder and review the literature focusing on non-acral cases. Nomenclature evolution from its first recognition in 2007 to the present is discussed.