Indolent CD8+ lymphoid proliferation of the ear: report of two cases and review of the literature

Abstract

Indolent CD8+ lymphoid proliferation of the ear is a recently described cutaneous lymphoid proliferation that clinically presents with slow growing lesion(s) on the ear(s). In cases reported to date, there has been indolent clinical behavior and no evidence of systemic involvement. Characteristic histopathologic features include a non-epidermotropic diffuse dermal infiltrate of CD8+ T-lymphocytes with a lymphoblast-like appearance. T-cell clonality has also been observed. Herein, we present two patients who show clinical, histopathologic and immunophenotypic features similar to the original index cases described by Petrella et al. In addition, we review the literature regarding this unusual lymphoid proliferation and provide evidence that this entity represents a phenotypic variant of primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma, a concept proposed by Beltraminelli et al. in 2009. Differentiation between this lymphoproliferative process and other more aggressive CD8+ lymphomas is essential in avoiding excessive treatment of a condition that combines worrisome histopathology with indolent clinical behavior.

Long‐term follow‐up and management of small and medium‐sized CD4+ T cell lymphoma and CD8+ lymphoid proliferations of acral sites: a multicenter experience

“…4,5 All three patients included in our study with CD8+ LP had single erythematous nodular lesion on their face. One patient however subsequently developed another lesion located on the back.…”

“…4,5,16 One of the largest studies by Beltaminelli et al, describes 136 patients with CD4+ SMPTCL and follow up of 45 patients suggesting an indolent clinical course. 7 Another recent study by James et al., questions the classification of these entities as lymphoma.…”

“…Often considered as a spectrum of the same disease, both entities are characterized by an indolent clinical course and favorable prognosis unlike other provisional entities in the PTCL, NOS-category. 4,5 In contrast to CD4+ SMPTCL, CD8+ LP does not show a follicular T- helper phenotype suggesting a different cell of origin. PD-1 is often used to differentiate CD8+ LP from CD8+ cases of SMPTCL and reactive infiltrates.…”

Managing Patients with Cutaneous B-Cell and T-Cell Lymphomas Other Than Mycosis Fungoides

“…45,46 Of note, there are reported cases of indolent CD8+ lymphoid proliferation of acral sites (CD8+ LP), which are thought to be low grade lymphomas with clinical presentation similar to CD4+ small/medium pleomorphic T-cell lymphoma (see below), comparable disease course, and infrequent need for systemic therapy, which should not be confused with aggressive CD8+ epidermotropic TCL. 47,48 …”

“…48,50,52–57 Treatment options include local excision, topical/intra-lesional steroids and local radiation. The rates of remission are high irrespective of the treatment modality used, and the relapse rates are low.…”