Pityriasis Lichenoides et Varioliformis Acuta With Numerous CD30+ Cells

Abstract

Pityriasis lichenoides comprises a clinicopathologic spectrum of cutaneous inflammatory disorders, with the 2 most common variants being pityriasis lichenoides et varioliformis acuta (PLEVA) and pityriasis lichenoides chronica. The aim of the study was to describe 13 cases of a unique PLEVA variant characterized in the conspicuous CD30 component and thus mimicking lymphomatoid papulosis (LyP), a condition currently classified in the spectrum of CD30 lymphoproliferative disorders. The cohort included 10 female and 3 male patients whose ages at diagnosis ranged from 7 to 89 years (mean 41 y; median 39 y). The clinical manifestation was that of PLEVA, with small erythematous macules quickly evolving into necrotic papules. No waxing and waning was seen on follow-up in any of the cases. Histopathologically, typical features of PLEVA were present, but an unusual finding was occurrence of a considerable number of CD30 small lymphocytes as detected immunohistochemically. Over half of the cases also displayed a large number of CD8 cells and showed coexpression of CD8 and CD30 in the intraepidermal and dermal component of the infiltrate. Of the 11 cases of PLEVA studied for T-cell receptor gene rearrangement, 6 evidenced a monoclonal T-cell population, and 5 were polyclonal. Parvovirus B19 (PVB19) DNA was identified in 4 of 10 cases investigated, and positive serology was observed for PVB19 in 2 patients, altogether suggesting that PVB19 is pathogenetically linked to PLEVA at least in a subset of cases. The presence of CD30 lymphocytes and CD8 lymphocytes would be consistent with an inflammatory antiviral response, as CD30, even atypically appearing lymphoid cells have been identified in some viral skin diseases. The main significance of the PLEVA variant is, however, its potential confusion with LyP or some cytotoxic lymphomas. Admittedly, the CD30 PLEVA variant described herein and LyP show considerable overlap if one takes into account all known variations of the 2 conditions recognized in recent years, thus suggesting that LyP and PLEVA may be much more biologically closely related entities than currently thought or can even occur on a clinicopathologic spectrum.

Significance of CD30 Expression by Epidermotropic T Cells in Lymphomatoid Papulosis and Lymphomatoid Pityriasis Lichenoides

“…This observation may be more than fortuitous considering that chronic virus infections including parvovirus B19 are suspected to trigger pityriasis lichenoides, LyP, and lupus erythematosus. [3,[21][22][23][24] We conclude that CD30 may be rarely expressed not only by CD8+ cells in some cases of PLEVA as reported by Kempf,[3] but also by epidermotropic T cells in lesions with histopathologic features of LyP. Our observation that one of our patients had both types of lesions suggests the possibility of a related histogenesis between LyP and CD30+ PLEVA, We demonstrate the variability of CD30 positivity in this heterogeneous population of selfhealing papulonodules.…”

“…CD30+ epidermotropic T cells may also occur in PLEVA and mycosis fungoides. [3,19] It is conceivable that three of the LyP-A/B cases (patients 4 to 6) are examples of lymphomatoid CD30+ PLEVA, especially patient 6 whose lesions showed features of both entities over time. Given that CD30 is an lymphocyte activation antigen and that normal T cells when stimulated in vitro via CD3 or CD2 in the presence of phorbol esters can develop cerebriform nuclei, [20] we hypothesize that CD30+ cerebriform lymphocytes in some cases might be the result of accumulation of benign stimulated CD8+ and CD4+ T cells rather than neoplastic cells.…”

“…Three of our 10 patients may have CD30+ PLEVA although one patient (described below) also had specimens that were typical of LyP, an observation that supports the concept of a close pathogenic relationship exists between these conditions. [3,5,6] We also discovered a possible association of CD30+ cutaneous lymphoproliferative disorders with undeclared lupus erythematosus.…”

“…[1,2] Recently, Kempf and coworkers reported 13 unusual cases of the acute ulceronecrotic form of pityriasis lichenoides, i.e., pityriasis lichenoides et varioliformis acuta (PLEVA) or Mucha-Habermann disease, in which a large proportion of lymphocytes within the epidermis and dermis expressed CD30. [3] As is frequently observed in PLEVA lesions, a large proportion of lymphocytes in the dermal infiltrate and overlying epidermis of their cases expressed CD8 (median, 70%; range 50 to 90%). However unlike typical PLEVA, CD30 was co-expressed by these cells (median, 60%; range, 15 to 80%).…”

Parvovirus B19: Un virus ADN asociado a múltiples manifestaciones cutáneas

“…Otro estudio incluyó 10 pacientes con PLEVA y 17 controles con papulosis linfomatoide para estudio de RPC para ADN de PVB19 en piel lesional. Se identificó PVB19 en tres (30%) pacientes con PLEVA; sin embargo, no se pudo detectar en los controles alguno 64 .…”