Medullary CD30+ T Cell Lymphoma with Eosinophilia and Hyper-IgE Supervening during the Relentless Course of Pityriasis lichenoides

Hermanns‐Lê, Trinh; Ge, Piérard

doi:10.1159/000018358

Cited by 8 publications

(5 citation statements)

References 16 publications

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“…(27), (72), (80) Parenthetically, association or progression to mycosis fungoides or another lymphoma type has been documented in PLEVA. (14), (26), (33), (40), (45), (54), (60), (64), (66), (8) Remarkably, eight of our 13 PLEVA cases (62%) displayed a high number of CD8-positive cells and showed coexpression of CD8 and CD30 in the intraepidermal and dermal component of the infiltrate. Therefore and due to the presence of necrotic keratinocytes, some of our cases histologically simulated primary cutaneous aggressive epidermotropic CD8positive T-cell lymphoma (CD8+ AECTCL).…”

Section: Molecular Biological Findingsmentioning

confidence: 65%

Pityriasis Lichenoides et Varioliformis Acuta With Numerous CD30+ Cells

Kempf

Kazakov

Palmedo

et al. 2012

American Journal of Surgical Pathology

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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.

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Section: Molecular Biological Findingsmentioning

confidence: 65%

Pityriasis Lichenoides et Varioliformis Acuta With Numerous CD30+ Cells

Kempf

Kazakov

Palmedo

et al. 2012

American Journal of Surgical Pathology

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“…MF, Hodgkin lymphoma, and other lymphomas have been rarely reported in patients with PL. 8,38,[58][59][60][61][62][63][64][65][66][67][68] Whether some of these cases are really unrecognized LyP rather than PL is unclear.…”

Section: Discussionmentioning

confidence: 99%

Lymphomatoid Papulosis Followed by Pityriasis Lichenoides: A Common Pathogenesis?

Vonderheid¹,

Kadin²,

Gocke³

2011

The American Journal of Dermatopathology

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Pityriasis lichenoides (PL) and lymphomatoid papulosis (LyP) are uncommon idiopathic eruptions with overlapping clinical and histological features. Although current opinion indicates that PL and LyP are distinct and separate entities, molecular genetic evidence of T-cell clonality in both conditions suggests that an etiopathogenic relationship may exist. We report a patient who was diagnosed with LyP type B in 1985 followed by PL after 11 years. We hypothesize that LyP followed by PL in the same patient reflects differences in the host immune response to a common antigenic stimulus.

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“…The reported fatalities included the occurrence of potential complications of treatment (sepsis in 9/14 cases, pulmonary embolism in 2/14 cases, and cardiac arrest in one case). 1,10,19,34,41 The more severely affected patients received more intense immunosuppressive treatment with a significant positive correlation of systemic steroid application to systemic involvement and mucosal involvement. For the use of systemic steroids, we could demonstrate a trend for an increased likelihood of an adverse outcome (Table 4).…”

Section: Literature Datamentioning

confidence: 99%

Mucha‐Habermann disease: a pediatric case report and proposal of a risk score

et al. 2021

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Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare inflammatory dermatological disease. A case of a 13-year-old boy with FUMHD possibly triggered by mycoplasma infection is presented. Based on FUMHD cases identified in a MEDLINE literature search, demographic, treatment, and outcome data were analyzed. An FUMHD mortality risk score is proposed based on the likelihood ratios of risk factors for a fatal outcome. Our FUMHD case had marked leukopenia and thrombocytopenia at admission. He recovered without systemic immunosuppressive treatment. Literature review revealed 119 FUMHD cases. Overall lethality was 14/119 (12%, CI 6-17%), and lethality in children was lower (1/54, 2%, CI 0-6%) compared to adults (13/65, 20%, CI 11-31%). Risk factors for a fatal outcome (likelihood ratio; P) were sepsis (24.97, P < 0.001), adult vs. pediatric patient age (11.19; P = 0.001), systemic involvement (19.97, P < 0.001), and mucosal involvement (4.58; P = 0.032). The proposed FUMHD mortality risk score = Age/ 10 + 4 + 4 (if systemic involvement) + 1 (if mucosal involvement) was discriminative (sensitivity 93%, specificity 77%). In FUMHD, immune-suppressive treatment intensity should be balanced against the mortality risk, as infectious complications are a frequent cause of death.

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Medullary CD30+ T Cell Lymphoma with Eosinophilia and Hyper-IgE Supervening during the Relentless Course of Pityriasis lichenoides

Cited by 8 publications

References 16 publications

Pityriasis Lichenoides et Varioliformis Acuta With Numerous CD30+ Cells

Pityriasis Lichenoides et Varioliformis Acuta With Numerous CD30+ Cells

Lymphomatoid Papulosis Followed by Pityriasis Lichenoides: A Common Pathogenesis?

Mucha‐Habermann disease: a pediatric case report and proposal of a risk score

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