163 posters,  6 topics,  812 authors,  292 institutions

ePostersLive® by SciGen® Technologies S.A. All rights reserved.

P007
The link between chronic inflammatory dermatoses and cutaneous T-cell lymphoma? Case reports and discussion
Board Screen 1 / Wed 8:30, 03 May 2017

Primary tabs

Rate

No votes yet

Statistics

120 reads

The  link between chronic inflammatory dermatoses and cutaneous T-cell lymphoma? Case reports and discussion

Background

Mycosis fungoides (MF) is a cutaneous non-Hodgkin T-cell lymphoma, with heterogeneous clinical aspect, variable course and still unclear ethiopathogenesis.

The relationship between MF and the chronic inflamatory dermatoses, with abnormal T-cell activity mediation, like psoriasis and atopic dermatitis is intriguing. Recently, several common pathogenic features were described but a direct link between MF and these inflammatory dermatoses is still under debate. Moreover, chronic dermatitis and psoriasis are the main clinical differential diagnoses of mycosis fungoides.

We aim to describe the clinico-pathological particularities of patients who developed mycosis fungoides after a long history of atopic dermatitis, respectively psoriasis vulgaris.

Methods and results

CASE REPORT NO. 1

A 44 years old male, with a history of approximately 20 years of atopic dermatitis (with histopathological confirmation) and asthma, presented with generalised alopecia and an eruption of intensely pruritic polymorphic patches and plaques, significantly aggravated over the past 2 years in the context of psychological distress. Several therapeutic options were attempted (systemic corticotherapy, Methotrexate, Azathioprine, Interferon-alpha), with temporary clinical remission.

The blood tests revealed slight leucocytosys, with neutrophilia, high IgE titre, vitamin D deficiency, negative HTLV-1 testing. The patient did not show lymphadenopathy.

Histology and immunohistochemistry tests revealed the  diagnosis of mycosis fungoides, in plaque stage IB [1]. No criteria were met for the diagnosis of systemic involvment.

The treatment with systemic corticosteroids, retinoids (Acitretin 0.5mg/kg), vitamin D supplementation, PUVA phototherapy, and symptomatic topicals, led to  significant clinical improvement after 1 month.

CASE REPORT NO. 2

A 55 years old male, with a 10 years history of psoriasis vulgaris (with histopathological confirmation) and toxoplasmosis, presented with erythroderma, slowly progressing over the past 4 years and generalised scarring alopecia. The patient followed several treatments, including Methotrexate and Infliximab, with significant clinical worsening after the first administration of TNF-alpha blockers. No criteria were met for the diagnosis of Sezary syndrome or systemic involvment.

The paraclinical tests revealed slight leucocytosys, with neutrophilia and eosinophilia, high IgE titre, vitamin D deficiency, negative HTLV-1 testing. Based on the results it was also diagnosed chronic renal disease stage II [1].

Histology and immunohistochemistry tests revealed the  diagnosis of mycosis fungoides, erythrodermic stage IIIA on psoriatic diatesis. A dermopathic lymphadenopathy was diagnosed by echographic examination, punction and histopathologic examination of the lymph nodes.

The treatment with systemic corticosteroids, retinoids (Acitretin 0.5mg/kg), vitamin D supplementation, PUVA phototherapy, and symptomatic topicals, led to  significant clinical improvement after 1 month.

Conclusions

The latest scientific results provide increasing proof of molecular and genetical shared pathogenic features of cutaneous T-cell lymphoma and inflammatory dermatoses. Thymic stromal lymphopoietin (TSLP) is a pro-inflammatory cytokine, of the interleukin-2 (IL-2) family, produced in response to pathogenic stimuli by skin keratinocytes and epithelial cells in the lung and gut, involved in the regulation of type 2 helper T cell (Th2) immunity at barrier surfaces [2]. TSLP might represent a common molecule within the complex cytokine networks of psoriasis vulgaris, atopic dermatitis, and cutaneous lymphoma. While TSLP is described as pivotal to the pathophysiology of allergic diseases mediated by Th2- type responses, including asthma and atopic dermatitis [2], it has recently been suggested that TSLP might act through Th-2 cytokine production to induce cutaneous T-cell lymphoma [3]. Moreover, it was shown that TSLP represents a link between keratinocytes and dendritic cell-derived IL-23 in patients with psoriasis [4].

The presented cases illustrate this link and support the need of further research in the discovery of novel diagnostic biomarkers and therapeutic targets. 

References

1. Willemze R et al. WHO/EORTC classification for cutaneous lymphomas. Blood 2005; 105:3768–85.

2. Verstraete K et al Structure and antagonism of the receptor complex mediated by human TSLP in allergy and asthma. Nat Commun 2017; 8:14937

3. Takahashi N et al. Thymic Stromal Chemokine TSLP Acts through Th2 Cytokine Production to Induce Cutaneous T-cell Lymphoma. Cancer Res. 2016;76(21):6241-6252.

4. Volpe E et al. Thymic stromal lymphopoietin links keratinocytes and dendritic cell-derived IL-23 in patients with psoriasis. J Allergy Clin Immunol. 2014 Aug;134(2):373-81. 

Enter Poster ID (e.gGoNextPreviousCurrent