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Lymphome B cutané au cours d’une sclérodermie systémique

Cutaneous B-cell lymphoma in a patient with systemic sclerosis

  • Cas Clinique / Case Report
  • Published:
Oncologie

Abstract

Background

Systemic sclerosis seems to be a predisposing factor malignant neoplasm, particularly lung and breast carcinomas. The occurrence of malignant lymphoma in this disease is rare. We report a case showing the recurrence of cutaneous B-cell lymphoma during a systemic sclerosis.

Case report

A 56 year-old woman presented with a systemic sclerosis with Raynaud’s phenomenon, sclerodactyly, pulmonary and esophageal involvements. The antinuclear antibodies were positive with a titer of 1/320 and the antiScl70 antibodies were also positive. Ten years after the onset of her disease, the patient developed a cutaneous B-cell lymphoma of the left supra orbital region. Regression of the cutaneous lesion was obtained after chemotherapy followed by local radiation therapy. However, six years later, she developed a recurrence of the cutaneous B-cell lymphoma in both cheeks.

Discussion

The association of systemic sclerosis and malignant lymphoma is rare. In this rheumatic disease, there is a predominance of B-cell malignant lymphoma. The lack of course correlation in the many cases described suggests the occasional nature of this association. However, the improvement of the scleroderma and the lymphoma, reported by some authors, suggests a close link between the two diseases. Autologous stem cell transplantation and rituximab may be proposed in the treatment of recurrent malignant lymphoma associated with systemic sclerosis.

Résumé

Introduction

La sclérodermie systémique semble prédisposer à la survenue des néoplasies. Il s’agit essentiellement de carcinomes bronchiques et de carcinomes mammaires. La survenue du lymphome malin au cours de cette connectivite reste rare. Nous rapportons un cas de sclérodermie systémique qui se complique d’un lymphome B cutané à grandes cellules.

Observation

Une femme âgée de 56 ans, qui présente une sclérodermie systémique avec un phénomène de Raynaud, une sclérodactylie, une atteinte pulmonaire et oesophagienne. Dix ans après le début de sa maladie, la patiente a présenté un lymphome B cutané à grandes cellules au niveau de la région sus-orbitaire gauche. Elle a bénéficié d’une chimiothérapie, suivie d’une radiothérapie locale avec une régression de la lésion cutanée. L’évolution ultérieure était marquée par la récidive du lymphome B cutané au niveau des deux joues après cinq ans et demi.

Discussion

L’association d’une sclérodermie systémique et d’un lymphome malin est rare. Les lymphomesmalins non hodgkiniens de phénotype B prédominent au cours de cette connectivite. L’absence de corrélation évolutive des deux pathologies dans la plupart des cas rapportés peut être en faveur du caractè re fortuit de cette association. Néanmoins, une étroite relation entre les deux affections a été suggérée devant leur évolution parfois parallèle après chimiothérapie. L’autogreffe de la moelle et le rituximab peuvent constituer des alternatives thérapeutiques intéressantes en cas de récidive d’un lymphome malin au cours d’une sclérodermie systémique.

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Authors and Affiliations

  1. Service de médecine interne, CHU Hédi-Chaker, T-3029, Sfax, Tunisie

    M. Frigui, N. Kaddour, S. Marzouk & Z. Bahloul

  2. Service de dermatologie et de vénéréologie, CHU Hédi-Chaker, T-3029, Sfax, Tunisie

    M. Mseddi & H. Turki

  3. Laboratoire d’anatomie pathologique et de cytologie, CHU Habib-Bourguiba, T-3029, Sfax, Tunisie

    T. Boudaouara

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  1. M. Frigui
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  2. N. Kaddour
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  3. M. Mseddi
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Correspondence to M. Frigui.

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Frigui, M., Kaddour, N., Mseddi, M. et al. Lymphome B cutané au cours d’une sclérodermie systémique. Oncologie 12 (Suppl 1), 21–25 (2010). https://doi.org/10.1007/s10269-008-0848-9

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  • DOI: https://doi.org/10.1007/s10269-008-0848-9

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