Cutaneous Involvement in Waldenström’s Macroglobulinaemia

Authors

  • Sarah Stien Department of Oncodermatology, Robert Debré Hospital, avenue du Général Koenig, FR-51092 Reims Cedex, France
  • Eric Durot
  • Anne Durlach
  • Marie Beylot-Barry
  • Henri Adamski
  • Helmut Beltraminelli
  • Gérôme Bohelay
  • Agnès Carlotti
  • Olivier Carpentier
  • Pascale Cornillet
  • Romain Dubois
  • Nathalie Franck
  • Bérengère Husson
  • Liliane Laroche
  • Eve Maubec
  • Christian Le Clech
  • Laurent Machet
  • Nicolas Ortonne
  • Caroline Ram-Wolff
  • Béatrice Vergier
  • Florent Grange

DOI:

https://doi.org/10.2340/00015555-3535

Keywords:

Waldenström’s macroglobulinaemia, cutaneous lymphoma, diffuse large B-cell lymphoma, histological transformation, extranodal involvement, MYD88 L265P mutation

Abstract

Cutaneous involvement in Waldenström’s macroglobulinaemia (WM) has been poorly characterized. To describe this involvement, a retrospective study of 19 patients with WM and cutaneous involvement of tumour B cells was performed. Twelve patients (group 1) had lymphoplasmacytic, non-transformed cutaneous proliferation, while in 7 cases (group 2) cutaneous involvement corresponded to histological transformation. In group 1, skin involvement was inaugural in 6 cases. The lesions were infiltrated plaques (83%), papules (25%) and tumours (42%). Four patients had a similar clinical picture (purplish, bilateral and symmetrical infiltration on the face). MYD88 L265P mutation was detected in the skin biopsy in all 6 cases tested. The 3-year specific survival rate was 88%. In group 2, cutaneous transformation occurred during the follow-up of the WM (71%). Lesions presented as ulcerated tumours (86%) of the trunk (57%) and lower limbs (57%). The 3-year specific survival rate was 22%. Skin involvement in WM has distinctive characteristics (e.g. clinical, histological, immunohistochemical, MYD88 L265P mutation).

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Published

2020-08-17

How to Cite

Stien, S., Durot, E., Durlach, A., Beylot-Barry, M., Adamski, H., Beltraminelli, H., … Grange, F. (2020). Cutaneous Involvement in Waldenström’s Macroglobulinaemia. Acta Dermato-Venereologica, 100(15), adv00225. https://doi.org/10.2340/00015555-3535