Acta Dermato-Venereologica (Aug 2020)

Cutaneous Involvement in Waldenström’s Macroglobulinaemia

  • Sarah Stien,
  • 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
Journal volume & issue
Vol. 100, no. 15
p. adv00225

Abstract

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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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