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Annales de Dermatologie et de Vénéréologie
Vol 134, N° 11  - novembre 2007
pp. 843-846
Doi : AD-11-2007-134-11-0151-9638-101019-200520008
Maladie de Rosai-Dorfman cutanée pure évoluant depuis 19 ans

[1] Service de Dermatologie, CHU de Caen.
[2] Laboratoire d'Anatomie-Pathologique, CHU de Caen.

Tirés à part : D. LEROY [1]

[1] Service de Dermatologie, Centre Hospitalier Universitaire, avenue Georges Clemenceau, 14033 Caen Cedex.


La maladie de Rosai-Dorfman est une histiocytose non langerhansienne atteignant principalement les aires ganglionnaires. Rarement, elle se présente sous la forme de lésions cutanées isolées, sans adénopathie. Il s'agit alors d'une affection bénigne, régressant spontanément en quelques mois ou années.


Un homme de 83 ans avait de multiples lésions nodulaires, rouge brun de la partie supérieure du corps évoluant progressivement depuis 19 ans. L'histologie montrait un infiltrat caractéristique de maladie de Rosai-Dorfman, avec de nombreux îlots histiocytaires dermiques exprimant la protéine S100 et n'exprimant pas le CD1a en immunohistochimie, et un phénomène d'empéripolèse. Les aires ganglionnaires étaient libres, le reste de l'examen clinique et les explorations paracliniques étaient normaux : il s'agissait d'une forme cutanée pure. Un traitement par isotrétinoïne était inefficace. Les lésions s'étendaient progressivement, traitées ponctuellement par laser CO2 ou cryothérapie.


Notre observation est atypique quant à la présentation clinique puisqu'il s'agit de lésions nodulaires diffuses. L'évolution est également inhabituelle car il n'a pas été constaté de régression spontanée malgré un recul de 19 ans.

Purely cutaneous Rosai-Dorfman disease present for 19 years.

Rosai-Dorfman disease is a non-Langerhans histiocytosis chiefly affecting lymph nodes sites. In rare cases, it presents in the form of isolated skin lesions, without adenopathy, in which case it is a benign disease that regresses spontaneously within our number of months and years.

Patients and methods

An 83 year-old man presented with multiple red-brown nodular lesions on the upper part of the body that had been progressing over a period of 19 years. Histological examination showed infiltrate characteristic of Rosai-Dorfman disease, with numerous dermal foci of histiocytes expressing protein S100 but not expressing CD1a on immunohistochemical analysis, as well as emperipolesis. The lymph nodes sites were unaffected, and the remainder of the clinical and laboratory examinations were normal, indicating a purely cutaneous form of the disease. Treatment with isotretinoin was ineffective and the lesions continued to spread gradually, being treated from time to time with CO2 laser or cryotherapy.


Our case is atypical in terms of clinical presentation since it involved diffuse nodular lesions. The disease course was also unusual in that no spontaneous regression was observed even after 19 years.

© 2007 Elsevier Masson SAS. Tous droits réservés.
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