10.5826/dpc.1401a43
text/xml
2160-9381
3469
en
Leonardo  Zichichi
https://creativecommons.org/licenses/by-nc/4.0
Dermatology Practical & Conceptual
clinical practice
Multifocal Cutaneous Rosai-Dorfman Disease Treated With Localized Radiotherapy
Text.Serial.Journal
https://dpcj.org/index.php/dpc
Research Letter
2024-01-31
2023-04-25
2024-01-31
2024-02-21
e2024043
Dermatol Pract Concept
Leonardo  Zichichi
2024/01/31
10.5826/dpc.1401a43
e2024043
Dermatology Unit, Ospedale S.A. Abate, Trapani, Italy
2160-9381
clinical practice
en
e2024043
Choi R, Ring N, McNiff JM, Wilson LD, Martin K, Leventhal J. Treatment of multifocal cutaneous Rosai-Dorfman disease with radiotherapy. JAAD Case Rep. 2022 Mar 31;23:106-108. doi: 10.1016/j.jdcr.2022.03.027. PMID: 35495976; PMCID: PMC9039858.
Multifocal Cutaneous Rosai-Dorfman Disease Treated With Localized Radiotherapy
https://dpcj.org/index.php/dpc/article/download/3469/2528
         Multifocal Cutaneous Rosai-Dorfman Disease
             Treated With Localized Radiotherapy
            Gaetano Licata1, Giovanni Spanò2, Domenico Messina2, Antonino Daidone3,
                                       Leonardo Zichichi1


1 Dermatology Unit, Ospedale S.A. Abate, Trapani, Italy
2 Pathology Unit, Ospedale S.A. Abate, Trapani, Italy
3 UO Radioterapia Oncologica, Villa Santa Teresa, Bagheria, Palermo, Italy



Key words: Cutaneous Rosai-Dorfman, radiotherapy, rare disease, clinical practice
Citation: Licata G, Spanò G, Messina D, Daidone A, Zichichi L. Multifocal Cutaneous Rosai-Dorfman Disease Treated With Localized
Radiotherapy. Dermatol Pract Concept. 2024;14(1):e2024043. DOI: https://doi.org/10.5826/dpc.1401a43
Accepted: August 17, 2023; Published: January 2024
Copyright: ©2024 Licata et al. This is an open-access article distributed under the terms of the Creative Commons Attribution-
NonCommercial License (BY-NC-4.0), https://creativecommons.org/licenses/by-nc/4.0/, which permits unrestricted noncommercial use,
distribution, and reproduction in any medium, provided the original authors and source are credited.
Funding: None.
Competing Interests: None.
Authorship: All authors have contributed significantly to this publication.
Corresponding Author: Dr. Gaetano Licata, MD, Dermatology Unit, Ospedale S.A. Abate, Trapani, Via Cosenza 82, 91016, Erice (TP),
Italy. Phone: 3276215976 E-mail gaetano.licata89@gmail.com




Introduction                                                             plaques (Figure 1, A and B). There were no systemic symp-
                                                                         toms and no palpable lymphadenopathy. We carried out
Rosai-Dorfman disease (RDD) is a rare histiocytic disorder               another skin biopsy of papules of breast and thigh and
characterized by a benign proliferation of S100-positive his-            the histological features confirmed the diagnosis of RDD
tiocytes [1]. Skin involvement is present in 10% of cases: a             (Figure 2, A-C). To rule out systemic disease, a whole body
purely cutaneous form (CRDD) is rare (3% of all cases) and               computed tomography scan (CT) was performed with no
has an older mean age of onset (43 years) than nodal disease             evidence of organ involvement but showing a 6.3x5.9x7.2
(20 years) [2].                                                          cm lesion in the left breast and 16.6x7.5x15.9 cm in the
                                                                         right thigh. Laboratory work-up included: full blood count,

Case Presentation                                                        liver function test, renal parameters, C-reactive protein, vi-
                                                                         ral serology (HIV, HBV, HCV), ANA, rheumatoid factor
A 38-year-old woman came to our attention presenting with                were carried out. Results were all within normal parame-
a hyperpigmented infiltrated plaque in her left breast and               ters. These findings confirmed a diagnosis of CRDD without
right thigh for six months. The patient had already under-               evidence of systemic RDD. As it was a localized cutaneous
gone biopsy with the diagnosis of: fibroadipose tissue, with             form, it was treated with topical clobetasol propionate twice
lymphoplasmacytic and histiocytic infiltrate and presence                daily for 2 months with little benefit. Given the subsequent
of foamy histiocytes. Clinical evaluation revealed numerous              worsening, the case was discussed in a multidisciplinary
pink-purple papulo-nodules with multiple yellowish-white                 consultation with a radiotherapist and an oncologist, and it
areas, in the context of well-circumscribed infiltrated                  was decided to treat the patient with localized radiotherapy




Research Letter | Dermatol Pract Concept. 2024;14(1):e2024043                                                                        1
                  Figure 1. A-D. (A,B) Pink-violaceous papulonodules and plaques on left breast and right thigh at the
                  time of the initial dermatology consultation (6.3x5.9x7.2 cm in the left breast and 16.6x7.5x15.9 cm
                  in the right thigh). (C,D) Near-complete response at 4-month follow-up after completion of radio-
                  therapy left breast lesion measured 3.21 x 3.68 x 3.4 cm (-47%), right thigh measured 10.56 x 5.4
                  x 7.8 cm (-38%).




                  Figure 2. A-C. (A) Infiltrate consisting mainly of histiocytes mixed with neutrophils, plasma cells
                  and T lymphocytes. H&E, original magnification: 4x. (B) Emperipolesis phenomena (presence of an
                  intact cell within the cytoplasm of another cell) and histiocytes with granular cytoplasm (square.)
                  H&E, original magnification: 40x. (C) Immunohistochemical shows positivity for S100 original
                  magnification: 20x.



at a dose of 40 Gy in 20 fractions of 2 Gy. The patient tol-          (-47%), right thigh measured 10.56 x 5.4 x 7.8 cm (-38%)
erated radiotherapy well with only mild radiodermatitis. A            and an almost complete disappearance of radiodermatitis
follow-up whole body CT was performed four months after               was observed (Figure 1, C and D). We hope for further im-
radiotherapy which showed a marked reduction in treated               provement, however a possible surgical treatment is being
lesions, left breast lesion measured 3.21 x 3.68 x 3.4 cm             evaluated.




2                                                                         Research Letter | Dermatol Pract Concept. 2024;14(1):e2024043
Conclusions                                                     References
CRDD is a very rare entity, the diagnosis may be suspected      1. Emile JF, Abla O, Fraitag S, et al. Revised classification of his-
                                                                   tiocytoses and neoplasms of the macrophage-dendritic cell lin-
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of cases [4]. Treatment options for CRDD include topical and       ment of cutaneous Rosai-Dorfman disease. Ann Diagn Pathol.
systemic corticosteroids, dapsone, thalidomide, isotretinoin,      2019;40:166-173. DOI: 10.1016/j.anndiagpath.2019.02.004.
imatinib, surgical excision (for localized form), chemother-       PMID: 31108464.
                                                                3. Bruce-Brand C, Schneider JW, Schubert P. Rosai-Dorfman dis-
apy. But also, physical treatments such as cryotherapy, local
                                                                   ease: an overview. J Clin Pathol. 2020;73(11):697-705. DOI:
radiotherapy and laser therapy. As suggested by Choi et al,
                                                                   10.1136/jclinpath-2020-206733. PMID: 32591351.
localized radiotherapy should be considered a valid therapeu-   4. Gaul M, Chang T. Cutaneous Rosai-Dorfman disease. Cutis.
tic option for CRDD, which is challenging to excise or with        2019;103(3):171-173. PMID: 31039225.
aesthetic impact and for neoadjuvant purposes [5].              5. Choi R, Ring N, McNiff JM, Wilson LD, Martin K, ­Leventhal J.
   A multidisciplinary approach in the treatment of rare           Treatment of multifocal cutaneous Rosai-Dorfman disease
                                                                   with radiotherapy. JAAD Case Rep. 2022;23:106-108. DOI:
skin diseases such as CRDD is important. Localized radio-
                                                                   10.1016/j.jdcr.2022.03.027. PMID: 35495976. PMCID:
therapy could be a valid therapeutic option, furthermore in
                                                                   PMC9039858.
order to achieve standard dosing guidelines, new case sub-
missions are essential.




Research Letter | Dermatol Pract Concept. 2024;14(1):e2024043                                                                      3
