Multifocal Rosai-Dorfman disease with involvement of the pinna

Hirt, Molly B.; Heskett, Jordan B.; Veerula, Vindhya; Warren, Simon; Avashia-Khemka, Nidhi; Mark, Lawrence A.

doi:10.1016/j.jdcr.2017.02.008

Cited by 5 publications

(9 citation statements)

References 11 publications

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“…In patients with RDD, bilateral and painless cervical lymphadenopathy is the most prominent clinical manifestation [1][2][3][4][5][9][10][11][12]. Axillary, inguinal, mediastinal, and retroperitoneal lymphadenopathy has been recorded to a minor degree [1,2,4].…”

Section: Discussionmentioning

confidence: 99%

“…Axillary, inguinal, mediastinal, and retroperitoneal lymphadenopathy has been recorded to a minor degree [1,2,4]. Extranodal involvement, such as the skin [1,[3][4][5]11], scalp, thyroid gland [12], palatine tonsil, soft tissues of the orbit, eyelid, and testicle [1,3], rhinopharynx [9], bone and breast, central nervous system, and gastrointestinal tract, has been reported in patients with RDD [1,3,4]. Even though presentation with fever, night sweats, malaise, and weight loss is common in patients with RDD, they maintain a good general condition [1,3,4,10].…”

Section: Discussionmentioning

confidence: 99%

“…Sinus histiocytosis with massive lymphadenopathy (SHML), also known as Rosai-Dorfman disease (RDD), was first described by Rosai et al [1][2][3][4]. e disease is a benign histiocytic disorder with massive lymphadenopathy [2][3][4][5] and is usually self-limiting, with an unknown cause [2,3]. Bilateral, painless, massive lymphadenopathy of the cervical lymph nodes is typical with the usual involvement of the other lymph nodes [2][3][4][5].…”

Section: Introductionmentioning

confidence: 99%

“…e disease is a benign histiocytic disorder with massive lymphadenopathy [2][3][4][5] and is usually self-limiting, with an unknown cause [2,3]. Bilateral, painless, massive lymphadenopathy of the cervical lymph nodes is typical with the usual involvement of the other lymph nodes [2][3][4][5]. Cases with extranodal involvement [5][6][7] or disease limited to the skin has been reported [4].…”

Section: Introductionmentioning

confidence: 99%

“…Bilateral, painless, massive lymphadenopathy of the cervical lymph nodes is typical with the usual involvement of the other lymph nodes [2][3][4][5]. Cases with extranodal involvement [5][6][7] or disease limited to the skin has been reported [4]. Affected tissues demonstrate marked infiltration of plasmacytosis and of histiocytes that exhibit emperipolesis [7].…”

Section: Introductionmentioning

confidence: 99%

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Long-Lasting Follow-Up with Low-Dose Steroid in an 18-Year-Old Male with Rosai–Dorfman Disease

Karakuş

Dere

Soysal

2020

Case Reports in Medicine

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Rosai–Dorfman disease (RDD) is a rare and benign pathology of sinus histiocytosis of unknown etiology. Lymphadenopathy is the predominant clinical manifestation, but diverse organs can also be affected. Histological features involve S-100+ histiocytes with characteristic nuclear features within the enlarged sinusoids of the lymph nodes. The clinical course is unpredictable, but is often benign with spontaneous resolution of disease in most patients. We report a patient with bilateral massive enlargement of cervical, axillary, and inguinal lymph nodes, moderately enlarged spleen, and a weight loss of 15 kg. Excisional biopsy from the cervical lymph node showed that the dilated sinusoids were infiltrated by lymphocytes, plasma cells, and large histiocytes with CD 68 and S-100 protein positive. Due to the slow progression of the disease, oral prednisolone with a body weight of 1 mg/kg was started in March 2016. The steroid dosage has been adjusted many times during the clinical follow-up. After 33 months, steroid treatment resulted in partial shrinkage of lymph nodes, the spleen returned to its normal size, and the patient gained weight. After 38 months of follow-up, no systemic symptoms, sign, or extranodal involvement were detected, and the patient continued with low-dose steroid treatment.

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