Naslov
Cutaneous Rosai-Dorfman disease: A case report

Autori
Bota, T ; Mužinić, D ; Müller, D ; Tomasović-Lončarić, Č

Vrsta, podvrsta i kategorija rada
Sažeci sa skupova, sažetak, stručni

Izvornik
Book of Abstracts 31-31 / - , 2017, 31-31

Skup
26th Ljudevit Jurak International Symposium on Comparative Pathology

Mjesto i datum
Zagreb, Hrvatska, 02.06.2017

Vrsta sudjelovanja
Poster

Vrsta recenzije
Međunarodna recenzija

Ključne riječi
Rosai-Dorfman ; skin

Sažetak
Rosai-Dorfman disease (RDD) is a rare but distinctive clinicopathologic entity of unknown etiology affecting lymph nodes as well as extranodal sites (43%). In around 3% of patients the disease is limited to the skin. This rare and not well documented form of the disease is known as Cutaneous Rosai-Dorfman disease (CRDD). CRDD typically occurs in older females and clinically presents in various forms, ranging from single papules to indurated plaques and tumor. It often fails to present any laboratory abnormalities, often progresses benignly and is self-limiting, with no need for more aggressive interventions. The hallmark microscopic signs are mixed inflammatory infiltrate with S-100 positive histiocytes with emperipolesis, the most important feature in a correct diagnosis. Our case is a 57-year-old female who presented with a one month history of a tumor located on her face. A white indurated area observed on a skin section microscopically corresponded to a well-restricted, dense inflammatory infiltrate of lymphocyte and plasma cells with numerous multinuclear cells and immunohistochemically S-100 and CD68 (KP1) positive histiocytes with visible emperipolesis. At the time of presentation she had no other systemic, extracutaneous or serologic manifestations. Awareness of the histological aspects present in different lesions, which do not always contain the hallmark microscopic signs of CRDD, is particularly important to correctly diagnose this disorder. Owing to its favorable outcome and spontaneous resolution, CRDD should not be confused with other benign or malignant lesions such as panniculitis, other cutaneous histiocytoses, xanthogranuloma, malignant histiocytosis, hemophagocytic syndrome, reticulohistiocytoma, sarcoidosis, tuberculosis, dermatofibroma or malignant tumors. Despite recurrence occurring in a few patients, surgical intervention remains to be the most effective modality of treatment, especially for solitary or localized lesions. A good response to radiotherapy, cryotherapy and chemotherapy has been documented as well as the use of Thalidomide to control extensive cutaneous disease.

Izvorni jezik
Engleski

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