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      Doença de Rosai-Dorfman cutânea: relato de caso Translated title: Cutaneous Rosai-Dorfman disease: a case report

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          Abstract

          A doença de Rosai-Dorfman, também denominada histiocitose sinusal com linfadenopatia maciça, é histiocitose de células não Langerhans, idiopática e de curso benigno. Descrita em 1969, caracteriza-se por linfadenomegalia não dolorosa, sendo a cadeia cervical a mais envolvida, além de febre, perda de peso e sudorese. O envolvimento extranodal ocorre em 43% dos casos, em que múltiplos sítios podem ser acometidos. Já foram descritos casos exclusivamente extranodais, inclusive formas limitadas à pele. Relata-se um caso de doença de Rosai-Dorfman extranodal cutânea pura, devido à raridade dessa apresentação clínica.

          Translated abstract

          Rosai-Dorfman disease, otherwise known as sinus histiocytosis with massive lymphadenopathy, is a non-Langerhans cell histiocytosis with a benign course and unknown etiology. It was described in 1969 as a painless cervical lymph node enlargement in association with fever, weight loss and sweating. Extranodal disease has been reported in 43% of cases, with involvement of multiple organs. Purely extranodal Rosai-Dorfman disease has been already reported, including forms restricted to the skin. This paper reports a case of purely cutaneous Rosai-Dorfman disease, which is of interest in view of the rarity of this condition.

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          Uncommon histiocytic disorders: the non-Langerhans cell histiocytoses.

          Sheila Weitzman, Ronald Jaffe (2005)
          Histiocytic disorders are currently identified by their component cells. The non-Langerhans Cell Histiocytoses (non-LCH) are a group of disorders defined by the accumulation of histiocytes that do not meet the phenotypic criteria for the diagnosis of Langerhans cells (LCs). The non-LCH consist of a long list of diverse disorders which have been difficult to categorize. A conceptual way to think of these disorders that make them less confusing and easier to remember is proposed based on immunophenotyping and clinical presentation. Clinically the non-LCH can be divided into 3 groups, those that predominantly affect skin, those that affect skin but have a major systemic component, and those that primarily involve extracutaneous sites, although skin may be involved. Immunohistochernically many of the non-LCH appear to arise from the same precursor cell namely the dermal dendrocyte. Juvenile Xanthogranuloma (JXG) is the model of the dermal dendrocyte-derived non-LCH. Other non-LCH with differing clinical presentation and occurring at different ages but with an identical immunophenotype appear to form a spectrum of the same disorder, deriving from the same precursor cell at different stages of maturation. They should be considered as members of a JXG family. Non-JXG family members include Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). The non-LCH can be classified as JXG family and non-JXG family and subdivided according to fairly clear-cut clinical criteria. Utilization of this type of approach will allow better categorization, easier review of the literature and more accurate therapy decision-making. (c) 2005 Wiley-Liss, Inc.
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            Cutaneous rosai-dorfman disease is a distinct clinical entity.

            Scott R. Granter, Niamh Leonard, Richard McKee … (2002)
            Rosai-Dorfman disease (RDD) is a rare but distinctive clinicopathologic entity of unknown etiology affecting lymph nodes as well as extranodal sites. Although cutaneous involvement in RDD is common, purely cutaneous disease is rare and not well documented. We report 22 patients with cutaneous and superficial subcutaneous RDD. The lesions presented as papules and nodules, often with discoloration (9/22) and frequent multifocality (13/22), without predilection for a specific site of the body. Age distribution was wide and ranged from 15 to 68 years, with a median of 43.5 years. Of the 17 patients for whom information on racial background was available, 7 were Asian, 8 were white, and 2 were black, with a marked female predominance (2:1). The lesions resolved in 6 of 13 patients for whom follow-up data were available, regardless of the treatment given. Lesions persisted or recurred in 7 patients. Histologically, the lesions are invariably characterized by a proliferation of polygonal S100-positive histiocytes showing emperipolesis and a mixed inflammatory infiltrate. This study characterizes the histologic spectrum of cutaneous RDD in regard to variation in the numbers of typical S100-positive histiocytes and emperipolesis, variation in the quality and quantity of the inflammatory response, and the degree of stromal fibrosis, which resulted in a strikingly storiform growth pattern in six lesions and a lobulated pattern in two lesions. Whereas the clinical as well as histologic appearance of the cutaneous and subcutaneous lesions in the purely extranodal forms of RDD is indistinguishable from that of systemic RDD, this study emphasizes that purely cutaneous RDD is a distinct clinical entity in regard to its epidemiology and remains localized to the skin even with long-term follow-up. Patients with purely cutaneous RDD are of an older age at onset of disease (median = 43.5 years), with a reversed male/female ratio. There are no significant systemic extracutaneous or serologic manifestations. Whereas systemic RDD is commonly seen in blacks and only rarely reported in Orientals, the majority of the patients in this series with purely cutaneous RDD are Asians and whites.
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              Cutaneous Rosai-Dorfman disease: clinicopathological profiles, spectrum and evolution of 21 lesions in six patients.

              David Q-H Wang, Wayne H C Hu, Alice W. Lee … (2006)
              An uncommon histiocytosis primarily involving the lymph nodes, Rosai-Dorfman disease (RDD, originally called sinus histiocytosis with massive lymphadenopathy) involves extranodal sites in 43% of cases; cutaneous RDD (C-RDD) is a rare form of RDD limited to the skin. The clinicopathological diagnosis of C-RDD may sometimes be difficult, with different clinical profiles from those of its nodal counterpart, and occasionally misleading histological pictures. There have been few multipatient studies of C-RDD and documentation of its histological spectrum is rare. To identify the clinical and histopathological profiles, associated features, and the chronological changes of this rare histiocytosis. From 1991 to 2002, patients diagnosed as having C-RDD were collected in four academic hospitals. Clinical presentations, treatments, and courses of each case were documented. In total, 21 biopsy specimens obtained from these patients were re-evaluated and scored microscopically with attention to the uncommon patterns and chronological evolution both clinically and histologically. We examined six patients with C-RDD, three men and three women. The mean age at the first visit was 43.7 years. The clinical presentations were mostly papules, nodules and plaques, varying with the duration and depth of lesions. Although the anatomical distribution was wide, the face was most commonly involved. Evolutional changes were identified clinically, as the lesions typically began with papules or plaques and grew to form nodules with satellite lesions and resolved with fibrotic plaques before complete remission. No patient had lymphadenopathy or extracutaneous lesions during follow-up (mean 50.5 months). At the end of follow-up, the lesions in four patients had completely resolved irrespective of treatment; two patients had persistent lesions. The histopathological pattern of the main infiltrate, the components of cells and the stromal responses showed dynamic changes according to the duration of lesions. The characteristic Rosai-Dorfman cells (RD cells) were found in association with a nodular or diffuse infiltrate in 15 lesions (71%). Four lesions (19%) demonstrated a patchy/interstitial pattern. One lesion (5%) assumed the pattern of a suppurative granuloma. RD cells were less readily found in these atypical patterns. Conspicuous proliferation of histiocytes associated with RD cells was found in three lesions, including xanthoma, localized Langerhans cell histiocytosis and xanthogranuloma. Along with lymphocytes, plasma cells were present in all lesions, often in large numbers with occasional binucleated or trinucleated cells. Variably found in the lesions were neutrophils (nine lesions, 43%) and eosinophils (13 lesions, 62%). The former occasionally formed microabscesses, while the latter were often few in number. Vascular proliferation was a relatively constant feature (90%). Fibrosis was found in 10 lesions (48%). Our study further confirms that C-RDD is a distinct entity with different age and possibly race distributions from RDD. Compared with its nodal counterpart, C-RDD demonstrates a wider histopathological spectrum with different clinicopathological phases depending on duration of the lesions. Awareness of these features is helpful in making a correct diagnosis. The associations of C-RDD with other histiocytoses may have important implications for the pathogenesis of this rare histiocytosis.
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                Author and article information

                Journal
                Journal ID (publisher-id): abd
                Title: Anais Brasileiros de Dermatologia
                Abbreviated Title: An. Bras. Dermatol.
                Publisher: Sociedade Brasileira de Dermatologia (Rio de Janeiro, RJ, Brazil )
                ISSN (Print): 0365-0596
                ISSN (Electronic): 1806-4841
                Publication date (Print and electronic): October 2010
                Volume: 85
                Issue: 5
                Pages: 687-690
                Affiliations
                [01] orgnameFundação Alfredo da Matta
                [02] Manaus AM orgnameSecretaria Municipal de Saúde Brasil
                Article
                Publisher ID: S0365-05962010000500014 Publisher ID: S0365-0596(10)08500514
                DOI: 10.1590/S0365-05962010000500014
                SO-VID: d9a9119a-6676-4c5d-a6b7-8456f113fdeb
                License:

                This work is licensed under a Creative Commons Attribution 4.0 International License.

                History
                Date accepted : 22 September 2008
                Date received : 13 August 2008
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 15, Pages: 4
                Product

                SciELO Brazil

                Categories
                Subject: Caso Clínico

                Keywords: Histiocytosis,Non-Langerhans-cell histiocytosis,Sinus histiocytosis,Histiocitose,Histiocitose de células não Langerhans,Histiocitose sinus
                Data availability:
                Keywords: Histiocytosis, Non-Langerhans-cell histiocytosis, Sinus histiocytosis, Histiocitose, Histiocitose de células não Langerhans, Histiocitose sinus

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