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Rosai-Dorfman disease presenting with extensive cutaneous manifestation - case report.

Leal PA, Adriano AL, Breckenfeld MP, Costa IS, de Sousa AR, Gonçalves Hde S - An Bras Dermatol (2013 Mar-Apr)

Bottom Line: In its typical form the disease is characterized by extensive cervical lymphadenopathy associated with fever, polyclonal gammopathy and leukocytosis with neutrophilia.The skin is the most common site affected.Extranodal manifestations have been reported in 43% of cases.

View Article: PubMed Central - PubMed

Affiliation: Dermatology Department, Dona Libânia Dermatology Center, Fortaleza, CE, Brazil. lealpauladl@gmail.com

ABSTRACT
Rosai-Dorfman disease is a benign, self-limited, idiopathic proliferative histiocytic disorder. It was first described in 1969 by Rosai and Dorfman. In its typical form the disease is characterized by extensive cervical lymphadenopathy associated with fever, polyclonal gammopathy and leukocytosis with neutrophilia. The skin is the most common site affected. Extranodal manifestations have been reported in 43% of cases. In this study, we report an atypical case of Rosai-Dorfman disease in a female with massive cutaneous manifestation on the thigh, associated with a minimal lymphadenopathy limited to the regional inguinal lymph nodes.

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Indurated hyperpigmented plaque with reddish-yellow nodules on the right thigh
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f01: Indurated hyperpigmented plaque with reddish-yellow nodules on the right thigh

Mentions: The examination revealed an indurated hyper-pigmented plaque with reddish-yellownodules, on her right thigh, measuring 15 cm in diameter (Figure 1). An erythematous nodule was presented on her right buttock (Figure 2). The patient reported that these lesionswere painless. A painless inguinal lymph node on the same side of the skin lesions wasalso revealed upon examination.


Rosai-Dorfman disease presenting with extensive cutaneous manifestation - case report.

Leal PA, Adriano AL, Breckenfeld MP, Costa IS, de Sousa AR, Gonçalves Hde S - An Bras Dermatol (2013 Mar-Apr)

Indurated hyperpigmented plaque with reddish-yellow nodules on the right thigh
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3750892&req=5

f01: Indurated hyperpigmented plaque with reddish-yellow nodules on the right thigh
Mentions: The examination revealed an indurated hyper-pigmented plaque with reddish-yellownodules, on her right thigh, measuring 15 cm in diameter (Figure 1). An erythematous nodule was presented on her right buttock (Figure 2). The patient reported that these lesionswere painless. A painless inguinal lymph node on the same side of the skin lesions wasalso revealed upon examination.

Bottom Line: In its typical form the disease is characterized by extensive cervical lymphadenopathy associated with fever, polyclonal gammopathy and leukocytosis with neutrophilia.The skin is the most common site affected.Extranodal manifestations have been reported in 43% of cases.

View Article: PubMed Central - PubMed

Affiliation: Dermatology Department, Dona Libânia Dermatology Center, Fortaleza, CE, Brazil. lealpauladl@gmail.com

ABSTRACT
Rosai-Dorfman disease is a benign, self-limited, idiopathic proliferative histiocytic disorder. It was first described in 1969 by Rosai and Dorfman. In its typical form the disease is characterized by extensive cervical lymphadenopathy associated with fever, polyclonal gammopathy and leukocytosis with neutrophilia. The skin is the most common site affected. Extranodal manifestations have been reported in 43% of cases. In this study, we report an atypical case of Rosai-Dorfman disease in a female with massive cutaneous manifestation on the thigh, associated with a minimal lymphadenopathy limited to the regional inguinal lymph nodes.

Show MeSH
Related in: MedlinePlus