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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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Skin biopsy. Difuse dermal infiltrates of histiocytes, lymphocytes, neutrophilsand plasma cells. (H&E stain, X100)
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f03: Skin biopsy. Difuse dermal infiltrates of histiocytes, lymphocytes, neutrophilsand plasma cells. (H&E stain, X100)

Mentions: The histopathology of the skin lesion exhibited a slightly acanthotic epidermis, dermisand hypodermis with a diffuse infiltrate, consisting of lymphocytes, plasma cells andneutrophils (Figure 3). Occasional histiocytescontaining intact inflammatory cells within the cytoplasm (emperipolesis) were also seen(Figure 4). Immunohistochemical studiesrevealed histiocytes with positivity for S100 and CD68 and negativity for CD1a. Thehistopathology of the inguinal lymph node showed dilated nodal sinuses with largenumbers of histiocytes exhibiting emperipolesis (Figure5).


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)

Skin biopsy. Difuse dermal infiltrates of histiocytes, lymphocytes, neutrophilsand plasma cells. (H&E stain, X100)
© Copyright Policy - open-access
Related In: Results  -  Collection

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

f03: Skin biopsy. Difuse dermal infiltrates of histiocytes, lymphocytes, neutrophilsand plasma cells. (H&E stain, X100)
Mentions: The histopathology of the skin lesion exhibited a slightly acanthotic epidermis, dermisand hypodermis with a diffuse infiltrate, consisting of lymphocytes, plasma cells andneutrophils (Figure 3). Occasional histiocytescontaining intact inflammatory cells within the cytoplasm (emperipolesis) were also seen(Figure 4). Immunohistochemical studiesrevealed histiocytes with positivity for S100 and CD68 and negativity for CD1a. Thehistopathology of the inguinal lymph node showed dilated nodal sinuses with largenumbers of histiocytes exhibiting emperipolesis (Figure5).

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