Cutaneous Rosai-Dorfman disease: histopathological presentation as inflammatory pseudotumor. A literature review.

Abstract

Purely cutaneous Rosai-Dorfman disease is exceptional. The disease is characterized histologically by large, proliferating histiocytes exhibiting inflammatory cells within their cytoplasm (emperipolesis). We present here a case of purely cutaneous generalized disease in which the routine histopathology was suggestive of an inflammatory pseudotumor. Positivity for S-100 protein, alpha1-antitrypsin, alpha1-antichymotrypsin, lysozyme, Mac387 and CD68 proteins, and negativity for CD1a protein confirmed the diagnosis of Rosai-Dorfman disease. The rarity of this case lies in the presence of conspicuous inflammatory pseudotumor-like histopathologic changes, masking an otherwise typical sinus histiocytosis cell infiltrate. This unusual presentation of the disease requires a high index of suspicion by clinicians and pathologists.

• Authors: George Kroumpouzos, Marie-France Demierre
• Journal: Acta dermato-venereologica (Acta Derm Venereol) Vol. 82 Issue 4 Pg. 292-6 ( 2002) ISSN: 0001-5555 [Print] Sweden
• PMID: 12361136 (Publication Type: Case Reports, Journal Article, Review)

Chemical References

• Antigens, CD
• Antigens, CD1
• Antigens, Differentiation, Myelomonocytic
• CD1a antigen
• CD68 antigen, human
• S100 Proteins
• alpha 1-Antichymotrypsin
• alpha 1-Antitrypsin
• Muramidase

Topics

• Adult
• Antigens, CD (analysis)
• Antigens, CD1 (analysis)
• Antigens, Differentiation, Myelomonocytic (analysis)
• Diagnosis, Differential
• Female
• Granuloma, Plasma Cell (pathology)
• Histiocytosis, Sinus (pathology)
• Humans
• Immunohistochemistry
• Muramidase (analysis)
• S100 Proteins (analysis)
• Skin Diseases (pathology)
• alpha 1-Antichymotrypsin (analysis)
• alpha 1-Antitrypsin (analysis)