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2016 ; 91
(5
): 634-637
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Histological variability and the importance of clinicopathological correlation in
cutaneous Rosai-Dorfman disease
#MMPMID27828638
Gameiro A
; Gouveia M
; Cardoso JC
; Tellechea O
An Bras Dermatol
2016[Sep]; 91
(5
): 634-637
PMID27828638
show ga
Rosai-Dorfman disease is a benign histiocytic proliferative disorder of unknown
etiology. The disease mainly affects lymph node tissue, although it is rarely
confined to the skin. Here, we describe a 53-year-old woman with purely cutaneous
Rosai-Dorfman disease. The patient presented with a large pigmented plaque on her
left leg, and sparse erythematous papules on her face and arms. A complete
clinical response was achieved with thalidomide, followed by recurrence at the
initial site one year later. The histological examination displayed the typical
features of Rosai-Dorfman disease in the recent lesions but not in the older
lesions. In the setting of no lymphadenopathy, the histopathological features of
Rosai-Dorfman disease are commonly misinterpreted. Therefore, awareness of the
histological aspects present at different stages, not always featuring the
hallmark microscopic signs of Rosai-Dorfman disease, is particularly important
for a correct diagnosis of this rare disorder.