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Purely cutaneous Rosai-Dorfman disease
M Skiljo1, E García-Lora, J Tercedor
1Department of Dermatology, Hospital Virgen de las Nieves, Granada, Spain.
Dermatology (Basel, Switzerland)
|January 1, 1995
Summary
Sinus histiocytosis with massive lymphadenopathy, a rare cutaneous form, can spontaneously resolve. Histology reveals characteristic histiocytes with lymphophagocytosis, neutrophils, and plasma cells, positive for S-100 and MAC 387.
Area of Science:
- Dermatology
- Pathology
- Oncology
Background:
- Sinus histiocytosis with massive lymphadenopathy (SHML), also known as Rosai-Dorfman disease, is a rare histiocytic disorder typically affecting lymph nodes.
- Purely cutaneous presentations of SHML are exceptionally uncommon, posing diagnostic challenges.
Observation:
- This report details two cases of purely cutaneous SHML exhibiting spontaneous involution.
- Clinical observation highlighted the self-resolving nature of these cutaneous lesions.
Findings:
- Histopathological examination revealed a polymorphic infiltrate in the dermis and hypodermis.
- Key findings included large histiocytes with lymphophagocytosis, neutrophils, and plasma cells.
- Immunohistochemical analysis confirmed histiocyte positivity for S-100 protein and MAC 387, consistent with SHML.
Implications:
- These cases expand the understanding of SHML's clinical spectrum, emphasizing its potential for spontaneous resolution in cutaneous forms.
- The findings underscore the importance of considering SHML in the differential diagnosis of cutaneous infiltrative disorders.
- Recognition of these histological and immunohistochemical markers is crucial for accurate diagnosis and management.