- Disease may be limited to the skin, or it may be a manifestation of systemic disease.
- Presents with indurated erythematous or violaceous patches or plaques
- Commonly affects the trunk and thighs
- Large atypical lymphocytes, often with prominent nucleoli, filling distended blood vessels within the dermis and subcutis
- The neoplastic lymphocytes are positive for pan-B-cell markers.
- CD5 is sometimes positive.
- A rare T-cell variant has also been described.