Sean Klepper M.D.
Artur Zembowicz M.D....
- Most common in young adults
- Present as multiple small, slow-growing, tender papules on the extremities or trunk
- Less commonly, may present as a larger solitary lesion, especially in males
- Some cases are falililal, with autosomal dominant inheritance.
- Associations include, HIV, CLL, erythrocytosis, uterine leiomyomas and papillary or collecting duct variants of renal cell carcinoma.
- Poorly defined dermal mass which merges imperceptibly with the surrounding dermal connective tissue
- Composed of interlacing fascicles of spindle cells with brightly eosinophilic cytoplasm and cigar-shaped nuclei
- Postitive for SMA, desmin, h-caldesmon and calponin