| • | summary: collagenomas with osteopoikilosis; autosomal dominant |
| • | pathogenesis: increased elastic fiber content in the skin; unknown defect linking bone and skin |
clinical:
Dermatofibrosis lenticularis disseminata:
| • | lenticularis = bean-like |
| • | skin colored to yellow dermal papules often with symmetric distribution on trunk, buttocks, and arms |
osteopoikilosis:
| • | = asymptomatic X-ray finding |
| • | 1-10mm well-circumscribed, round opacities within carpal, tarsal bones, and phalanges of the hands and feet |
| • | ~ local areas of bony sclerosis, not prone to fracture |
ddx: tuberous sclerosis, PXE, familial cutaneous collagenomas
lab data: X-ray hands and feet in a patient with an elastoma
histology: Verhoeff-van Gieson or Orcein stain
prognosis: benign
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