J Am Acad Derm 1994;30:103; Nejm 1993;329:1237

Cause:Genetic; autosomal recessive, 2 types; less common autosomal dominant, 2 types

Pathophys:Premature degeneration and calcification of elastic fibers. Angioid streaks are tears in Bruch's membrane, which look like brown jagged streaks radiating from the optic disc (picture—Nejm 1997;337:829)

Rare. Women men

Sx:Onset of sx between age 20-50 yr usually, though may be present at birth

Si:

60% have all 3

• Skin changes: yellow papules/plaques on flexor skin; small soft papules parallel to skin folds, looks like leather, may coalesce on exposed and unexposed areas unlike senile elastosis; r/o multiple myeloma and biliary cirrhosis
• Eye changes: angioid streaking (r/o Paget's, sickle cell disease), may lead to some visual loss. Chorioretinitis and hemorrhages may look like dark stippling of fundus
• CV changes: premature atherosclerosis especially of medium vessels leads to MIs. Hypertension in adolescence due to renal artery stenosis

GI bleeding (10%), especially common in pregnancy (Grönblad-Strandberg syndrome)

Lab: Path:Skin bx shows diagnostic granular accumulations of basophilic material with calcium in middle and lower dermis, stains like elastic tissue but is periodic like collagen, especially in scars (Nejm 1987;317:347)

Xray:Calcified falx cerebri