Table 1.
Histopathological clues of PXE and related disorders.
| Disease | Histopathological clue(s) |
|---|---|
| Pseudoxanthoma elasticum | LM: Mid-dermal calcification and fragmentation of elastic fibers |
| EM: Mineralization in elastic fiber core | |
| PXE-like disease with coagulation deficiency | LM: Middermal calcification and fragmentation of elastic fibers |
| EM: Mineralization in elastic fiber periphery | |
| Haemoglobinopathies | LM: Middermal calcification and fragmentation of elastic fibers |
| EM: Mineralization in elastic fiber core | |
| PXE-like papillary dermal elastolysis | LM: Selective elastic tissue elimination in the papillary dermis and presence of melanophages |
| White fibrous papulosis of the neck | LM: Dermal fibrosis in papillary and mid-reticular dermis |
| EM: Decrease of elastic fibers; fragmentation of remaining fibers | |
| Late-onset focal dermal elastosis | LM: Accumulation of elastic fibers in mid- and reticular dermis without fragmentation or calcification |
| Perforating calcific elastosis | LM: Middermal calcification and degeneration of elastic fibers with transepidermal elimination |
| Buschke-Ollendorff syndrome | LM: Increased amount of hypertrophic elastic fibers in dermis |
| EM: Altered translucent elastic fibers | |
| Elastosis perforans serpiginosa | LM: Transepidermal or perifollicular perforating canals |
| Papular elastorrhexis | LM: Thickening of collagen bundles next to loss and fragmentation of elastic fibers |
| Upper dermal elastolysis | LM: Complete loss of elastic fibers in the upper dermis |
| Middermal elastolysis | LM: Complete absence of elastic fibers in the middermis |
| Linear focal elastosis | LM: Massive basophilic fibers; clumping of elastic fibers in papillary dermis |
| Elastoderma | LM: Increased, intertwining thin elastic fibers in papillary and upper reticular dermis |
| Calcinosis cutis | LM: Deposits of calcium in the dermis |