Review Article

Histopathology of Pseudoxanthoma Elasticum and Related Disorders: Histological Hallmarks and Diagnostic Clues

Table 1

Histopathological clues of PXE and related disorders.

DiseaseHistopathological clue(s)

Pseudoxanthoma elasticumLM: Mid-dermal calcification and fragmentation of elastic fibers
EM: Mineralization in elastic fiber core
PXE-like disease with coagulation deficiencyLM: Middermal calcification and fragmentation of elastic fibers
EM: Mineralization in elastic fiber periphery
HaemoglobinopathiesLM: Middermal calcification and fragmentation of elastic fibers
EM: Mineralization in elastic fiber core
PXE-like papillary dermal elastolysisLM: Selective elastic tissue elimination in the papillary dermis and presence of melanophages
White fibrous papulosis of the neckLM: Dermal fibrosis in papillary and mid-reticular dermis
EM: Decrease of elastic fibers; fragmentation of remaining fibers
Late-onset focal dermal elastosisLM: Accumulation of elastic fibers in mid- and reticular dermis without fragmentation or calcification
Perforating calcific elastosisLM: Middermal calcification and degeneration of elastic fibers with transepidermal elimination
Buschke-Ollendorff syndromeLM: Increased amount of hypertrophic elastic fibers in dermis
EM: Altered translucent elastic fibers
Elastosis perforans serpiginosaLM: Transepidermal or perifollicular perforating canals
Papular elastorrhexisLM: Thickening of collagen bundles next to loss and fragmentation of elastic fibers
Upper dermal elastolysisLM: Complete loss of elastic fibers in the upper dermis
Middermal elastolysisLM: Complete absence of elastic fibers in the middermis
Linear focal elastosisLM: Massive basophilic fibers; clumping of elastic fibers in papillary dermis
ElastodermaLM: Increased, intertwining thin elastic fibers in papillary and upper reticular dermis
Calcinosis cutisLM: Deposits of calcium in the dermis