Pseudoxanthoma elasticum with transepidermal elimination

Schwartz, R. A.

doi:10.1001/archderm.114.2.279

Cited by 17 publications

(9 citation statements)

References 0 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…As PXE, PCE is characterized histologically be degenerated elastic fibers that become infiltrated with calcium and lie predominantly in the middermis [79]. Hematoxylin-eosin-stained sections shows altered elastic fibers throughout the dermis that are short, thick, irregularly clumped, and basophilic (Figure 10).…”

Section: Dermatological Diseases With (Near-) Identical Skin Lesionsmentioning

confidence: 99%

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

2012

View full text Add to dashboard Cite

Among ectopic mineralization disorders, pseudoxanthoma elasticum (PXE)—a rare genodermatosis associated with ocular and cardiovascular manifestations—is considered a paradigm disease. The symptoms of PXE are the result of mineralization and fragmentation of elastic fibers, the exact pathophysiology of which is incompletely understood. Though molecular analysis of the causal gene, ABCC6, has a high mutation uptake, a skin biopsy has until now been considered the golden standard to confirm the clinical diagnosis. Although the histological hallmarks of PXE are rather specific, several other diseases—particularly those affecting the skin—can present with clinical and/or histological characteristics identical to or highly resemblant of PXE. In this paper, we will summarize the histopathological features of PXE together with those of disorders that are most frequently considered in the differential diagnosis of PXE.

show abstract

Section: Dermatological Diseases With (Near-) Identical Skin Lesionsmentioning

confidence: 99%

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

2012

View full text Add to dashboard Cite

show abstract

“…85,97,98 The initial eruption may contain asymptomatic or mildly pruritic, erythematous papules, plaques, or nodules. Over the course of months to several years, the lesions tend to resolve, leaving a flat, well-demarcated, hyperpigmented, reticulated, atrophic central plaque with a raised, scaly border.…”

Section: Elastosis Perforans Serpiginosamentioning

confidence: 99%

Acquired disorders of elastic tissue: part I. increased elastic tissue and solar elastotic syndromes

Lewis

Bercovitch

Dill

et al. 2004

Journal of the American Academy of Dermatology

171

178

View full text Add to dashboard Cite

“…Perforating pseudoxanthoma elasticum differs from PXE, which is a multisystem disorder involving the elastic tissue of the dermis, the blood vessels, and Bruch’s membrane of the eyes. However, some opinions consider PPXE as a localized cutaneous form of hereditary PXE 2 …”

Section: Discussionmentioning

confidence: 99%

Periumbilical perforating pseudoxanthoma elasticum ‐ an acquired perforating disorder

Budania¹,

De²,

Saikia³

et al. 2012

Int J Dermatology

View full text Add to dashboard Cite

Pseudoxanthoma elasticum with transepidermal elimination

Cited by 17 publications

References 0 publications

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

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

Acquired disorders of elastic tissue: part I. increased elastic tissue and solar elastotic syndromes

Periumbilical perforating pseudoxanthoma elasticum ‐ an acquired perforating disorder

Contact Info

Product

Resources

About