DermatopathologyPseudoxanthoma elasticum–like papillary dermal elastolysis: A large case series with clinicopathological correlation
Section snippets
Methods
A series of 17 patients with clinically and pathologically confirmed diagnosis of PXE-like PDE was collected prospectively. This included two cases from the original description1 and 15 new cases. Sixteen of the cases were seen at the Section of Dermatology, University of Genoa, Italy, between 1992 and 2010, and one case was seen at the Department of Dermatology, University of Miami, FL, in 2010. All cases were evaluated for common demographic and clinical features such as age, sex, chronic sun
Demographic and clinical findings
The most common demographic and clinical findings are summarized in Table I.
All patients were female (100%) with mean age of 61.8 years (51-79 years, SD 7.04). The clinical presentation included cobblestone plaques of confluent, dome-shaped, nonfollicular, soft, yellow 2- to 3-mm papules located on the lateral sides and back of the neck in all 17 patients (100%) (Fig 1, Fig 2). The plaques were seen involving also the supraclavicular region (n = 7, 41.2%), axilla (Fig 3) (n = 6, 35.3%),
Discussion
Our article adds to the literature a series of 17 cases of PXE-like PDE. The first two cases were described in the original report by Rongioletti and Rebora in 1992.1 Since then, less than 20 new cases have been reported, only on anecdotal basis.3, 4, 5, 6, 7 As confirmed by our series, PXE-like PDE affects only women, after the fifth decade, although one case in early middle age has been reported.8 Some authors explain the exclusive female predominance with the likelihood of women to seek
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Pseudoxanthoma elasticum–like papillary dermal elastolysis: A mimicker of genetic pseudoxanthoma elasticum
2021, Clinics in DermatologyCitation Excerpt :Exposure to ultraviolet radiation, intrinsic aging, or abnormal elastogenesis has been suggested as the main related factor. The role of ultraviolet radiation is controversial and cannot be considered the sole contributor; in fact, although most of the lesions are located on photodistributed sites (neck), the involvement of sun-protected areas (such as the axillae) is not uncommon and many patients lack a history of prolonged sun exposure.16 In addition, there is often a lack of actinic damage in the affected areas, with only a recent report documenting solar elastosis in the subjacent subpapillary dermis.13
Pseudoxanthoma elasticum-like papillary dermal elastolysis: A case report
2020, Annales de Dermatologie et de VenereologiePseudoxanthoma elasticum-like papillary dermal elastolysis in non-exposed skin
2020, Anais Brasileiros de DermatologiaBilateral axillary plaques
2023, Journal of Cutaneous PathologyPapillary dermal elastolysis histopathology mimicking folliculotropic mycosis fungoides
2023, Journal of Cutaneous Pathology
Funding sources: None.
Conflicts of interest: None declared.