Dermatopathology
Pseudoxanthoma elasticum–like papillary dermal elastolysis: A large case series with clinicopathological correlation

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Background

Pseudoxanthoma elasticum (PXE)-like papillary dermal elastolysis (PDE) is a rare acquired elastic tissue disorder. To date, less than 20 cases have been reported.

Objective

We report a case series of 17 patients presenting with PXE-like PDE and discuss the clinicopathological correlation.

Methods

Seventeen cases of PXE-like PDE were collected prospectively and evaluated for common demographic, clinical, and histopathological features.

Results

All patients were women with a mean age of 61.8 years. The lateral sides and back of neck were the most common sites of involvement (100%), followed by the supraclavicular region (41.2%) and the axilla (35.3%). Systemic involvement was absent in all cases, and in 7 patients the discovery of PXE-like PDE was an incidental finding. The main histopathologic features included complete loss (82.4%) or marked reduction (17.6%) of elastic fibers in the papillary dermis and the presence of melanophages in the same zone (88.2%).

Limitations

Our results require validation with a larger series.

Conclusions

Our findings help to differentiate PXE-like PDE from similar elastic tissue disorders based on the selective elastic tissue elimination in the papillary dermis and the presence of melanophages in the same zone as a possible consequence of subclinical junctional photodamage. PXE-like PDE is likely underdiagnosed rather than rare, and dermatologists should be aware of its similarity to inherited PXE to spare unnecessary investigations because of the lack of systemic involvement. Clinicopathologic correlation is critical as hematoxylin-eosin staining is nonspecific and elastic tissue stains are necessary to make the correct diagnosis.

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

References (23)

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    Funding sources: None.

    Conflicts of interest: None declared.

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