Abstract
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.
| Original language | English |
|---|---|
| Pages (from-to) | 128-135 |
| Number of pages | 8 |
| Journal | Journal of the American Academy of Dermatology |
| Volume | 67 |
| Issue number | 1 |
| DOIs | |
| Publication status | Published - Jul 2012 |
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Keywords
- elastic tissue disorders
- pseudoxanthoma elasticum-like papillary dermal elastolysis
ASJC Scopus subject areas
- Dermatology
Cite this
Pseudoxanthoma elasticum-like papillary dermal elastolysis : A large case series with clinicopathological correlation. / Rongioletti, Franco; Izakovic, Jan; Romanelli, Paolo; Lanuti, Emma; Miteva, Mariya.
In: Journal of the American Academy of Dermatology, Vol. 67, No. 1, 07.2012, p. 128-135.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Pseudoxanthoma elasticum-like papillary dermal elastolysis
T2 - A large case series with clinicopathological correlation
AU - Rongioletti, Franco
AU - Izakovic, Jan
AU - Romanelli, Paolo
AU - Lanuti, Emma
AU - Miteva, Mariya
PY - 2012/7
Y1 - 2012/7
N2 - 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.
AB - 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.
KW - elastic tissue disorders
KW - pseudoxanthoma elasticum-like papillary dermal elastolysis
UR - http://www.scopus.com/inward/record.url?scp=84862279192&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84862279192&partnerID=8YFLogxK
U2 - 10.1016/j.jaad.2011.09.008
DO - 10.1016/j.jaad.2011.09.008
M3 - Article
C2 - 22018757
AN - SCOPUS:84862279192
VL - 67
SP - 128
EP - 135
JO - Journal of the American Academy of Dermatology
JF - Journal of the American Academy of Dermatology
SN - 0190-9622
IS - 1
ER -