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Pseudoxanthoma elasticum-like papillary dermal elastolysis* * Study conducted at Hospital do Servidor Público Estadual de São Paulo - São Paulo -SP, Brazil.

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Elastic fibers are important components of the extracellular matrix of the connective tissue. The elastic system comprises oxytalan and eulanin fibers. Various acquired and hereditary conditions are associated to changes of these fibers, among which we highlight pseudoxanthoma elasticum-like papillary dermal elastolysis (PXE-PDE).

PXE-PDE is an acquired condition, that is manifested by multiple asymptomatic, sometimes itchy, non-follicular yellow or normochromic papules, with a diameter of 2-3mm, symmetrically distributed, that can coalesce into "cobblestone" plaques with predilection for the neck area, but also supraclavicular, axillary, flexor aspect of forearms, inframammary and lower abdomen regions. The lesions develop slowly, in months to years, and are clinically similar to pseudoxanthoma elasticum. However, they are differentiated by histopathology and for the lack of systemic involvement.11 Rongioletti F, Rebora A. Pseudoxanthoma elasticum-like papillary dermal elastolysis. J Am Acad Dermatol. 1992;26:648-50.

Histopathology features include loss or marked reduction in the papillary dermis elastic fibers.

Prevalence is believed to be underestimated for this rare condition, what reinforces the importance of better clinical and histological identification by dermatologists and pathologists, to avoid mistaking it for pseudoxanthoma elasticum.22 Rongioletti F, Izakovic J, Romanelli P, Lanuti E, Miteva M. Pseudoxanthoma elasticum-like papillary dermal elastolysis: A large case series with clinicopathological correlation. J Am Acad Dermatol. 2012;67:128-35.

The patient was a 71-year-old woman who presented with multiple asymptomatic non-follicular normochromic papules, symmetrically distributed on the cervical, supraclavicular and antecubital regions, that were progressively enlarging over a 10-year period (Figure 1).

Figure 1
Normochromic non-follicular papules in the cervical region

On dermoscopy, there were multiple normochromic papules and linear vessels (Figure 2).

Figure 2
Linear vessels on dermoscopy

Considering the diagnosis of pseudoxanthoma elasticum, the patient was referred for ophthalmologic evaluation, when no typical angioid streaks were seen. A biopsy was taken from the cervical region, and histopathology with Verhoeff stain revealed loss of elastic fibers in the papillary dermis (Figure 3).

Figure 3
Loss of elastic fibers in the papillary dermis (Verhoeff, X200)

Described in 1992 by Rongioletti and Rebora, PXE-PDE is an acquired, rare disease that affects exclusively elderly women, usually after the fifth decade of life.11 Rongioletti F, Rebora A. Pseudoxanthoma elasticum-like papillary dermal elastolysis. J Am Acad Dermatol. 1992;26:648-50.

Etiology is unknown, but is believed to be due to aging, exposure to ultraviolet radiation and abnormal elastogenesis.22 Rongioletti F, Izakovic J, Romanelli P, Lanuti E, Miteva M. Pseudoxanthoma elasticum-like papillary dermal elastolysis: A large case series with clinicopathological correlation. J Am Acad Dermatol. 2012;67:128-35.,33 Oiso N, Kato M, Kawada A. Fibroelastolytic papulosis in an elderly woman with a 30-year history: overlapping between pseudoxanthoma elasticum-like papillary dermal elastolysis and white fibrous papulosis of the neck. Eur J Dermatol. 2014;24:688-9. There is a reported case of familial occurrence of the condition, suggesting a genetic component in the etiopathogenesis.

The diagnosis is confirmed by histopathology with special stains specific for elastic fibers, such as orcein, Verhoeff-Van Gieson or Weigert. The most common change is loss or marked reduction of eulanin and oxytalan elastic fibers in the papillary dermis, and the presence of melanophages. Epidermis can be normal or slightly thinned, with few cases of unspecific perivascular lymphocytic infiltrate in the papillary dermis. No elastic fiber calcification was seen with von Kossa stain, as in pseudoxanthoma elasticum.22 Rongioletti F, Izakovic J, Romanelli P, Lanuti E, Miteva M. Pseudoxanthoma elasticum-like papillary dermal elastolysis: A large case series with clinicopathological correlation. J Am Acad Dermatol. 2012;67:128-35. With immunochemistry, loss of fibrillin 1 and 2 and microfibril-associated glycoprotein 1 and 24 can be seen. Monoclonal anti-amyloid P component antibody can also be used to demonstrate loss of elastic fibers in the papillary dermis. There are no relevant findings on H&E.

On dermoscopy, besides non-follicular papules, linear and arborizing vessels were described, corresponding to telangiectases seen in the papillary dermis on histology. 44 Ito T, Fujita Y, Nomura T, Abe R, Shimizu H. Dermoscopy of pseudoxanthoma elasticum-like papillary dermal elastolysis. J Am Acad Dermatol. 2013;69:e202-3.,55 Vázquez-Osorio I, Rosón E, Suárez-Peñaranda JM, Vázquez-Veiga H. Elastólisis de la dermis papilar similar a pseudoxantoma elástico. Actas Dermo-Sifiliográficas. 2015;106:333-6.

The differential diagnosis should be made with pseudoxanthoma elasticum, white fibrous papulosis of the neck and mid-dermal elastolysis.22 Rongioletti F, Izakovic J, Romanelli P, Lanuti E, Miteva M. Pseudoxanthoma elasticum-like papillary dermal elastolysis: A large case series with clinicopathological correlation. J Am Acad Dermatol. 2012;67:128-35.

Pseudoxanthoma elasticum is an inherited disorder characterized by calcification and fragmentation of elastic fibers that affects the skin, retina (with the appearance of angioid streaks) and cardiovascular system. Unlike pseudoxanthoma elasticum, PXE-PDE has no systemic manifestations.

The coexistence of PXE-PDE with white fibrous papulosis of the neck is known as fibroelastolytic papulosis.33 Oiso N, Kato M, Kawada A. Fibroelastolytic papulosis in an elderly woman with a 30-year history: overlapping between pseudoxanthoma elasticum-like papillary dermal elastolysis and white fibrous papulosis of the neck. Eur J Dermatol. 2014;24:688-9. There are no relevant data regarding treatment.

Based on the presented case and the reviewed literature, we highlight the relevance of being familiar with PXE-PDE and of differentiating it from pseudoxanthoma elasticum, to avoid extensive, unnecessary systemic investigations. It is also of importance to look for effective treatments.

  • *
    Study conducted at Hospital do Servidor Público Estadual de São Paulo - São Paulo -SP, Brazil.
  • Financial support: None.

REFERENCES

  • 1
    Rongioletti F, Rebora A. Pseudoxanthoma elasticum-like papillary dermal elastolysis. J Am Acad Dermatol. 1992;26:648-50.
  • 2
    Rongioletti F, Izakovic J, Romanelli P, Lanuti E, Miteva M. Pseudoxanthoma elasticum-like papillary dermal elastolysis: A large case series with clinicopathological correlation. J Am Acad Dermatol. 2012;67:128-35.
  • 3
    Oiso N, Kato M, Kawada A. Fibroelastolytic papulosis in an elderly woman with a 30-year history: overlapping between pseudoxanthoma elasticum-like papillary dermal elastolysis and white fibrous papulosis of the neck. Eur J Dermatol. 2014;24:688-9.
  • 4
    Ito T, Fujita Y, Nomura T, Abe R, Shimizu H. Dermoscopy of pseudoxanthoma elasticum-like papillary dermal elastolysis. J Am Acad Dermatol. 2013;69:e202-3.
  • 5
    Vázquez-Osorio I, Rosón E, Suárez-Peñaranda JM, Vázquez-Veiga H. Elastólisis de la dermis papilar similar a pseudoxantoma elástico. Actas Dermo-Sifiliográficas. 2015;106:333-6.

Publication Dates

  • Publication in this collection
    Nov-Dec 2017

History

  • Received
    20 Dec 2016
  • Accepted
    13 June 2017
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