Pseudoxanthoma elasticum-like papillary dermal elastolysis in non-exposed skin^☆, ^☆☆

Abstract

Pseudoxanthoma elasticum-like papillary dermal elastolysis is an acquired elastic tissue disorder clinically similar to pseudoxanthoma elasticum in the absence of systemic involvement. Histopathologically, special staining of elastic fibers demonstrates a total or partial band-like loss of elastic fibers in the papillary dermis. Although ultraviolet radiation seems to be one of the main etiological factors in this entity, we report a case of pseudoxanthoma elasticum-like papillary dermal elastolysis on the neck of a woman who wore hijab.

KEYWORDS
Dermis; Elastic tissue; Female; Pseudoxanthoma elasticum

A 54-year-old female of Moroccan origin who habitually wears a hijab presented a 2 year history of mildly pruritic lesions on the neck. She denied systemic symptoms and family history of similar findings. Her medical history included mixed anxiety-depressive disorder treated with olanzapine and sertraline. Physical examination revealed white-to-yellowish millimetric non-follicular papules on the lateral aspects of the neck and supraclavicular fossae (Fig. 1). Dermoscopic examination showed multiple white-colored non-follicular papules, coalescing into plaques with arboriform vessels (Fig. 2). The biopsy showed slight sclerosis of the papillary dermis with neovascularization and a mild inflammatory infiltrate including lymphocytes and some melanophages (Fig. 3). In the same area, van Gieson stain demonstrated a decrease in the number of elastic fibers that were often thin and fragmented (Fig. 4) compatible with pseudoxanthoma elasticum-like papillary dermal elastolysis (PXE-PDE). Cardiac and ophthalmological investigations performed were unremarkable.

Figure 1
Whitish papules on the neck and supraclavicular fossae.

Figure 2
Multiple whitish non-follicular papules, coalescing into plaques with linear vessels, on dermoscopy.

Figure 3
Slight sclerosis of the papillary dermis, neovascularization and a mild inflammatory infiltrate (Hematoxylin & eosin, x100).

Figure 4
Reduction of elastic fibers in the papillary dermis (van Gieson, x200).

PXE-PDE is a rare acquired elastic tissue disorder characterized by non-follicular yellowish papules coalescing into plaques with predilection for neck, supraclavicular fossae and flexural areas. ¹ The lesions are usually asymptomatic, but mild itch is sometimes reported, as seen in our case. To date, it affects exclusively women mostly in middle age ² and it is not associated with any systemic involvement. Dermoscopic findings consist of multiple white-colored non-follicular papules, coalescing into plaques with linear vessels. ³

Histopathologically, hematoxylin eosin staining does not reveal any specific changes. The focal inflammatory changes present in our case have not been described previously; however, it is presumed that elastic fiber loss could be the result of a transient phenomenon of inflammation. Special staining of elastic fibers with van Gieson or orcein stains are required to demonstrate a total or partial band-like loss of elastic fibers in the papillary dermis. ² Calcification or fragmentation of the elastic fibers is absent. Immunohistochemical studies using monoclonal antibodies against antibody P component can also demonstrate partial or complete loss of elastic fibers in papillary dermis. ¹ The presence of melanophages in the papillary dermis constitutes an additional helpful diagnostic feature. ⁴

The cause of PXE-PDE remains unclear, and some etiopathogenic theories have been proposed: ultraviolet radiation, intrinsic aging, abnormal elastogenesis, and genetic or inheritable factors.^1,2 In our case, ultraviolet radiation's etiopathogenic theory is unlikely because the patient wore hijab.

Differential diagnosis of PXE-PDE includes white fibrous papulosis of the neck, mid-dermal elastolysis, and papillary dermal elastosis. Nevertheless, the main differential diagnosis must be established with pseudoxanthoma elasticum (PXE), a hereditary disorder caused by mutation on ABCC6 gene. Clinically, PXE resembles PXE-PDE, but it appears at a younger age, and it is usually associated with ocular and cardiovascular complications. Histopathologically, PXE presents fragmentation and calcification of elastic fibers demonstrated with von Kossa stain.

Treatments for PXE-PDE, including topical retinoids, have shown poor results ²; however, non-ablative fractional resurfacing laser has demonstrated to be effective in some cases. ⁵

Herein we present a case of PXE-PDE in a patient who did not receive UV radiation because she wore hijab. In our opinion, more studies are needed in order to better understand the etiopathogenesis of PXE-PDE. It is important that dermatologists recognize this entity and differentiate it from PXE to avoid unnecessary investigation. Clinicopathologic correlation is important and elastic tissue stains are required to correctly diagnose PXE-PDE.

• ☆
  How to cite this article: Setó-Torrent N, Iglesias-Sancho M, Arandes-Marcocci J, Fernández-Figueras MT. Pseudoxanthoma elasticum-like papillary dermal elastolysis in non-exposed skin. An Bras Dermatol. 2020;95:247-9.
• ☆☆
  Study conducted at the Hospital Universitari Sagrat Cor-Grupo Quirón Salud, Barcelona, Spain.
• Financial support
  None declared.

References

• ¹ Revelles JM, Machan S, Pielasinski Ú, Camacho D, Vallés L, Santonja C, et al. Pseudoxanthoma elasticum-like papillary dermal elastolysis: immunohistochemical study using elastic fiber cross-reactivity with an antibody against amyloid P component. Am J Dermatopathol. 2012;34:637-43.
• ² Panagou E, Ratynska M, Heelan K. Pseudoxanthoma elasticum-like papillary dermal elastolysis: a case report and review of literature. Int J Dermatol. 2019;58:93-7.
• ³ Ribeiro CP, Abuawad YG, Swiczar BCC, Valente NYS. Pseudoxanthoma elasticum-like papillary dermal elastolysis. An Bras Dermatol. 2017;92:897-8.
• ⁴ 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.
• ⁵ Foering K, Torbeck RL, Frank MP, Saedi N. Treatment of pseudoxanthoma elasticum-like papillary dermal elastolysis with nonablative fractional resurfacing laser resulting in clinical and histologic improvement in elastin and collagen. J Cosmet Laser Ther. 2018;20:382-4.

Publication Dates

• Publication in this collection
  06 July 2020
• Date of issue
  May-Jun 2020

History

• Received
  14 Apr 2019
• Accepted
  11 Aug 2019
• Published
  12 Feb 2020