[Pseudoxanthoma elasticum-like papillary dermal elastolysis: A case report]

Abstract

Background: Pseudoxanthoma elasticum-like papillary dermal elastolysis (PXE-PDE) is a rare disease clinically resembling pseudoxanthoma elasticum (PXE). Herein we report a typical case.

Patients and methods: A 77-year-old woman consulted for an acquired papular eruption present for 4 years. Her history included breast cancer, which was considered to be in remission. The eruption had begun on the right armpit before extending to the right side of the chest, left armpit, neck and right inguinal fold. It was completely asymptomatic. It consisted of non-follicular flabby, skin-colored papules, without anetoderma. Histological examination with hematoxylin-eosin and orcein staining revealed papillary and mid-dermal elastolysis without elastorrhexis. Based on the clinical aspect of PXE as well as histologically demonstrated elastolysis, a diagnosis of PXE-PDE was made.

Discussion: PXE-PDE is a rare acquired entity that affects only women, usually after the age of 60 years. Although it is clinically similar to PXE, PXE-PDE may be differentiated through its late onset, the absence of systemic symptoms, and the attendant histological features. Dermoscopy may also contribute to differential diagnosis. Histological examination allows confirmation of the diagnosis and shows normal elastic fibers that may be either missing or present in vastly reduced quantities in the papillary and mid-dermis. The physiopathology continues to be unclear, but may involve skin aging, elastogenesis abnormalities and UV exposure. To date, no treatment has demonstrated its efficiency.

Conclusion: PXE-PDE is a rare condition, but it displays typical histological and clinical features. Knowledge of this entity avoids unnecessary explorations and enables rapid reassurance of patients.

Keywords: Derme papillaire; Elastolysis; Elastorrhexis; Papillary dermis; Pseudoxanthoma elasticum; Pseudoxanthome élastique; Élastolyse; Élastorrhexie.