[Pseudoxanthoma elasticum-like papillary dermal elastolysis: A case report]. / Élastolyse du derme papillaire « pseudoxanthome élastique-like ¼ : un cas.

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.

[Mid-dermal elastolysis after insertion of a pacemaker]. / Élastolyse du derme moyen après pose d'un stimulateur cardiaque.

BACKGROUND: Mid-dermal elastolysis (MDE) is a rare acquired disease of elastic tissue histologically characterized by focal loss of elastic fibres within the mid-dermis. While the mechanisms leading to MDE remain unknown, increased degradation of elastic fibres may be involved. Many factors potentially triggering such degradation have been suggested. PATIENTS AND METHODS: A 58-year-old man consulted for an asymptomatic reticulated eruption that began in the area of a pacemaker implanted six weeks earlier. The eruption consisted of erythematous polycyclic and coalescing macules with a wrinkled centre. Histopathology with orcein staining revealed focal loss of elastic fibres in the superficial reticular dermis only. Hypersensitivity reaction to any components of the pacemaker was ruled out by means of allergy exploration. Laboratory investigations including autoimmunological and haematological factors were unremarkable. A diagnosis was made of a reticular variant of MDE following insertion of a pacemaker. DISCUSSION: We report the second case of MDE following the insertion of a pacemaker, which could have triggered an inflammatory response directed specifically towards the elastic fibres.