Abstract
Pseudoxanthoma elasticum (PXE), also known as Gröenblad-Strandberg syndrome, is an autosomal recessive connective tissue disease characterized by aberrant mineralization of soft connective tissue resulting in fragmentation of elastic fibers, involving primarily the skin, eyes and cardiovascular. To our knowledge, there is only two other reports of the dermoscopic features of PXE in the literature. We report clinical and dermoscopic description of two new cases. We hypothesize for the first time that the dermoscopic appearance may differ according to the age of PXE lesions.
Highlights
Pseudoxanthoma elasticum (PXE) is an inherited disease characterized by aberrant mineralization of soft tissue and fragmentation of elastic fibres [1]
Cutaneous findings present mainly a cosmetic issue, but they allow the identification of the disease and the possibility of monitoring the risks of ocular and cardiovascular complications, which are associated with considerable morbidity and mortality [4]
Histopathological examination of a skin biopsy stained with haematoxylin and eosin (H&E) showed preserved morphology of the epidermal, the superficial, medium and deep dermis is encumbered with elastic clumped and irregularly packed fibers
Summary
Pseudoxanthoma elasticum (PXE) is an inherited disease characterized by aberrant mineralization of soft tissue and fragmentation of elastic fibres [1]. Histopathological examination of a skin biopsy stained with haematoxylin and eosin (H&E) showed preserved morphology of the epidermal, the superficial, medium and deep dermis is encumbered with elastic clumped and irregularly packed fibers. These fibers colored in black by Orcein are intimately mixed with collagen fibers without any abnormality Based on these findings, we diagnosed the patient with PXE. Patient 2 was a 21-year-old young woman, who presented with asymptomatic skin lesions of her neck These lesions had appeared at 15 and had since, slowly enlarged. © Our Dermatol Online e.2021 the first patient, we diagnosed the patient with PXE
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