ABSTRACT
Pseudoxanthoma elasticum-like papillary dermal elastolysis (PXE-like PDE) is a rare acquired elastolytic disorder that clinically resembles pseudoxanthoma elasticum. The pathogenesis of this disease is uncertain, but it seems to be related to intrinsic aging, abnormal elastogenesis, a genetic predisposition and exposure to ultraviolet radiation. It generally develops on the sides of the neck and supraclavicular regions in elderly persons. Histologically, the characteristic features are the loss of elastic fibers in the papillary dermis and no solar elastosis. We report here on a case of PXE-like PDE in a 26-year-old female whose skin lesion developed 9 years previously. Despite the preferential occurrence at an old age, our patient was young.
Subject(s)
Adult , Aged , Female , Humans , Aging , Dermis , Elastic Tissue , Genetic Predisposition to Disease , Neck , Pseudoxanthoma Elasticum , SkinABSTRACT
Pseudoxanthoma elasticum-like papillary dermal elastolysis is a disorder of the dermis, characterized by clinical features similar to pseudoxanthoma elasticum and the pathologic features of elastolysis limited to the papillary dermis, such as sun-protected, intrinsically-aged skin. A 64-year old woman presented with a 7 year history of a localized skin lesion on the back of her neck. Physical examination revealed skin to yellow colored, non-follicular grouped uniform papules, sized 5~8 mm, and coalescent plaques localized below the hair line. Histopathologic and immunohistochemical findings of decrease and elastolysis of the elastic fibers in the papillary dermis confirmed the diagnosis. In this article, we report a case of pseudoxanthoma elasticum-like papillary dermal elastolysis which presented with typical clinical and pathologic features.
Subject(s)
Female , Humans , Middle Aged , Dermis , Diagnosis , Elastic Tissue , Hair , Neck , Physical Examination , Pseudoxanthoma Elasticum , SkinABSTRACT
Pseudoxanthoma elasticum (PXE)-like papillary dermal elastolysis (PDE) is an unusual acquired elastolytic disorder that clinically resembles PXE but shows partial or complete loss of the elastic network in the papillary dermis. PXE-like PDE is characterized by the multiple yellowish nonfollicular papules, small in diameter, on the antecubital fossae, flexor aspect of the forearms, supraclavicular region, sides of the neck, and lower abdominal area. The multiple papules coalesce to form plaques with a cobble stone appearance resembling "plucked chicken skin". No systemic involvement has been noted in this disorder. Histopathologically, Hematoxylin-eosin stain shows nearly normal finding, but elastic stain shows partial or complete loss of elastic fibers focally in the papillary dermis. We report a case of pseudoxanthoma elasticum-like papillary dermal elastolysis with typical clinical and pathological features.
Subject(s)
Chickens , Dermis , Elastic Tissue , Forearm , Neck , Pseudoxanthoma ElasticumABSTRACT
Fibroelastolytic papulosis of the neck(FEPN) was introduced by Balus et al in 1997. Before this term was used, white fibrous papulosis of the neck(WFPN) and pseudoxanthoma elasticumlike papillary dermal elastolysis(PDE) had differently been used. These two disease entities had clinical similarities such as asymptomatic, white to yellow discolored, papular eruptions on the neck in elderly persons. As names implicated, however, they showed variable histological findings, for examples, fibrosis in WFPN or elastolysis in PDE or both in FEPN. Taken into ac-count together, instead of using WFPN or PDE separately, FEPN might be preferred to describe these two kinds of skin diseases. We report a case of a 36-year-old male patient with papules compatible with FEPN, whose biopsy mainly showed elastolysis.