Pseudoxanthoma elasticum-like papillary dermal elastolysis: A mimicker of genetic pseudoxanthoma elasticum. 2021

Laura Atzori, and Caterina Ferreli, and Luca Pilloni, and Franco Rongioletti
Dermatology Clinic, Department of Medical Sciences and Public Health, University of Cagliari, Cagliari, Italy. Electronic address: atzoril@unica.it.

Pseudoxanthoma elasticum (PXE)-like papillary dermal elastolysis (PDE) is an acquired, rare, elastic tissue disorder that presents with multiple asymptomatic, nonfollicular yellowish or normochromic papules, coalescing into symmetrical cobblestone plaques, with a predilection for the neck of postmenopausal women. The condition develops slowly, in months to years, and, although clinically similar to PXE, it is devoid of any systemic implication, being only of esthetic concern. The etiology is unknown, but it is speculated that there is a multifactorial pathogenesis, with the contribution of intrinsic aging, exposure to ultraviolet radiation, abnormal elastogenesis, and genetic factors. The diagnosis is confirmed by histopathology, with special stains specific for elastic fibers revealing loss of elastic plexus in the papillary dermis and the presence of melanophages. Its prevalence is probably underestimated, which reinforces the importance of better clinical and histologic identification to distinguish it from inherited PXE and to avoid extensive systemic investigations and unnecessary medical visits for the patient.

UI MeSH Term Description Entries
D011561 Pseudoxanthoma Elasticum An inherited disorder of connective tissue with extensive degeneration and calcification of ELASTIC TISSUE primarily in the skin, eye, and vasculature. At least two forms exist, autosomal recessive and autosomal dominant. This disorder is caused by mutations of one of the ATP-BINDING CASSETTE TRANSPORTERS. Patients are predisposed to MYOCARDIAL INFARCTION and GASTROINTESTINAL HEMORRHAGE. Gronblad-Strandberg Syndrome,Groenblad-Strandberg Syndrome,Pseudoxanthoma Elasticum, Autosomal Dominant,Pseudoxanthoma Elasticum, Forme Fruste,Pseudoxanthoma Elasticum, Incomplete,Elasticum, Incomplete Pseudoxanthoma,Elasticums, Incomplete Pseudoxanthoma,Gronblad Strandberg Syndrome,Incomplete Pseudoxanthoma Elasticum,Incomplete Pseudoxanthoma Elasticums,Pseudoxanthoma Elasticums, Incomplete,Syndrome, Gronblad-Strandberg
D004547 Elastic Tissue Connective tissue comprised chiefly of elastic fibers. Elastic fibers have two components: ELASTIN and MICROFIBRILS. Elastic Fibers,Elastic Fiber,Elastic Tissues,Fiber, Elastic,Fibers, Elastic,Tissue, Elastic,Tissues, Elastic
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D014466 Ultraviolet Rays That portion of the electromagnetic spectrum immediately below the visible range and extending into the x-ray frequencies. The longer wavelengths (near-UV or biotic or vital rays) are necessary for the endogenous synthesis of vitamin D and are also called antirachitic rays; the shorter, ionizing wavelengths (far-UV or abiotic or extravital rays) are viricidal, bactericidal, mutagenic, and carcinogenic and are used as disinfectants. Actinic Rays,Black Light, Ultraviolet,UV Light,UV Radiation,Ultra-Violet Rays,Ultraviolet Light,Ultraviolet Radiation,Actinic Ray,Light, UV,Light, Ultraviolet,Radiation, UV,Radiation, Ultraviolet,Ray, Actinic,Ray, Ultra-Violet,Ray, Ultraviolet,Ultra Violet Rays,Ultra-Violet Ray,Ultraviolet Black Light,Ultraviolet Black Lights,Ultraviolet Radiations,Ultraviolet Ray
D020405 Dermis A layer of vascularized connective tissue underneath the EPIDERMIS. The surface of the dermis contains innervated papillae. Embedded in or beneath the dermis are SWEAT GLANDS; HAIR FOLLICLES; and SEBACEOUS GLANDS. Corium
D035583 Rare Diseases A large group of diseases which are characterized by a low prevalence in the population. They frequently are associated with problems in diagnosis and treatment. Orphan Diseases,Disease, Orphan,Disease, Rare,Orphan Disease,Rare Disease

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