Elastosis perforans serpiginosa

A condition characterized by transepidermal elimination of ELASTIC TISSUE and the development of lesions in a serpiginous pattern, where scar tissue is deposited beneath the dermis. It occurs in several connective tissue disorders including MARFAN SYNDROME; EHLERS-DANLOS SYNDROME; OSTEOGENESIS IMPERFECTA; PSEUDOXANTHOMA ELASTICUM as well as DOWN SYNDROME. It may also occur in isolation as autosomal dominant genetic trait. OMIM: 130100

Also Known As:

Networked: 65 relevant articles (6 outcomes, 2 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Hepatolenticular Degeneration (Wilson's Disease)
2.	Pseudoxanthoma Elasticum (Gronblad Strandberg Syndrome)
3.	Skin Diseases (Skin Disease)
4.	Cutis Laxa
5.	Down Syndrome (Down's Syndrome)

Experts

1.	Chen, Lianjun: 2 articles (07/2016 - 03/2015)
2.	Liang, Jun: 2 articles (07/2016 - 03/2015)
3.	Wang, Duoqin: 2 articles (07/2016 - 03/2015)
4.	Xu, Jinhua: 2 articles (07/2016 - 03/2015)
5.	Heusinkveld, Lauren E: 1 article (12/2021)
6.	Lucas, Jennifer: 1 article (12/2021)
7.	Michalik, Daniel: 1 article (12/2021)
8.	Enos, Tyler: 1 article (05/2021)
9.	Rainwater, Yevgeniya: 1 article (05/2021)
10.	Vandergriff, Travis: 1 article (05/2021)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Elastosis perforans serpiginosa:

1.	Penicillamine (Cuprimine)FDA Link 10/01/1982 - "Penicillamine induced elastosis perforans serpiginosa: an ultrastructural study of two cases." 11/01/2020 - "A case of elastosis perforans serpiginosa associated with long-term treatment with penicillamine." 08/20/2017 - "D-penicillamine-induced Elastosis Perforans Serpiginosa." 07/01/2016 - "Two cases of D-penicillamine-induced elastosis perforans serpiginosa." 03/01/2015 - "A case of D-penicillamine(DPA) induced elastosis perforans serpiginosa(EPS) in a 32-year-old Chinese man was reported. "
2.	ElastinIBA 10/01/2000 - "In the lesional skins of patients with elastosis perforans serpiginosa, the 67 kDa elastin receptor was specifically expressed in the epidermis immediately surrounding the elastic materials that were being eliminated. " 04/01/2018 - "Elastosis perforans serpiginosa (EPS) is a rare skin disorder in which there is transepithelial elimination of elastin fibers. " 10/01/2000 - "The elastin receptor may be involved in the interaction between keratinocytes and elastin in elastosis perforans serpiginosa." 10/01/2000 - "To delineate the molecular mechanism of transepidermal elimination of dermal elastic materials in elastosis perforans serpiginosa, the interaction between elastin and cultured keratinocytes was studied in vitro. " 01/01/1978 - "Ultrastructural and biochemical studies on the collagen and elastin fibres of the dermis from a patient with Elastosis perforans serpiginosa are described. "
3.	NitrogenIBA 11/01/1990 - "Elastosis perforans serpiginosa: treatment with liquid nitrogen." 01/01/1998 - "As its efficacy has been reported in elastosis perforans serpiginosa, we used liquid nitrogen cryotherapy on the inflammatory lesions with good results (separation of epidermis from dermis perhaps promoting transepithelial elimination of the abnormal dermal elastic tissue)." 05/01/1983 - "Liquid nitrogen cryotherapy in a case of elastosis perforans serpiginosa."
4.	Isotretinoin (Accutane)FDA LinkGeneric 01/01/2019 - "To the best of our knowledge no such report has been published in the past, even though there is a single report of effectiveness of isotretinoin in elastosis perforans serpiginosa. " 01/01/1994 - "Penicillamine-induced elastosis perforans serpiginosa treated successfully with isotretinoin."
5.	1-phenyl-3,3-dimethyltriazene (PDT)IBA 03/01/2015 - "Effective treatment of d-penicillamine induced elastosis perforans serpiginosa with ALA-PDT."
6.	CollagenIBA 01/01/1988 - "Structural alterations of collagen fibrils in the reactive type of elastosis perforans serpiginosa." 08/01/2018 - "Elastosis perforans serpiginosa (EPS) is an uncommon cutaneous disorder classified under perforating diseases (PD); a group of dermatoses with transepidermal extrusion of collagen or elastic tissue. " 01/01/1978 - "Ultrastructural and biochemical studies on the collagen and elastin fibres of the dermis from a patient with Elastosis perforans serpiginosa are described. " 04/01/1998 - "Each of the classic perforating disorders of skin, including elastosis perforans serpiginosa, perforating folliculitis, reactive perforating collagenosis, Kyrle's disease, and perforating disorder of uremia, have been shown to extrude collagen, elastin, and related extracellular matrix components through the epidermis. " 01/01/1988 - "D-penicillamine (DPA) leads to side effects in different ways: collagen and elastin crosslinking are inhibited, which results in thin and vulnerable skin, cutis laxa, elastosis perforans serpiginosa, wound healing defects and embryopathy. "
7.	AcitretinFDA Link 05/01/2021 - "Treatment of extensive elastosis perforans serpiginosa with acitretin in a man with Down syndrome." 01/01/2019 - "D-penicillamine-induced pseudo-pseudoxanthoma elasticum and extensive elastosis perforans serpiginosa with excellent response to acitretin."
8.	Imiquimod (Aldara)FDA LinkGeneric 07/01/2006 - "Imiquimod therapy for elastosis perforans serpiginosa." 08/31/2010 - "Elastosis Perforans Serpiginosa in a patient with Down syndrome treated with imiquimod 5% cream."
9.	Cyclosporine (Ciclosporin)FDA LinkGeneric 01/01/2020 - "Elastosis perforans serpiginosa induced by d-penicillamine treated with cyclosporine and allopurinol."
10.	Biomarkers (Surrogate Marker)IBA 04/01/2008 - "Development of elastosis perforans serpiginosa in association with progressive vaso-occlusive disease may be a biological marker of progression of moyamoya disease."

Therapies and Procedures

1.	Therapeutics 01/01/2019 - "Our results show that pulse dye laser therapy is a safe and effective treatment for elastosis perforans serpiginosa." 01/01/2019 - "He was diagnosed with Elastosis Perforans Serpiginosa on tissue biopsy and underwent pulse dye laser therapy over four years with excellent results. " 01/01/2019 - "Pulse Dye Laser Therapy Successful for Elastosis Perforans Serpiginosa." 01/01/2021 - "A case of acquired disseminated elastosis perforans serpiginosa in a patient with HIV, hepatitis B and hepatitis C successfully treated with narrowband ultraviolet B therapy." 01/01/2011 - "Elastosis perforans serpiginosa (EPS) is a rare complication of chronic therapy with a high-dose of D-penicillamine (1 g daily for more than 5 years), characterized by the elimination of abnormal elastic fibers from the upper dermis through the epidermis. "
2.	Dye Lasers 06/01/2003 - "Response of elastosis perforans serpiginosa to pulsed CO2, Er:YAG, and dye lasers." 11/01/2000 - "Treatment of elastosis perforans serpiginosa with the flashlamp pulsed dye laser." 01/01/2019 - "Our results show that pulse dye laser therapy is a safe and effective treatment for elastosis perforans serpiginosa." 01/01/2019 - "He was diagnosed with Elastosis Perforans Serpiginosa on tissue biopsy and underwent pulse dye laser therapy over four years with excellent results. " 01/01/2019 - "Pulse Dye Laser Therapy Successful for Elastosis Perforans Serpiginosa."
3.	Cryotherapy (Therapy, Cold) 12/01/2012 - "A case of elastosis perforans serpiginosa treatment with cryotherapy." 01/01/1998 - "As its efficacy has been reported in elastosis perforans serpiginosa, we used liquid nitrogen cryotherapy on the inflammatory lesions with good results (separation of epidermis from dermis perhaps promoting transepithelial elimination of the abnormal dermal elastic tissue)." 05/01/1983 - "Liquid nitrogen cryotherapy in a case of elastosis perforans serpiginosa."
4.	Lasers (Laser) 09/01/2000 - "Localized idiopathic elastosis perforans serpiginosa effectively treated by the Coherent Ultrapulse 5000C aesthetic laser." 01/01/2019 - "We describe a case of elastosis perforans serpiginosa and its successful management with PDL laser. "
5.	Gas Lasers 10/01/2017 - "Treatment of Elastosis Perforans Serpiginosa Using a Fractional Carbon Dioxide Laser." 04/01/2011 - "Treatment of elastosis perforans serpiginosa with the pinhole method using a carbon dioxide laser."