Presentation
(B) Nuclear debris and eosinophilic-degenerated elastic fibers present in multiple channel lumens (H&E, ×400). [ncbi.nlm.nih.gov]
It presents as a group of small reddish bumps. [dermnetnz.org]
These lesions were present on her neck, in her axillary skin folds, antecubital fossa and groin. She had associated skin laxity (figures 1 and 2). [casereports.bmj.com]
Skin
- Kinky Hair
Determination of apoceruloplasmin by radioimmunoassay in nutritional copper deficiency, Menkes' kinky hair syndrome, Wilson's disease, and umbilical cord blood. Pediatr Re. 1974;8:821-4. [PubMed] 13. Nimni ME. Penicillamine and collagen metabolism. [escholarship.org]
Workup
Progress logically from the histologic pattern, through the appropriate workup, around the pitfalls, to the best diagnosis. [books.google.de]
Treatment
However, no 'gold standard' therapy exists among these treatment modalities. [ncbi.nlm.nih.gov]
Elastosis perforans serpiginosa: treatment with liquid nitrogen. Int J Dermatol. 1990 Nov. 29(9):655-6. [QxMD MEDLINE Link]. Humphrey S, Hemmati I, Randhawa R, Crawford RI, Hong CH. [emedicine.medscape.com]
Treatment Options Treatment options are listed in Table I. A consistently effective treatment for EPS is not currently available. [dermatologyadvisor.com]
Prognosis
(Outcomes/Resolutions) The prognosis of Elastosis Perforans Serpiginosa depends upon the severity of the condition; mild cases (idiopathic and reactive forms) have better prognosis than severe ones It is also dependent upon the underlying health condition [dovemed.com]
▪ PROGNOSIS, CLINICAL COURSE, AND TREATMENT The perforating disorders are difficult to treat. Their prognosis is tied to the underlying diseases, so treatment of the underlying cause can be effective. [dermaamin.com]
Prognosis - Ehlers-Danlos syndrome - general Treatment - Ehlers-Danlos syndrome - general Resources - Ehlers-Danlos syndrome - general [checkorphan.org]
Etiology
Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology. [icd10coded.com]
The etiology, pathophysiology, natural history, and treatment options for DPA-induced EPS are reviewed, and the authors suggest this method of treatment to be effective and safe for patients of DPA-induced EPS refractory to conventional therapy. [ncbi.nlm.nih.gov]
Etiology Elastic fibers are perceived by the skin to be abnormal and become the primary objects of an inflammatory attack. They may be abnormal as a result of genetic influence or penicillamine. [emedicine.medscape.com]
There are three distinct etiologies of EPS: Idiopathic EPS - likely a genetic predisposition and the exact cause remains unknown. Approximately 60-70% of EPS cases fall in this category. [aocd.org]
HISTORY ETIOLOGY AND PATHOGENESIS D- penicillamine appears to be a clear trigger for EPS. [dermaamin.com]
Epidemiology
You might also be interested in Dermatology Occurrence/Epidemiology This section has been translated automatically. m>w Manifestation This section has been translated automatically. The first symptoms can already appear in childhood. [altmeyers.org]
Epidemiology Either idiopathic, drug induced (D-penicillamine), or associated with other inherited disorders (Down syndrome, Ehlers-Danlos syndrome, osteogenesis imperfecta, Marfan syndrome, Rothmund-Thomson syndrome) Diagrams / tables Images hosted on [pathologyoutlines.com]
Epidemiology Frequency Elastosis perforans serpiginosa is a rare condition. No geographic preferences are apparent for elastosis perforans serpiginosa. Race No racial differences have been reported for elastosis perforans serpiginosa. [emedicine.medscape.com]
[…] between EPS and Down's syndrome is unknown; however, factors present in this syndrome such as premature skin aging, joint hyperelasticity and acrocyanosis may suggest an underlying connective tissue disorder. 1,2 table 1 illustrates some clinical and epidemiological [scielo.br]
Pathophysiology
The pathophysiology of OI involves mutations in genes encoding α-chains of type I collagen, the main component of the dermis. [jhu.pure.elsevier.com]
The etiology, pathophysiology, natural history, and treatment options for DPA-induced EPS are reviewed, and the authors suggest this method of treatment to be effective and safe for patients of DPA-induced EPS refractory to conventional therapy. [ncbi.nlm.nih.gov]
The pathophysiology is not well understood. [renalfellow.blogspot.com]
There are a few reports of its familial occurrence. [2] The pathophysiology of d-penicillamine induced EPS is due to not only a decrease in the rate of synthesis of elastic fibers in papillary dermis but also over-proliferative state of elastic fibers [e-ijd.org]
Pathophysiology Elastosis perforans serpiginosa (EPS) may be a form of granulomatous inflammation that displays an unusual method for removing elastic tissue from the area of involvement. [emedicine.medscape.com]
Prevention
Your skin Holds body fluids in, preventing dehydration Keeps harmful microbes out, preventing infections Helps you feel things like heat, cold, and pain Keeps your body temperature even Makes vitamin D when the sun shines on it Anything that irritates [icdlist.com]
Key words: Ehlers-Danlos syndrome, elastosis perforans serpiginosa Advertisement American Journal of Preventive Medicine and Public Health SUBMIT YOUR ARTICLE NOW [scopemed.org]
The patient should avoid sun exposure to prevent recurrence. elastosis perforans serpiginosa See: perforating disorder [medical-dictionary.thefreedictionary.com]
An early and lifelong therapy with chelators (penicillamine, trientine) or zinc is necessary to prevent disease evolution. [journals.lww.com]