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 ). Figure 1 Keratotic papules, axillary fold. [casereports.bmj.com]

We present a new case of D-penicillamine (DPA)-induced EPS, and describe the clinical, dermoscopic, histopathological and direct immunofluorescence (DIF) findings. A 33-year-old woman receiving treatment with DPA presented with annular skin lesions. [ncbi.nlm.nih.gov]

• Asymptomatic

  […] keratotic papules; the epidermis is thickened around a central plug of dermal elastic tissue which is extruded through the epidermis. e·las·to·sis per·fo·rans ser·pi·gi·no·sa ( ē-las-tōsis perfōr-anz sĕr-pi-ji-nōsă ) Circinate groups of asymptomatic [medical-dictionary.thefreedictionary.com]

  The lesions are generally asymptomatic or slightly itching. Several treatments have been reported with poor long-term success; these include intralesional and topical corticosteroids, tazarotene, imiquimod, and cryotherapy. [ncbi.nlm.nih.gov]

• Trisomy 21

  A 12-year-old girl with trisomy 21 (Downs syndrome) presented with an approximate 2-year history of an asymptomatic eruption on the right upper arm and left lateral neck that was clinically and histologically diagnostic of elastosis perforans serpiginosa [ncbi.nlm.nih.gov]

  The systemic disorders reportedly associated with EPS include trisomy 21, Ehlers-Danlos syndrome, osteogenesis imperfecta, scleroderma, cutis laxa, Rothmund-Thomson syndrome, acrogeria, pseudoxanthoma elasticum, and Moya Moya disease.[6,7] Trisomy 21 [jcadonline.com]

  Elastosis perforans serpiginosa in a patient with trisomy 21. Dermatol Online J. 2006;12: 5. 4. Mehta RK, Burrows NP, Payne CM, Mendelsohn SS, Pope FM, Rytina F. Elastosis perforans serpiginosa and associated disorders. [revista.asocolderma.org.co]

• Arthritis

  We report a case of D-penicillamine-induced elastosis perforans serpiginosa with unique clinical features occurring in a 10-year-old child with juvenile rheumatoid arthritis who received only 71 gm of the drug over 9 months. [ncbi.nlm.nih.gov]

  Register or Login Full Menu Full Menu CME Diseases & Conditions Acne Actinic Keratosis Aesthetic Dermatology Atopic Dermatitis Autoimmune Diseases Contact Dermatitis Hair & Nails Melanoma Nonmelanoma Skin Cancer Pigmentation Disorders Psoriasis Psoriatic Arthritis [mdedge.com]

  NIH: National Institute of Arthritis and Musculoskeletal and Skin Diseases Acrodermatitis (Medical Encyclopedia) Cryotherapy (Medical Encyclopedia) Cutaneous skin tags (Medical Encyclopedia) Dry skin -- self-care (Medical Encyclopedia) Erythema multiforme [icdlist.com]

• Papule

  Figure 1 Keratotic papules, axillary fold. Figure 2 Skin laxity and keratotic papules grouped in serpiginous and arciform pattern, anterior neck. [casereports.bmj.com]

  On cutaneous examination, there was evidence of multiple papules, few nodules and a single plaque involving nape and front of the neck and the left upper limb, respectively. [e-ijd.org]

  In addition, there were several small yellowish papules on both sides of the neck which eventually became confluent to form plaques. Histopathology confirmed the diagnosis of elastosis perforans serpiginosa and pseudoxanthoma elasticum. [ncbi.nlm.nih.gov]

  On clinical examination, erythematous papules with central crusts, coalescing to form serpiginous lesions were seen over arms, [Figures 1 and 2]. [docksci.com]

  […] in the epidermis; the lesions are usually arranged in arc-shaped, continuous clusters on the sides of the nape, face, or arms. e·las·to·sis per·'fo·rans ser·pi·gi·no·'sa [MIM*130100] circinate groups of asymptomatic keratotic papules; the epidermis is [medical-dictionary.thefreedictionary.com]

• Eruptions

  These lesions begin as figurate groups of papules which are dome shaped and often umbilicated, and usually only one area at a time breaks out, except in Down's Syndrome patients, where they can have a generalized eruption. [dermchallenge.blogspot.com]

  The elastosis perforans serpiginosa eruption may appear 1 or more years after the start of treatment. This eruption often is symmetrical, widespread, and long lasting. [emedicine.medscape.com]

  A 12-year-old girl with trisomy 21 (Downs syndrome) presented with an approximate 2-year history of an asymptomatic eruption on the right upper arm and left lateral neck that was clinically and histologically diagnostic of elastosis perforans serpiginosa [ncbi.nlm.nih.gov]

  The result is a papular eruption that is usually arranged serpiginously, annularly, or arcuately. [jamanetwork.com]

• Skin Disease

  […] sweat gland disease + systemic scleroderma + Ter Haar Syndrome tyrosinemia type II Upton Young Syndrome urticaria + vascular skin disease + verruciform xanthoma of skin vesiculobullous skin disease + [rgd.mcw.edu]

  Identify challenging types of skin cancer, including cutaneous lymphomas and melanocytic lesions, as well as non-neoplastic skin diseases such as panniculitis; drug eruptions, including reactions to newer chemotherapeutic agents; infectious diseases; [books.google.de]

  Elastosis perforans serpiginosa is a rare skin disease in which abnormal elastic fibers, other connective tissue elements, and cellular debris are expelled from the papillary dermis through the epidermis. [ncbi.nlm.nih.gov]

• Keratosis

  Ichthyosis, acquired Keratoderma blennorrhagicum Keratoderma foot, acquired Keratoderma punctata Keratosis Keratosis follicularis et parafollicularis in cutem penetrans Keratosis punctata Keratosis, pilaris, acquired Secondary keratoderma 701.1 Excludes [icd9data.com]

  L85.1 Acquired keratosis [keratoderma] palmaris et plantaris L85.2 Keratosis punctata (palmaris et plantaris) L85.8 Other specified epidermal thickening L85.9 Epidermal thickening, unspecified L86 Keratoderma in diseases classified elsewhere L87 Transepidermal [icd10data.com]

  Lutz W (1953) Keratosis follicularis serpiginosa. Dermatologica 106: 318-320 Kim SW et al.(2014) A clinicopathologic study of thirty cases of acquired perforating dermatosis in Korea. [altmeyers.org]

  […] subcutaneous tissue (L80-L99)|L87 Transepidermal elimination disorders Results for Elastosis perforans serpiginosa and additional synonyms L87.2 Elastosis perforans serpiginosa Synonyms: Elastosis perforans|Elastosis perforans serpiginosa (disorder)|Keratosis [averbis.com]

• Acrogeria

  Down syndrome, osteogenesis imperfecta, acrogeria, Ehlers-Danlos syndrome type IV, Marfan syndrome, pseudoxanthoma elasticum, Rothmund-Thomson syndrome, and scleroderma have all been associated with EPS. [ncbi.nlm.nih.gov]

  Acrogeria lacks systemic symptoms, but will have features of premature aging, with skin/subcutaneous atrophy that tends to favor the distal extremities. [neurologyadvisor.com]

  EPS may be associated with various underlying conditions and connective tissue disorders including Down's syndrome, Ehlers-Danlos syndrome type IV (vascular type), osteogenesis imperfecta, scleroderma, diabetes mellitus, acrogeria, Marfan syndrome, Rothmund-Thomson [xpertdox.com]

  Reactive elastosis perforans serpiginosa This form of elastosis perforans serpiginosa is associated with other diseases (eg, Down syndrome, Ehlers-Danlos syndrome, Marfan syndrome, osteogenesis imperfecta, scleroderma, acrogeria, perforating granuloma [emedicine.medscape.com]

• Irritability

  Allergies, irritants, your genetic makeup, and certain diseases and immune system problems can cause rashes, hives, and other skin conditions. Many skin problems, such as acne, also affect your appearance. [icdlist.com]

  The only adverse effect observed in both cases was mild irritation. However, the irritation subsided after a few weeks of therapy. Both patients continue to use tazarotene daily for intermittent courses when their disease flares. [jamanetwork.com]

  Focal dermal irritation is believed to induce the formation of a transepidermal channel to eliminate the irritant agent. [scielo.br]

  […] dermis may induce formation of epidermal and follicular channels to extrude the irritating agent. [de.slideshare.net]

Progress logically from the histologic pattern, through the appropriate workup, around the pitfalls, to the best diagnosis. [books.google.de]

Kawakami T et al.(2020) Guideline: Clinical practice guide for the treatment of perforating dermatosis doi.org/10.1111/1346-8138.15647. Lutz W (1953) Keratosis follicularis serpiginosa. [altmeyers.org]

However, no 'gold standard' therapy exists among these treatment modalities. [ncbi.nlm.nih.gov]

[…] authors.[2‑4] In this case, patient was given the option of treatment with CO 2 laser and cryotherapy. [docksci.com]

Treatment Options Treatment options are listed in Table I. A consistently effective treatment for EPS is not currently available. [neurologyadvisor.com]

(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]

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]

McKee's Pathology of the Skin is the most complete, in-depth resource on dermatopathology, covering etiology, pathogenesis, disease mechanisms, and recent genetic, molecular, and basic science data. Drs. J. [books.google.es]

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]

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]

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]

You might also be interested in Internal medicine 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]

[…] 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]

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]

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 of OI involves mutations in genes encoding α-chains of type I collagen, the main component of the dermis. [jhu.pure.elsevier.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]

The pathophysiology is not well understood. [renalfellow.blogspot.com]

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]

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]

The patient should avoid sun exposure to prevent recurrence. elastosis perforans serpiginosa See: perforating disorder [medical-dictionary.thefreedictionary.com]

Key words: Ehlers-Danlos syndrome, elastosis perforans serpiginosa Advertisement American Journal of Preventive Medicine and Public Health SUBMIT YOUR ARTICLE NOW [scopemed.org]

Currently, there are no specific methods or guidelines to prevent Elastosis Perforans Serpiginosa Drugs that cause the drug-induced form of EPS may be discontinued or alternative medications used Undertaking proper treatment of associated disorders can [dovemed.com]

An early and lifelong therapy with chelators (penicillamine, trientine) or zinc is necessary to prevent disease evolution. [journals.lww.com]