Elastosis Perforans Serpiginosa Research Articles

A Systematic Review of Case Reports of a Rare Dermatological Condition: Elastosis Perforans Serpiginosa.

A systematic review was carried out on a rare dermatological condition affecting papillary dermal tissue fibers of the skin known as elastosis perforans serpiginosa (EPS). The aim of this review was to highlight this skin disease, its association with other medical conditions, and its management. The search was conducted by using the keywords"elastosis perforans serpiginosa" and "case reports" in the databases. A total of 10 case reports were analyzed and presented by the parameters like age, gender, chief complaints, and medical history. The most common causes of EPS were drug-induced and occurred along with the Wilson disease. The study concluded that as EPS is an uncommon disease with few instances, there is a need for further research to analyze randomized controlled trials that have been conducted in relation to the condition.

Miscellaneous annular diseases.

Annular lesions represent a unique macro-morphologic pattern in various skin disorders that may be found in pityriasis rotunda, elastosis perforans serpiginosa, subacute nodular migratory panniculitis, keratolysis exfoliativa, neutrophilic eccrine hidradenitis, hemophagocytic lymphohistiocytosis, and intentionally induced annular lesions. This group is highly heterogenous and variable in clinical presentation. Whereas some are benign self-limiting disorders like pityriasis rotunda, others such as hemophagocytic lymphohistiocytosis follow a chronic course or have a potential of being life-threatening. Epidemiology, pathogenesis, histopathology, clinical presentation and diagnosis, differential diagnosis, and treatment are discussed.

Kyrle’s Disease: A Case Report and Literature Review

BACKGROUND: Perforating dermatoses are heterogeneous skin disorders characterized by transepidermal elimination of dermal tissue components. They can be characterized by the primary or secondary pathogenesis. Primary perforating dermatoses can be divided into four types, according to the eliminated dermal materials: Kyrle disease, perforating reactive collagenosis, elastosis perforans serpiginosa, and perforating folliculitis. The secondary form is also known as acquired perforating dermatosis and designates adult patients with coexisting systemic diseases, regardless of the dermal materials eliminated. The aim of this review is to investigate the potential relationship between Kyrle disease and associated conditions, reporting a rare case of this disease in a patient with chronic kidney disease. CASE PRESENTATION: We report the case of a 55-year-old male patient who presented generalized pruritus and skin lesions on the upper limbs and trunk for about 6 months. Pathological history revealed systemic arterial hypertension and chronic kidney disease (CKD), for which the patient had been on hemodialysis since more than 3 years. CONCLUSION: We reviewed the literature, reporting a rare case of KD in a patient with chronic renal failure on hemodialysis. KD is an acquired dermatologic condition that lacks a standardized treatment approach and can be notoriously difficult to cure. This article provides an overview of the most frequent comorbidities and treatment methodologies previously reported in the literature. If KD is suspected, underlying systemic disorders need to be investigated.

Підхід до діагнозу перфоруючого серпігінуючого еластозу

The goal is to demonstrate a clinical case of serpiginous perforating elastosis for better awareness of doctors.The hallmark of the perforating diseases is the transepidermal elimination of degraded dermal componenrs. The material extruded may represent abnormally constituded collagen, elastic fibers or other matrix components. Four classical forms of primary perforating dermatosis are described: reactive perforating collagenosis, Kyrle’s disease, perforating folliculitis and elastosis perforans serpiginosa.

Familial Reactive Perforating Collagenosis: Clinical, Dermoscopy, and Histopathological Features in Two Siblings

Transepidermal elimination disorders include reactive perforating collagenosis (RPC), perforating folliculitis, elastosis perforans serpiginosa, and Kyrle’s disease. RPC is a rare transepidermal elimination disorder wherein altered collagen is extruded through the epidermis. It has two types: rare inherited form and relatively common acquired form. Most inherited cases present during infancy or early childhood with papules on extensor surface of hands, elbows, knees preceded by superficial trauma. We report two female siblings of 11 and 10 years with multiple, symmetrically distributed, hyperpigmented papules with central keratotic plug, predominantly over extensor aspects of extremities with no aggravating and relieving factors. Clinical diagnosis of familial RPC was confirmed by consistent dermoscopic and histopathological findings. Both children were started on oral isotretinoin 10 mg/day with topical 12% ammonium lactate.

Newly detected gene mutation in Ehlers-Danlos syndrome with elastosis perforans serpiginosa.

Newly detected gene mutation in Ehlers-Danlos syndrome with elastosis perforans serpiginosa.

Treatment of elastosis perforans serpiginosa with the 585-nm pulsed dye laser

Treatment of elastosis perforans serpiginosa with the 585-nm pulsed dye laser

Elastic oddity: A case report of a rare occurrence of elastosis perforans serpiginosa in Down’s syndrome

Elastic oddity: A case report of a rare occurrence of elastosis perforans serpiginosa in Down’s syndrome

Perforating folliculitis in Down’s syndrome − a rare case report

Perforating folliculitis is a primary perforating disorder, characterized by the phenomenon of transepidermal elimination. It is associated with various disorders such as psoriasis, juvenile acanthosis nigricans, diabetic mellitus, renal failure, cardiac failure, and malignant neoplasia. Other primary perforating dermatoses are Kyrle’s disease, reactive perforating collagenosis, and elastosis perforans serpiginosa. Among the association of perforating dermatoses with genetic diseases like Down’s syndrome, elastosis perforans serpiginosa and very rarely reactive perforating collagenosis have been reported. We report the first case of an 11-year-old boy who is a known case of Down syndrome presented with perforating folliculitis.

A rare case report of elastosis perforans serpiginosa in acquired cutis laxa

Elastosis perforans serpiginous (EPS) is a rare perforating disorder characterized by trans epidermal elimination of fragmented elastic fibres.The cutaneous features include asymptomatic hyperkeratotic papules. Here we present a case of 27-year old female presented with hyperkeratotic papules with central depression arranged transversely in a serpiginous fashion on front of the laxed skin of the neck region. The histopathological features are consistent with the features of EPS on Cutis Laxa. Elastosis perforans serpiginosa in Acquired cutis laxa has not been reported very rarely, hence this case has been reported.

Acquired perforating dermatoses show increased levels of cutaneous advanced glycation end-products.

Acquired perforating dermatoses (APDs) are characterized by transepidermal elimination of skin materials. Altered glycation of dermal components may be involved in pathogenesis. To assess whether patients affected by APDs have increased levels of cutaneous advanced glycation end-products (AGEs). A cross-sectional controlled study involving a total of 109 patients was conducted, enrolling 29 patients consecutively diagnosed with primary APDs [reactive perforating collagenosis (RPC), elastosis perforans serpiginosa (EPS), perforating folliculitis (PF) and Kyrle disease (KD)], 40 age- and sex-matched healthy controls (HCs) and 40 patients with mild atopic dermatitis (AD). The levels of cutaneous AGEs were measured using a validated fluorescence technique. The median skin autofluorescence value in patients with APDs was significantly higher [2.7 arbitrary units (AU), interquartile range (IQR) 1.9-3.9 AU] compared with HCs (1.8 AU, IQR 1.6-2.3 AU; P < 0.001) and patients with AD (2.1 AU, IQR 1.9-2.3 AU; P = 0.01). Median values were 3.5 AU (IQR 2.7-4.6 AU) for RPC, 1.83.5 AU (1.4-2.4 AU) for EPS, 3.1 AU (2.4-4.4 AU) for PF and 2.6 AU (2.3-3.1 AU) for KD. Our results may suggest a possible physiopathological role of AGEs in the transepidermal elimination mechanisms involved in certain APDs.

The dermatological aspects of hEDS in women.

Ehlers–Danlos syndrome (EDS) is a group of inherited connective tissue disorders that may present with a wide range of multisystemic symptoms. Hypermobile EDS, one of 13 identified subtypes of EDS, is the only variant without a known associated genetic mutation. A review of the literature suggests the five primary dermatological changes associated with hypermobile EDS are soft skin, atrophic cutaneous scars, piezogenic papules, hyperextensive stretchability, and hematomas. Our paper will address these cutaneous manifestations and delve into how they affect patients (primarily women). Possible consequences and treatment options for these different dermatological changes, as well as other skin manifestations such as livedo reticularis and elastosis perforans serpiginosa, will also be further explored.

Clinical practice guide for the treatment of perforating dermatosis

Perforating dermatoses are a heterogeneous skin disease group defined by transepidermal elimination of various skin materials. Four classical forms of primary perforating dermatosis have been described, where the transepidermal elimination mechanism represents the hallmark of the disease: acquired reactive perforating collagenosis, elastosis perforans serpiginosa, Kyrle's disease and perforating folliculitis. Acquired reactive perforating collagenosis presents with transepidermal elimination of collagen fibers. Elastosis perforans serpiginosum presents with the elimination of elastic fibers. Kyrle's disease presents with transepidermal elimination of abnormal keratin. In perforating folliculitis, it is the content of the follicle. We established diagnostic criteria and severity classification. In addition, the Japanese guideline for treatment of perforating dermatoses was updated using the Medical Information Network Distribution Service (MINDS) methodology. The guideline is based on a systematic published work review completed from 1989 to 2019, and on a formal consensus and approval process. Most medical published work on the treatment is limited to individual case reports and small series of patients. The guideline covers treatment options considered relevant by the expert panel and approved in Japan at the time of the consensus conference.

A case of elastosis perforans serpiginosa associated with long‐term treatment with penicillamine

A case of elastosis perforans serpiginosa associated with long‐term treatment with penicillamine

Elastosis perforans serpiginosa induced byd‐penicillamine treated with cyclosporine and allopurinol

Elastosis perforans serpiginosa (EPS) is a rare condition within the group of perforating dermatoses. It is characterized by the synthesis of anomalous elastic fibers that are eliminated through perforating channels (transepidermal elimination). It is classified into three subtypes. One of them is drug-induced by prolonged treatment with d-penicillamine. This drug is a heavy metal chelator used to treat diseases such as rheumatoid arthritis, cystinuria, and Wilson's disease. Years of treatment with d-penicillamine at high doses are required for developing EPS, with occasional slow regression after drug withdrawal. There is no established treatment for EPS, with described cases using various treatment options such as corticoids, retinoids, tazarotene, cryotherapy, imiquimod, photodynamic therapy, electrosurgery, and CO2 laser among others with inconsistent results. We present a case of EPS induced by d-penicillamine with favorable response to cyclosporine and allopurinol in a patient with a history of Wilson's disease since childhood. They maybe considered as possible therapeutic options not described so far for an entity with variable response to current treatments. We highlight the extensive involvement of the case with progression, despite the suspension of d-penicillamine and failure to previous treatments with photodynamic therapy and retinoids.

Elastosis perforans serpiginosa- A rare case report

Elastosis perforans serpiginosa is a rare primary perforating disorder. In EPS, there is elimination of altered elastic fibres from papillary layer of dermis to epidermis which is described as transelimination disorder. It is characterized as multiple hyperkeratotic umbilicated papule which are arranged in annular or serpiginous pattern with raised edge and central plug. These are more commonly distributed over back, sides of neck and extremities in bilateral symmetrical pattern. It is common in age group of 5 to 20 years with male predominance. Lesions are generally self limiting in nature. Isotretinoin is used in penicillamine induced disease. A 45 year old diabetic female presented with chief complaints of asymptomatic dark coloured raised lesions on face, sides of neck and upper extremities in bilateral symmetrical pattern for last four years. On cutaneous examination multiple asymptomatic hyperpigmented keratotic papular lesions, coalescing to form plaques were arranged in annular, serpiginous and polycyclic pattern over face, sides of neck, chest and forearms distributed in bilaterally symmetrical pattern with central sparing and atrophy. Patient underwent general and systemic examination followed by routine and histopathological examination. Routine investigations were normal except random blood sugar and HBA1C levels were raised. Histopathological examination revealed hyperkeratotic and acanthotic epidermis with transepidermal channels having nuclear debris and elastic fibres. Patient was explained about course and prognosis of disease and was stared on topical and oral retinoids. Later, she was lost follow up.

Elastosis perforans serpiginosa in two sibling patients with Down syndrome: Case report

Elastosis perforans serpiginosa in two sibling patients with Down syndrome: Case report

Cutaneous Elastic Tissue Anomalies.

After a review of the physiology in the formation and degradation of cutaneous elastic tissue, we describe the clinicopathologic disorders characterized by increased and decreased cutaneous elastic tissue. Cutaneous disorders characterized by increased and/or abnormal elastic tissue in the dermis include elastoma, also named nevus elasticus, dermatosis lenticularis disseminata, pseudoxanthoma elasticum, late-onset focal dermal elastosis, linear focal elastosis, elastoderma, elastofibroma dorsi, and elastosis perforans serpiginosa. In some of these conditions, the specific histopathologic diagnosis may be rendered with hematoxylin-eosin stain, whereas in other ones special elastic tissue stains are necessary to demonstrate the anomalies. Cutaneous disorders characterized by decreased dermal elastic tissue include nevus anelasticus, papular elastorrhexis, perifollicular elastolysis, anetoderma cutis laxa, postinflammatory elastolysis and cutis laxa, white fibrous papulosis of the neck, pseudoxanthoma elasticum-like papillary dermal elastolysis, and mid dermal elastolysis. In most of these conditions, the histopathologic anomalies are only seen with elastic tissue stains, and cutaneous biopsies of these processes stained with hematoxylin-eosin show appearance of normal skin. The diagnosis of some of these disorders characterized by increased or decreased elastic dermal tissue should be followed by general exploration of the patient to rule out associated severe systemic anomalies, and in some cases, a genetic counseling should be offered to the family.

D-penicillamine-induced elastosis perforans serpiginosa in a case of Wilson's disease

The use of D-penicillamine has been well established in various disorders, mainly Wilson's disease, cystinuria, and rheumatoid arthritis. Elastosis perforans serpiginosa (EPS) is a rare perforating dermatosis, occurring as an uncommon sequel of long-term high-dose D-penicillamine therapy. We present a rare case of EPS in a known case of Wilson's Disease on D-penicillamine therapy. Biopsy revealed characteristic “lumpy-bumpy” elastic fibers, a finding specific to penicillamine-induced EPS.

Elastosis perforans serpiginosa related to vascular Ehlers-Danlos syndrome

Elastosis perforans serpiginosa (EPS) is a rare skin disease with elimination of connective tissue fibers from dermis to epidermis. The typical presentation shows hyperkeratotic red or skin-colored papules arranged in a circinate pattern. We present a 26-year-old woman with EPS known to have vascular Ehlers-Danlos syndrome.