Elastosis perforans serpiginosa induced by d-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.

Perforating pseudoxanthoma elasticum with secondary elastosis perforans serpiginosa-like changes: dermoscopy, confocal microscopy and histopathological correlation.

Pseudoxanthoma elasticum is a rare congenital inherited elastolytic disorder that has sometimes been observed in association with transepidermal elimination of altered and calcified elastic fibers resulting in elastosis perforans serpiginosa-like changes. In this case, histopathology is usually performed to rule out other conditions. The case of a 38-year-old woman with two slowly enlarging asymptomatic plaques occurring on the neck and surrounded by coalescing yellowish papules with a typical cobblestone appearance, evaluated by polarized light dermoscopy and reflectance confocal microscopy with histopathologic correlations, is described. Noteworthy, with reflectance confocal microscopy, the transepidermal elimination of the altered elastic fibers in the plaques was detected as hyperreflective material filling the dermal papillae, whereas the transversal cleavage of the calcified elastic fibers yielded a peculiar 'eggs-in-the-basket' feature.

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.

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.

Idiopathic elastosis perforans serpiginosa with satisfactory response after 5-ALA photodynamic therapy.

Photodynamic therapy (PDT) involves the use of photochemical reactions mediated through the interaction of photosensitizing agents, light, and oxygen for the treatment of malignant or benign diseases. Topical photosensitizers employed in dermatology are 5-aminolevulinic acid (5 ALA) and methyl aminolevulinate, classically used for the treatment of superficial non-melanoma skin cancer and their precursors. Recently the efficacy of PDT has been introduced in other benign diseases. Elastosis perforans serpiginosa (EPS) is a rare skin disorder characterized by transepidermal elimination of abnormal elastic fibers. Management of this condition is complicated, various methods have been used but with limited success. We report a case of EPS in a 30-yeard-old woman treated with 5 ALA-PDT. After 4 sessions the lesions have almost completely disappeared with no residual side effects. Therefore we present an effective and safe alternative for the treatment of EPS.

Elastosis perforans serpiginosa: causes and associated disorders.

BACKGROUND: 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. Three EPS subtypes have been reported that differ according to aetiology, associated diseases, and histopathological features. Herein, we report a systematic review of the literature, as well as a case of a 41-year-old woman with Wilson disease treated with penicillamine (PCM), who developed EPS after 11 years of drug intake. OBJECTIVES: To analyse and characterise EPS subtypes based on an evaluation of potential different histological patterns. MATERIALS & METHODS: A systematic literature search in Pubmed was performed to identify articles describing EPS. RESULTS: A peculiar histological pattern was identified in EPS PCM-related patients, either in affected or unaffected skin samples. Using specific elastic fibre stains (Verhoeff-van Gieson, Weigert, and Orcein), fibres appeared with an irregular surface with thorn-like protrusion, probably due to weaker fibre cross-links, making them unable to re-expand after contraction along their long axis. Interestingly, similar histological patterns have also been reported in elastic tissues of vessel walls of the lungs and upper respiratory tract, joints, visceral adventitia, and kidney. CONCLUSIONS: A distinctive histological pattern of PCM-related EPS is observed in affected and normal-appearing skin, as well as extracutaneous elastic tissue, suggesting serious potential widespread drug-induced systemic elastolytic damage.

Elastosis perforans serpiginosa in a case of pseudoxanthoma elasticum: A rare association.

Elastosis perforans serpiginosa (EPS), characterized by transepidermal elimination of fragmented elastic fibers, clinically presents as hyperkeratotic papules. EPS is classified into three types: (1) Idiopathic; (2) reactive, with associated connective tissue diseases such as pseudoxanthoma elasticum (PXE), Ehlers-Danlos syndrome, cutis laxa, Marfan syndrome, osteogenesis imperfecta, Down's syndrome; (3) the one that is induced by D-penicillamine. A rare association of EPS with PXE, which is primarily a defect of transmembrane transporter protein with accumulation of certain metabolic compounds and secondary calcification of elastic fibers has been documented in the literature. We report a case of PXE with associated lesions that were histopathologically compatible with EPS.

Effective treatment of d-penicillamine induced elastosis perforans serpiginosa with ALA-PDT.

A case of D-penicillamine(DPA) induced elastosis perforans serpiginosa(EPS) in a 32-year-old Chinese man was reported. The presentation lasted two years and was refractory to traditional medical treatment. He was then commenced on 7.6% 5-aminolevulinic acid (ALA) induced photodynamic therapy(PDT) by a LED light of 633 nm at dose levels of 130J/ cm2 for each session with total 3 sessions at one week interval. The patient was tolerated and responded well to this new approach for DPA-induced EPS without any adverse events. 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.

Elastosis perforans serpiginosa.

Elastosis perforans serpiginosa is a rare skin disease characterized by transepidermal elimination of abnormal elastic fibers. This condition classically presents as small papules arranged in serpiginous or annular patterns on the neck, face, arms, or other flexural areas. While these lesions may spontaneously resolve, they often persist for longer periods of time. Though numerous treatment modalities have been described in the literature, most are not very effective. A 35-year-old man presented to Gangnam Severance Hospital with a 2-year history of skin eruptions on his neck, which were refractory to topical antifungal or steroid treatment. On examination, the patient showed multiple crusted and erythematous papules arranged in an annular pattern with central clearing. A biopsy specimen revealed acanthosis with notable transepidermal elimination of nuclear debris and eosinophilic degenerated elastic fibers from the dermis via an epidermal channel. Verhoeff-van Gieson staining showed dense clumps of altered elastic fibers in the papillary dermis. Based on these findings, a diagnosis of elastosis perforans serpisinosa was made. Treatment with topical 0.05% tretinoin application for 6 months resulted in no improvement.

A clinicopathologic study of thirty cases of acquired perforating dermatosis in Korea.

BACKGROUND: Acquired perforating dermatosis (APD) is histopathologically characterized by transepidermal elimination of materials from the upper dermis. APD can be divided into four diseases: Kyrle's disease, perforating folliculitis, elastosis perforans serpiginosa, and reactive perforating collagenosis. APD is usually associated with systemic diseases, especially diabetes mellitus or chronic renal failure. So far, there have only been a few Korean studies of APD, which have a limited number of patients. OBJECTIVE: The aim of this study is to evaluate the clinical and histopathologic characteristics of 30 cases of APD and to examine the association with systemic diseases. METHODS: We retrospectively reviewed the medical records and biopsy specimens of 30 patients who were diagnosed with APD. RESULTS: The mean age was 55.5 years, and the average duration of the lesion was 7.8 months. The lower extremities (73.3%) were the most frequently occurring sites of the lesion. Twenty-five patients (83.3%) had pruritus, and Koebner's phenomenon was present in 11 patients. Patients of 63.3% had at least one systemic disease. Diabetes mellitus (n=17, 56.7%) and chronic renal failure (n=10, 33.3%) were the most commonly associated conditions. Most patients received topical steroids (93.3%) and antihistamines (80.0%). The most common histopathologic type was reactive perforating collagenosis (n=23, 73.3%). CONCLUSION: In this study, most patients had a systemic association to the diseases. Therefore, we suggest that further evaluation is necessary for patients who present with APD. This includes reviewing patient's comprehensive past medical history, clinical exam, and additional diagnostic testing to check for the possibility of associated systemic diseases.

Tacalcitol in the treatment of acquired perforating collagenosis.

Acquired perforating collagenosis (APC) is a rare perforating dermatosis characterized by transepidermal collagen elimination. We describe a 65-year-old patient, with long-standing type 2 diabetes mellitus and a 2-year history of itchy hyperkeratotic nodules situated on the back, who was subsequently diagnosed with APC. Treatment included topical corticosteroids and antihistamines, without improvement of the lesions. However, therapy with topical tacalcitol administered for 2 months produced a significant response leading to complete remission of APC.

Elastosis Perforans Serpiginosa in a patient with Down syndrome treated with imiquimod 5% cream.

Elastosis Perforans Serpiginosa (EPS) is a rare skin disease characterised by hyperkeratotic papules, transepidermal elimination of abnormal elastic fibres, and focal dermal elastosis. The aetiology is unknown, but an association with underlying systemic disorders, including Down syndrome has been described. Treatment is often difficult. A 45-year old man with Down syndrome presented with symmetrical annular elements on forearms and femora. The elements were erythematous with atrophic hypopigmented central healing and peripherally, infiltrated keratotic papules with desquamation. A punch biopsy showed the classical histopathologic features of EPS. We found no clinical signs of cerebrovascular or cardiovascular disease. We initiated topical therapy with imiquimod 5% cream once a day for 6 weeks followed by 3 times weekly for 4 weeks to a single element. As regression of EPS was observed and the patient tolerated the therapy well, treatment of other lesions was commenced, and further regression was seen.