Case Of Elastosis Perforans Serpiginosa Research Articles

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.

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.

D-Penicillamine induced elastosis perforans serpiginosa with involvement of glans penis

Elastosis perforans serpiginosa (EPS) is an unusual perforating disorder characterized by extrusion of altered elastic fibers through the epidermis. Clinically, it presents as keratotic papules with a tendency for serpiginous or annular distribution that most commonly involves the sides of the neck and the back. However, involvement of the penis has rarely been reported. We present a case of EPS involving the neck, axilla, and glans penis in a 42-year-old man who had received long-term d -penicillamine treatment for Wilson disease. Skin biopsy revealed perforating channels containing numerous altered elastic fibers, with a characteristic “bramble brush” or “lumpy-bumpy” appearance as demonstrated by an elastin stain. The latter is thought to be pathognomonic for penicillamine-induced degenerative elastosis. These degenerative changes occurring in glans penis have rarely been described in the literature. Prompt recognition of the rare presentation could lead to early discontinuation of the offending drug, to prevent further sequelae.

A Case of Elastosis Perforans Serpiginosa Complicated with Down's Disease

A Case of Elastosis Perforans Serpiginosa Complicated with Down's Disease

A case of Elastosis perforans serpiginosa associated with Pseudoxanthoma elasticum.

A case of Elastosis perforans serpiginosa associated with Pseudoxanthoma elasticum.

Penicillamine-induced Elastosis Perforans Serpiginosa and Cutis Laxa in a Patient with Wilson's Disease

Elastosis perforans serpiginosa (EPS) is a rare reactive perforating dermatosis that is characterized by the transepidermal elimination of abnormal elastic fibers. Penicillamine, which is one of the clear triggers for EPS, is a heavy metal chelator that is primarily used for disorders such as cystinuria and Wilson's disease. It may cause alterations in the dermal elastic tissue such as pseudo-pseudoxanthoma elasticum, acquired cutis laxa, EPS and anetoderma. Herein we present a case of cutis laxa and EPS in a 34-year-old man who was previously on a long-term, high-dose of penicillamine for Wilson's disease. The combination of EPS and cutis laxa induced by penicillamine has rarely been reported and we report the first such case in Korea.

Elastosis Perforans Serpignosa: Treatment with Liquid Nitrogen Cryotherapy and Review of the Literature

Elastosis perforans serpiginosa (EPS) is a rare skin disease in which abnormal elastic tissue fibers, other connective tissue elements, and cellular debris are expelled from the papillary dermis via transepithelial elimination. It is characterized by an eruption of small grouped hyperkeratotic papules in a serpiginous arrangement. This condition presents a therapeutic challenge as many treatments have been reported with inconsistent efficacy. This article reports a case of a 13-year-old male who presented to our outpatient clinic with a 1-year history of facial lesions showing multiple annular keratotic plaques with slight central atrophy. We report a case of EPS treated successfully with liquid nitrogen cryotherapy. Liquid nitrogen cryotherapy is well tolerated, with few side effects, and can be considered in the management of EPS.

Expression of the 67-kDa elastin receptor in perforating skin disorders.

Perforating skin dermatoses include elastosis perforans serpiginosa (EPS), reactive perforating collagenosis, Kyrle's disease and perforating folliculitis. In addition to these four diseases, an acquired form of perforating dermatosis associated with diabetes mellitus and/or chronic renal failure has been reported for which the term acquired perforating dermatosis (APD) was proposed. The molecular mechanism of transepidermal elimination of dermal components in perforating skin dermatoses remains unclear. We recently demonstrated that the 67-kDa elastin receptor can be detected in the epidermis eliminating altered elastic fibres in EPS, suggesting that the elastin-keratinocyte interaction may play a role in transepidermal elimination in EPS. To determine whether the 67-kDa elastin receptor is involved in other perforating diseases. Paraffin-embedded skin specimens from new cases of EPS (n = 2), APD (n = 15) and perforating granuloma annulare (PGA; n = 2) were studied immunohistochemically using a specific antibody to the 67-kDa elastin receptor. In one case of EPS, two different sites from a single lesion, a central atrophic area and a peripheral keratotic area, were studied. Expression of the elastin receptor was detected in the epidermis surrounding the elastic materials in both cases of EPS. The elastin receptor was not detected in the central inactive area, whereas it was expressed strongly in the peripheral keratotic active area. The elastin receptor was also detected in three of 15 cases of APD in which a few elastic fibres were found in the eliminated dermal materials. In one case of APD, the elastin receptor was not detected in spite of the presence of a few elastic fibres in the eliminated materials. The elastin receptor was not detected in either case of PGA. Expression of the elastin receptor in EPS was seen in both cases studied and was dependent on the stage of the lesion. Expression of the elastin receptor in APD appeared to be related to the amount of elastic fibres in the eliminated materials. Thus, expression of the elastin receptor in perforating skin disorders may depend on the stage of the lesion and/or the content of elastic fibres in the dermal materials being eliminated.

Acquired perforating dermatosis in diabetes mellitus: An unusual case

A case of elastosis perforans serpiginosa in a patient who presented with insulin-dependent diabetes mellitus secondary to pancreatic insufficiency in a background of common variable immunodeficiency and endocrinopathy, as evidenced by pernicious anaemia and growth hormone deficiency, is described. In acquired perforating dermatosis occurring in patients with diabetes or renal failure, there is a spectrum of changes that may show an overlap of histological features of the four classic perforating diseases. The biopsy changes of the patient described in the present study most closely resembled those of elastosis perforans serpiginosa.

Elastosis Perforans Serpiginosa in a Patient With Renal Disease

We encountered a case of elastosis perforans serpiginosa in a patient with coexisting renal disease, the first such reported association of which we are aware. The perforating disorders tend to have atypical clinical features when associated with renal disease. The exact pathogenic mechanism by which renal disease predisposes to a perforating disease remains to be elucidated but is likely to be related to minor trauma, which occurs in renal disease.

Ultrastructural and biochemical studies on a case of elastosis perforans serpiginosa.

Ultrastructural and biochemical studies on the collagen and elastin fibres of the dermis from a patient with Elastosis perforans serpiginosa are described. Qualitative and quantitative alterations on collagen and elastin are shown, which give some evidence for considering the disease as a connective tissue defect.

Elastosis Perforans Serpiginosa

Elastosis perforans serpiginosa (EPS), a rare disease, has been associated with mongolism and several connective tissue disorders, including osteogenesis imperfecta, pseudoxanthoma elasticum, Marfan syndrome, and Ehlers-Danlos syndrome. The first description of EPS was made by Lutz in 1953.¹Two years later, Miescher²presented a complete histopathologic study. The first cases of EPS in the United States were described by Hitch and Lund in 1959.³ To my knowledge, this is the third patient reported to have EPS coincident with osteogenesis imperfecta, which is a generalized disorder of connective tissue involving the fascia, ligaments, skin, sclera, tendons, inner ears, and bones, that typically results in the patient's having blue sclera and brittle bones.⁴ <h3>REPORT OF A CASE</h3> A 17-year-old boy had had blue sclerae since infancy. As a child, he had had several fractures, and x-ray films taken on each occasion were consistent with the diagnosis of osteogenesis

Elastosis perforans serpiginosa. Two cases involving the ear.

Elastosis perforans serpiginosa most frequently involves the nape and sides of the neck, but has been described involving the face, trunk, and upper extremities. To our knowledge, these are the first two reported cases of elastosis perforans serpiginosa in which the pinna was affected.

PSEUDOXANTHOMA ELASTICUM AND ELASTOSIS PERFORANS SERPIGINOSA.

This paper is intended to emphasize the significance of the association of elastosis perforans serpiginosa with heritable disorders of connective tissue. A case is reported in which a patient with pseudoxanthoma elasticum developed multiple lesions of elastosis perforans serpiginosa. A review of the literature revealed only two such cases previously reported. The cases of elastosis perforans serpiginosa previously reported in association with other diseases are summarized. The case is that of a 35-year-old Caucasian male with the skin changes of pseudoxanthoma elasticum present since childhood. A sister also has this disease. Over the past seven years he developed multiple lesions of elastosis perforans serpiginosa which were located in the areas most affected by pseudoxanthoma elasticum. Seventeen such lesions were present at the time of examination. Histologic studies demonstrated the typical findings of both diseases.

ELASTOSIS PERFORANS SERPIGINOSA WITH OSTEOGENESIS IMPERFECTA

<h3>To the Editor:</h3> I would like to record that I have recently seen a case of elastosis perforans serpiginosa associated with osteogenesis imperfecta in a European boy aged 19 years. This is the second case to be recorded in the literature. The first case was described in theArchives of Dermatology, 89:342-344 (Feb) 1964, by Drs. William B. Reed and Joseph W. Pidgeon of California. Another interesting observation about my case is that the lesions were extensive and symmetrical. In theArchives of Dermatologyfor August, 1963, p 215, Dr. Warren L. Macaulay describes a case of elastosis perforans serpiginosa in which the lesions were also symmetrically disposed.

Elastosis perforans serpiginosa; report of a case.

The recent description of perforating elastosis by Hitch and Lund1has clarified a diagnostic problem of long standing. The following is a brief report of a case of elastosis perforans serpiginosa of 14 years' duration. Report of Case A white woman, age 22 years, a mongoloid, was first seen at the age of 8 years presenting a peculiar, nonpruritic ringed, papular eruption over the extensors of the arms, forearms, and lower extremities. Repeated mycologic studies were negative, but owing to the general resemblance of the eruption to tinea infection, it was treated as such by painting the lesions with tincture of iodine and spraying them with ethyl chloride. The patient was followed and treated in this manner from 1946 through 1949 with some success. She was seen by another dermatologist in 1950, and repeated mycology studies were negative (Fig. 1). A biopsy was done, and sections were submitted to