Elastosis perforans serpiginosa (EPS) is a rare disorder classified as a primary perforating dermatosis. That group of diseases also includes. Four types of elastosis perforans serpiginosa (EPS) have been described in literature: 1) idiopathic EPS, 2) reactive perforating elastosis. Elastosis perforans serpiginosa (EPS) is a rare dermatologic condition where connective and elastic tissues are viewed as foreign objects and subsequently.
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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 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. Elastosis perforans serpiginosa EPS is a rare skin condition characterized by hyperkeratotic papules, transepidermal elimination of abnormal elastic fibers, and focal dermal elastosis. Since it was first described by Lutz inEPS is one of four classic primary perforating disorders along with reactive perforating collagenosis, perforating folliculitis, and Kyrle disease 1.
We report an additional case of this rare cutaneous condition and review EPS cases described in the Korean literature. A summary of the 5 cases of elastosis perforance serpiginosa reported in the Korean literature including our case. A year-old healthy man visited Gangnam Severance Hospital with hyperkeratotic papules on his anterior neck. These papules first appeared 2 years ago and slowly increased in number. Over time, the skin lesions had spread peripherally with some central clearing.
The patient stated that perfoans skin lesions had developed after a skin injury. Previous treatments with topical antifungal and topical steroid were ineffective. The patient’s medical and family history was also negative for EPS or any other connective tissue diseases. On examination, multiple crusted erythematous papules 2 to 5 mm in size were arranged in an annular and serpiginous pattern Fig. A potassium hydroxide preparation obtained from the skin lesion was negative for fungal elements.
A punch biopsy specimen revealed focal epidermal hyperplasia with the formation of a narrow vertical transepidermal channel containing nuclear debris and eosinophilic degenerated elastic fibers Fig. The amount and thickness of the elastic fibers in the papillary dermis had increased markedly.
It looked like entering the canals toward the outside of the skin Fig. The papillary dermis and superficial reticular dermis were infiltrated by numerous inflammatory cells, including lymphocytes and histiocytes Fig. Verhoeff-van Gieson staining showed a markedly increased number of altered elastic fibers forming dense clumps in the upper dermis Fig.
Gomori methenamine silver stain and D-PAS stain were both negative for fungal elements. A diagnosis of EPS was made based on these clinical and histopathological findings. Despite treatment with topical 0. Perforating diseases are histologically characterized by transepidermal elimination of dermal substances. Elastic fibers perforate through the epidermis in patients with EPS.
Table 1 summarizes the clinical data from four cases of EPS in the Korean literature in addition to our case. EPS is more commonly reported in men and usually occurs during childhood or early adulthood 1. The data from the five Korean elqstosis with EPS further support these findings: Familial cases of EPS have also been reported, with the mode of inheritance suggested to be autosomal dominant with variable trait expressivity 67.
All five Korean patients including our patient presented with skin lesions on the neck, the most frequently affected EPS site. The most prominent histopathological findings in patients with EPS are localized segments of hyperplastic epidermis where basophilic nuclear debris and brightly eosinophilic fragmented elastic fibers are being eliminated through transepidermal channels that extend upward from the dermis in straight or corkscrew patterns 1. In addition to a marked increase in the amount and thickness of papillary dermal elastic tissue, a chronic inflammatory elaztosis containing lymphocytes, macrophages, or multinucleated giant cells may be present in the dermis at the sites of perforation.
While the exact pathogenic zerpiginosa of EPS remains unknown, associations with various underlying systemic conditions and connective tissue disorders have been reported in the literature, including Down’s syndrome, osteogenesis pervorans, scleroderma, acrogeria, Ehlers-Danlos syndrome type IV vascular typeMarfan syndrome, Rothmund-Thomson syndrome, cutis laxa, diabetes mellitus, perforating folliculitis, and chronic renal failure 89.
However, it remains unclear whether the relationship between these conditions and EPS is causal or coincidental, as EPS also commonly occurs in individuals without any underlying disease. Notably, two of the five reported Korean patients with EPS had comorbid pseudoxanthoma elasticum.
Tazarotene Is an Effective Therapy for Elastosis Perforans Serpiginosa
Isolated reports also indicate a possible association between EPS and Wilson’s disease sserpiginosa cystinuria, particularly in patients on long-term penicillamine therapy 510 – pwrforans Such a case of EPS associated with penicillamine therapy was reported in the Korean literature perfoans. In these cases, elastin synthesis may be affected either directly by penicillamine or via a localized copper deficiency, ultimately inducing the production of abnormal elastic fibers that are transepidermally eliminated from the dermis.
In cases of EPS associated with penicillamine therapy, the characteristic skin lesions typically develop within the first year of treatment and can be histologically distinguished from the other forms of EPS by the presence of calcium deposits in transepidermal elastic fibers 7. While calcinosis cutis, granuloma annulare, Tinea corporisannular sarcoidosis, and porokeratosis of Mibelli may present with clinically similar features, these disorders are easily distinguished by histological examination.
Moreover, even though transepidermal elimination can occur in any of the three other perforating diseases, the elastic tissue changes present in EPS are virtually pathognomonic. Although lesions elasttosis spontaneously resolve, they often persist ser;iginosa longer periods. Numerous treatment modalities have been described, including dry ice 8cellophane tape stripping 813electrodessication and curettage 14cryotherapy 13 – 15intralesional and topical corticosteroid therapy 1516topical calcipotriol 14topical tretinoin 141517oral isotretinoin 1415topical tazarotene 15topical imiquimod 18topical glycolic or salicylic acid therapy 14 elasfosis, 17narrow band ultraviolet B radiation 14pulsed dye laser, Er: YAG laser 16and carbon dioxide laser therapy 15 However, no ‘gold standard’ therapy exists among these treatment modalities.
Treatment with topical tretinoin including topical 0.
Laser treatment, including carbon dioxide laser and Er: YAG laser, are reported to induce complete clearing of EPS lesions, but another perfforans showed only mild improvement of EPS lesions and subtle atrophic scarring after carbon dioxide laser treatment The pinhole method using a carbon dioxide laser showed complete clearing without post-treatment scarring Topical imiquimod and topical tazarotene therapy is reported to improve EPS skin lesions 15 However, further studies are needed to evaluate the effectiveness of such topical therapy for treating EPS.
In addition, discontinuing penicillamine therapy does not guarantee preventing further development of EPS lesions perforanx patients undergoing penicillamine therapy 15 In our case, we chose treatment with topical 0. National Center for Biotechnology InformationU.
Journal List Ann Dermatol v. Published online Serpigginosa Find articles by Si-Hyung Lee. Find articles by Yuri Choi. Find articles by Soo-Chan Kim. Author information Article notes Copyright and License information Disclaimer.
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Elastosis Perforans Serpiginosa
Abstract Elastosis perforans serpiginosa is a rare skin disease characterized by transepidermal elimination of abnormal elastic fibers. Elastosis perforans serpiginosa, Therapeutics. Table 1 Elastosos summary of the 5 cases of elastosis perforance serpiginosa reported in the Korean literature including our case.
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Acquired disorders of elastic tissue: Increased elastic tissue and solar elastotic syndromes. J Am Acad Dermatol. A case of elastosis perforans serpiginosa. 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 with simultaneous onset in two sisters.
Familial elastosis perforans serpiginosa. Disorders of transepidermal elimination. D-penicillamine-induced elastosis perforans serpiginosa in a child with juvenile rheumatoid arthritis. Report of a case and review of the literature. Multiple side effects of penicillamine therapy in one patient with rheumatoid arthritis. Elastosis perforans serpiginosa and associated disorders. Tazarotene is an effective therapy for elastosis perforans serpiginosa.
Saxena M, Tope WD. Response of elastosis perforans serpiginosa to pulsed CO2, Er: YAG, and dye lasers. Treatment of elastosis perforans serpiginosa with the flashlamp pulsed dye laser. Imiquimod therapy for elastosis perforans serpiginosa. Treatment of elastosis perforans serpiginpsa with the pinhole method using a carbon dioxide laser. Elastosis perforans serpiginosa associated with pseudo-pseudoxanthoma elasticum during treatment of Wilson’s disease with penicillamine.