Elastosis perforans serpiginosa L87.20

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 22.09.2022

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Synonym(s)

Elastoma intrapapillary perforans verruciforme; Elastomer perforating; élastose perforante serpigineuse de Lutz-Miescher; Elastosis perforans; EPS; Keratosis follicularis serpiginosa; Lutz-Miescher disease; perforating elastomer; perforating elastosis; perforating serpiginous elastosis

History
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Lutz, 1953; Miescher, 1955

Definition
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Rare, circumscribed connective tissue disorder with transepidermal elimination of elastic fibers.

Occurrence/Epidemiology
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m>w

Etiopathogenesis
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Both autosomal dominant and autosomal recessive inheritances have been described.

Isolated occurrence, also in Ehlers-Danlos syndrome, Marfan syndrome, pseudoxanthoma elasticum, Rothmund syndrome or Thomson syndrome, Down syndrome, osteogenesis imperfecta, in renal dysfunction or D-penicillamine-induced(long-term therapy in Wilson's disease and cystinuria).

Manifestation
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The first symptoms can already appear in childhood.

Average first manifestation at age 55. Duration of illness 7-8 months .

Localization
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Mainly lower extremities, more rarely on the neck, neck, arms (elbow area).

Clinical features
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Onset with single 0.2-0.5 cm, red, firm, smooth or slightly keratotic, asymptomatic (mild itching possible) papules that aggregate into anular or serpiginous plaques with hyperkeratotic overlays. Subsequently, the clinical picture is characterized by peripheral progression and central healing tendency, so that anular formations (up to 5.0-7.0 cm in diameter) are even more accentuated (see Fig.).

Histology
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Homogeneous elastotic material in the papillary body that is eliminated transepidermally. The surrounding infiltrate corresponds to that of a foreign body granuloma.

Histopathologically transepidermal elimination of morphologically and biochemically altered elastic fibers and necrotic cells of dermal connective tissue. Reactive proliferation of the epithelium with acanthosis, hyperkeratosis and inclusion of elastotic material.

Differential diagnosis
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Granuloma anulare perforans

Hyperkeratosis follicularis et parafollicularis in cutem penetrans (M.Kyrle)

Reactive perforating collagenosis

Therapy
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Local therapy with Vit. D3 analogues(Tacalcitol, Calcipotriol) is described as successful (multiple publications)

Alternatively: excision in toto or curettage of cosmetically disturbing foci.

Alternative: Successes with dye laser are described in individual cases. Experimental cryosurgery.

Alternative: Glucocorticoids under occlusive conditions or intralesional with triamcinolone acetonide (Volon A 10 crystal suspension diluted 1:4 with local anesthetic like mepivacaine).

Remark: Caution with aggressive forms of therapy, as keloids are often formed!

Progression/forecast
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Usually years of progression. Spontaneous regression or keloid formation possible.

Literature
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  1. Campanati A et al. (2014) Elastosis perforans serpiginosa: a case successfully treated with intralesional steroids and topical allium cepa-allantoin-pentaglycan gel. Acta Dermatovenerol Alp Pannonica Adriat 23:39-41
  2. Deguti MM et al (2002) Elastosis perforans serpiginosa secondary to D-penicillamine treatment in a Wilson's disease patient. Am J Gastroenterol 97: 2153-2154.
  3. De Pasquale R et al (2002) Elastosis perforans serpiginosa in an adult with Down's syndrome: report of a case with symmetrical localized involvement. J Eur Acad Dermatol Venereol 16: 387-389.
  4. Escribano-Stablé JC et al (2014) Tacalcitol in the treatment of acquired perforating collagenosis. Case Rep Dermatol 6:69-73
  5. Fischer H (1927) Peculiar anatomical findings in a skin disease. Zbl Haut-Geschlechtskrkh 220: 599.
  6. Heusinkveld LE et al (2021) Penicillamine-induced elastosis perforans serpiginosa and pseudo-pseudoxanthoma elasticum. Int J Womens Dermatol 7: 822-823.
  7. Lutz W (1953) Keratosis follicularis serpiginosa. Dermatologica 106: 318-320.
  8. Kim SW et al.(2014) A clinicopathologic study of thirty cases of acquired perforating dermatosis in Korea. Ann Dermatol 26:162-171
  9. Lother AS et al (2013) Chronic annular lesions of the cheeks. Elastosis perforans serpiginosa (EPS). Int J Dermatol 52:649-650
  10. Miescher G (1955) Elastoma intrapapillare perforans verruciforme. Dermatologica 110: 254-266
  11. Valenzuela-Ubiña S et al (2020) Elastosis perforans serpiginosa induced by d-penicillamine treated with cyclosporine and allopurinol. Dermatol Ther 33:e13692.
  12. Wilhem KP et al (1994) Penicillamine-induced elastosis perforans serpiginosa. Dermatol Ther 45: 45-47
  13. Wilms A et al (1997) Elastosis perforans serpiginosa in Down syndrome. Dermatologist 48: 923-925

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 22.09.2022