Elastosis perforans serpiginosa L87.20

Elastosis perforans serpiginosa is a rare skin disease of unknown etiology characterized by transepidermal shedding of abnormal elastic fibers. About 25% of cases are associated with an underlying systemic disease.

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Both autosomal dominant and autosomal recessive inheritance patterns have been described.

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

The first symptoms can already appear in childhood.

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

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

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

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.

Granuloma anulare perforans:

Hyperkeratosis follicularis et parafollicularis in cutem penetrans (M.Kyrle) - its existence is often doubted!

Reactive perforating collagenosis: Rare chronic skin disease characterized by transepidermal elimination of collagen and/or elastin by the skin. In the acquired (acquired collagenosis) form occurring in adults, it is often associated with diabetes mellitus and/or chronic end-stage renal failure. No serpiginous or anular formations. Hsitology is diagnostic.

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

Alternative: Excision in toto or curettage of cosmetically disturbing foci.

Alternative: Success with dye laser has been described in individual cases. Trial cryosurgery.

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

Note: Caution with aggressive invasive forms of therapy, as keloids often develop!

Usually progression over many years. Spontaneous regression or keloid formation possible.

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