Elastolysis

Elastolysis refers to the localized or generalized breakdown or loss of elastic fibers in connective tissue due to enzymatic (e.g., by activatedproteases) or inflammatory-degenerative processes. In the skin, elastolytic processes primarily affect the elastic fiber network of the dermis. Histologically, there is a reduction, fragmentation, or complete absence of elastic fibers; detection is performed using elastin stains, such as the Verhoeff–Van Gieson, orcein, or resorcinol-fuchsin stains. Cutaneous elastolysis may be a monogtoric sign of a systemic disease.

Anetoderma: Well-defined areas of loose or sagging skin that can sometimes be indented like a “buttonhole” upon palpation.

Pseudoxanthoma elasticum-like syndrome: Primarily women over 60 years of age; affects the lateral neck and nape; no systemic changes.  

Mid-dermal elastolysis: Extensive, finely wrinkled skin with a prematurely aged appearance, primarily on the trunk and proximal extremities; occasionally presents with perifollicular papular lesions. Review article

Cutis laxa: Generalized loose, sagging skin with poor elasticity; hereditary or acquired. In systemic forms, blood vessels and the lungs may also be affected.

Post-inflammatory elastolysis: Loss of elastic fibers following inflammatory skin lesions, with subsequent skin laxity or atrophy. May be associated with systemic involvement, e.g., aortitis (Christensen CC et al. 1983).

Perifollicular elastolysis: Small, follicularly arranged, flaccid, or sunken skin lesions resulting from predominantly perifollicular elastin loss.

Granulomatous slack skin: A rare variant of cutaneous T-cell lymphoma characterized by large, slack skin folds, particularly in the flexural areas.

Papular elastorrhexis(elastorrhexis, papular): Multiple small, pale, non-follicular papules, primarily on the trunk; histologically: fragmentation and reduction of elastic fibers.

Clinically, the spectrum thus ranges from localized flaccid areas to fine skin wrinkling to generalized cutis laxa. A distinction must be made between clinical presentations based on acquired elastolysis and those based on hereditary disorders of elastogenesis or elastic fiber disorders:

• Actinic granuloma: UV-associated destruction of elastic fibers with elastophagocytosis. Annular plaques on chronically sun-damaged skin
• Anetoderma: Focal loss of elastic fibers, primarily in the upper and middle dermis. Well-defined, flaccid, sack-like protrusions or sunken areas of skin (door-knob phenomenon)
• Annular elastolytic giant cell granuloma: Giant cell reaction with elastophagocytosis and central loss of elastic fibers. Ring-shaped plaques, frequently on sun-exposed skin.
• Cutis laxa syndromes: Hereditary cutis laxa syndromes are often classified under the umbrella term “generalized elastolysis.” From a pathogenetic perspective, increased degradation is not always present. Rather, it often involves a genetically determined disorder of elastin synthesis, microfibrils, or the assembly of elastic fibers. Genes involved include:
  • Acquired cutis laxa: Diffuse dermal degradation of elastic fibers. Generalized or localized loose, sagging skin.
• Granulomatous slack skin: A clinical variant of cutaneous T-cell lymphoma. Loss of elastin due to granulomatous infiltrates and giant cells. Flaccid, pendulous skin folds, particularly in intertriginous areas;
• Mid-dermal elastolysis (Elastolysis acquisita mediodermalis) (Types I–III): Described to date exclusively in Caucasians. >50 years of age; more common in women than in men; band-like, selective loss of elastic fibers in the mid-dermis. Finely wrinkled, often symmetrical areas on the trunk and upper arms
• Papular elastorrhexis (papular elastorrhexis): Multiple small, pale, non-follicular papules, primarily on the trunk; histologically: fragmentation and reduction of elastic fibers.
• Perifollicular elastolysis: Small, follicularly arranged, flaccid or sunken skin lesions resulting from a predominantly perifollicular loss of the elastic fiber system.
• Piezogenic nodules: found exclusively on the medial or lateral edge of the foot. Pressure-induced focal degeneration of elastic fibers with circumscribed herniation of the subcutaneous adipose tissue.
• Post-inflammatory elastolysis: Loss of elastic fibers following inflammatory skin lesions, with subsequent focal loss of the elastic fiber system. Clinically, focal skin laxity or atrophy is observed. May be associated with systemic involvement, e.g., aortitis (Christensen CC et al. 1983).
• Pseudoxanthoma elasticum-like papillary dermal elastolysis (pseudoxanthoma elasticum-like syndrome; white fibrous papulosis of the neck): Loss of elastic fibers in the papillary dermis. Yellowish to skin-colored papules; affects individuals over 60 years of age; more common in women than in men; primarily located on the lateral aspects of the neck.

Histologically, there is a reduction, fragmentation, or complete loss of elastic fibers, which can be detected using special elastic fiber stains.

A distinction must be made from elastosis, which involves a qualitative and quantitative change or accumulation of abnormal elastic material. Another term to note is elastophagocytosis.

Another term that needs to be distinguished is “elastorrhexis,” which is no longer commonly used today.

1. Christensen CC et al. (1983) Postinflammatory elastolysis and cutis laxa: a case report of a patient with aortitis. Pediatr Pathol 1:199-210.
2. Lewis PG et al. (1990) Postinflammatory elastolysis and cutis laxa. A case report. J Am Acad Dermatol 22:40–48.
3. Tronnier M (2018) Cutaneous disorders characterized by elastolysis or loss of elastic tissue. J Dtsch Dermatol Ges 16:183–191.
4. Yamashita A et al. (2024) Acquired cutis laxa secondary to acute generalized exanthematous pustulosis: A case report and mini-review of the literature. J Dermatol 51:287–293.