Elastolysis mediodermal L90.9

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 22.09.2022

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Synonym(s)

Elastolysis acquisita mediodermalis; MDE; Mediodermal Elastolysis; Mid-dermal elastolysis

History
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Shelly and Wood, 1977

Definition
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Rare, etiologically unexplained skin disease caused by an acquired loss of elastic fibrous material in the mid-dermis (mid-dermal). Clinically, the disease is characterized by the localized or disseminated appearance of soft, skin-colored crinkly plaques. The disease may also be characterized by perifollicular protrusions.

Etiopathogenesis
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Postinflammatory and immunogenic imbalances in the cutaneous elastin metabolism with increased elastolytic activity are discussed.

Manifestation
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Mostly observed in middle-aged women from North America and Central Europe. No familiarity.

Localization
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trunk, proximal extremities, face

Clinical features
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Sharply defined plaques with fine puckering as well as peau d'orange-like perifollicular protrusions on the trunk and proximal extremities, partly also discrete, laminar erythema.

The clinical picture can be divided into 3 different appearances:

  • Type 1: circumscribed roundish or oval lesions with fine surface puckering
  • Type 2: Perfollicular protrusions
  • Type3: reticular erythema (rare: overlap with type1/type2 is possible)

Histology
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Selective loss of elastic fibers in the mid dermis, partial lymphohistiocytic infiltrates, elastophagocytosis, and multinucleated giant cells with granuloma formation. Ultrastructurally, multinucleated giant cells were found with phagocytosed elastic material.

In medio-dermal elastolysis, in contrast to anetoderma, the loss of elastic fibers is limited to the middle dermis.

Immunohistologically, a reduced expression of fibulin 4 and fibulin 5 is found (proteins which are important in the processing of elastic fibers - see also Cutis laxa).

Differential diagnosis
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Anetoderma (see also the differential diagnostic explanations there).

Anular elastolytic giant cell granuloma

Cutis laxa

Striae cutis distensae

Therapy
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No effective therapy known. Sun protection is recommended.

Progression/forecast
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After slow progression irreversible, atrophic terminal stage.

Case report(s)
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A 45-year-old woman noticed circumscribed, unusual, wrinkled skin areas on the arms for 2 years. later, the wrinkling spread to the trunk, back, and proximal portions of the legs. Except for occasional pruritus, skin findings were asymptomatic, and there were no previous erythema.

Medical history was completely unremarkable; she had not traveled recently. The pateinten was athletic, very active outdoors, and often exercised without sunscreen. She was not taking any medications, including oral contraceptives, and had no known allergies.

Physical examination revealed extensive, slightly bulging, skin-colored, wrinkled papules and plaques. The changes were most apparent on the abdomen, trunk, and proximal arms and legs, whereas the face and hands were spared.

Histologically, rarefaction of the elastic fiber network was demonstrated. No inflammatory changes.

Literature
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  1. Adams BB, Mutasim DF et al (2003) Colocalization of granuloma annulare and mid-dermal elastolysis. J Am Acad Dermatol 48: S25-27.
  2. Gambichler T et al (1999) Mediodermal elastolysis - Quantitative evaluation of 44 cases from the literature. Act Dermatol 25: 55-59
  3. Gambichler T, Breuckmann F, Kreuter A, Boms S, Altmeyer P, Stucker M (2004) Immunohistochemical investigation of mid-dermal elastolysis. Clin Exp Dermatol 29: 192-195
  4. Gambichler T et al.(2020) A Brief Literature Update on Mid-dermal Elastolysis with an Emphasis on Pathogenetic and Therapeutic Aspects. J Clin Aesthet Dermatol 13:E53-E58.
  5. Hardin J et al (2015) Mid-dermal elastolysis: A female-centric disease; case report and updated review of the literature. International Journal of Women's Dermatology 1(3):126-130.
  6. Patroi I et al (2003) Mid-dermal elastolysis: a clinical, histologic, and immunohistochemical study of 11 patients. J Am Acad Dermatol 48: 846-851
  7. Rothfleisch JE (2001) Mid-dermal elastolysis. Dermatol Online J 7: 15
  8. Rudolph RI (1990) Mid-dermal elastolysis. J Am Acad Dermatol 22: 203-206.
  9. Shelly WB, Wood MG (1977) Wrinkles due to idiopathic loss of mid-dermal elastic tissue. Br J Dermatol 97: 441-445.
  10. Streams BN (2003) Mid-dermal elastolysis. Cutis 71: 312-314

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