Fine wrinkles on the neck, chest, axilla, trunk, back, and upper arms

Mid-dermal elastolysis (MDE) is a rare acquired elastic tissue disorder that is characterized clinically by well-circumscribed patches and plaques of fine wrinkles and histopathologically by focal loss of elastic fibers in the mid-dermis.1,2 MDE frequently manifests on the trunk and...

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Mid-dermal elastolysis (MDE) is a rare acquired elastic tissue disorder that is characterized clinically by well-circumscribed patches and plaques of fine wrinkles and histopathologically by focal loss of elastic fibers in the mid-dermis.^1,2 MDE frequently manifests on the trunk and proximal extremities. The clinical appearance of MDE is variable and may include perifollicular papular protrusions and inflammatory skin changes. There is a significant sex predominance observed in MDE; most cases are in white women of reproductive age, and the median age of onset is 34 years.² MDE is exceedingly rare; fewer than 100 cases have been reported in the literature since its first description in 1977.

MDE is a chronic condition in which the only clinical manifestation is fine wrinkling that gives the skin a prematurely aged appearance. MDE is categorized into types according to the observable skin changes: well-circumscribed patches of fine wrinkles arranged parallel to skin cleavage lines (type I), small soft papular lesions composed of perifollicular protrusions (type II), or persistent reticular erythema with wrinkling (type III).^2,3 The most frequently affected sites are the trunk, neck, and proximal aspects of the extremities; the face and hands are notably spared of wrinkling.^2,3 Most patients with MDE present with asymptomatic, well-demarcated, symmetrically arranged lesions of fine wrinkling that follow Blaschko lines, a normally invisible pattern that represents pathways of epidermal cell migration during development.^1,2

 

The size of the lesions can vary from a few centimeters in diameter to much larger, diffuse areas that involve the entire back. The affected skin generally lacks pigmentary changes, erythema, scaling, induration, atrophy, and telangiectasia.^3,4 Patients typically do not experience any itching, burning, or pain, although some report mild erythema. MDE appears to be a localized cutaneous disorder that has no associated systemic involvement. Once the lesions appear, they remain relatively stable in morphology for life.

Sporadic cases have been reported in which the onset of MDE is preceded or accompanied by an inflammatory dermatosis; for most patients, however, there is no history of skin disorders. Reported associations include urticaria, atopic dermatitis, granuloma annulare, pityriasis rosea, Sweet syndrome, phototoxic dermatitis, and guttate psoriasis.^2,4 Autoimmune diseases such as Hashimoto thyroiditis, Grave disease, lupus erythematosus, and rheumatoid arthritis have also been described in patients with MDE.⁴

The pathognomonic feature of MDE is the characteristic histopathologic finding of a complete band-like loss of elastic fibers in the mid-dermis of affected skin, which can be visualized by elastic tissue stains.^1,2 MDE is characterized by an absence of inflammatory infiltrate, but a mild perivascular infiltrate is sometimes detected in the dermis. The elastolysis is localized only to the mid-dermis, with preservation of normal elastic tissue in the superficial papillary dermis, in the reticular dermis, and along adjacent hair follicles.¹

The precise pathogenesis of MDE remains unknown, although several mechanisms have been proposed to explain its etiology. Recent data suggest that an altered balance between matrix metalloproteinases (MMPs; mainly MMP-2 and MMP-9) and proteolytic inhibitors results in increased degradation of extracellular matrix proteins and elastic fibers.³ Other possible mechanisms include defects in elastic fiber synthesis, autoimmunity against elastic fibers, and damage to elastic fibers via unregulated release of elastase by inflammatory cells.¹ There is no known genetic predisposition for MDE.

Ultraviolet light exposure has been suggested as a contributing factor in the development of MDE lesions, because approximately half of patients with MDE report noticing the appearance of fine wrinkles following prolonged sun exposure.³ There is evidence that ultraviolet light induces MMPs, resulting in increased destruction of elastic fibers. However, MDE does not manifest in a photodistribution, notably sparing the face and dorsum of the hands. In addition, the typical histologic features of ultraviolet-induced damage are not consistently observed in MDE, thereby suggesting that sun exposure may be contributory but not causative of the disease process.

The differential diagnosis of MDE should include closely related dermatologic disorders such as anetoderma, perifollicular elastolysis, Marshall syndrome (postinflammatory elastolysis and cutis laxa), and pseudoxanthoma elasticum-like papillary dermal elastolysis. Anetoderma is a disorder characterized by elastolysis in any part of the dermis and herniation of subcutaneous tissue upon palpation. It is similar to MDE in its distribution on the trunk and proximal extremities, but is characterized by small, soft macules and papules instead of fine wrinkles.^1,2 Perifollicular elastolysis is caused by Staphylococcus epidermidis and can be distinguished from MDE by the selective loss of elastic fibers surrounding hair follicles.¹ Marshall syndrome is characterized by an inflammatory phase, consisting of an urticarial eruption with malaise and fever, followed by acute destruction of elastic tissue that results in atrophy and severe disfigurement.^1,2 It is distinguished from MDE by its occurrence in children, elastolysis pattern, and face involvement. Pseudoxanthoma elasticum-like papillary dermal elastolysis clinically manifests as yellow nonfollicular cobblestone-appearing papules that may coalesce into large plaques on the neck, flexural forearms, and the axillae.²

No definitive therapy exists for MDE. Current treatment options are empiric and reports of improvement of the lesions are anecdotal. Pharmacologic agents such as vitamin E, clofazimine, oral and topical corticosteroids, colchicine, and chloroquine have been used with little to no benefit.⁴ Topical tretinoin has been reported to improve the appearance of wrinkles, but does not alter the course of the disease.

For the patient in the vignette, a punch biopsy of the skin revealed classic band-like loss of elastic fibers in the mid-dermis. The patient was not significantly bothered by the appearance of his skin and decided not to treat his MDE.

Julie Nguyen, BS, is a medical student and Maura Holcomb, MD, is a dermatology resident at Baylor College of Medicine in Houston.

References

1. Maari C, Powell J. Anetoderma and other atrophic disorders of the skin. In: Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ, Wolff K. Fitzpatrick’s Dermatology in General Medicine. 8th ed. New York, NY: McGraw-Hill; 2012:718-723.
2. Gambichler T. Mid-dermal elastolysis revisited. Arch Dermatol Res. 2010;302(2):85-93.
3. Patroi I, Annessi G, Girolomoni G. Mid-dermal elastolysis: a clinical, histologic, and immunohistochemical study of 11 patients. J Am Acad Dermatol. 2003;48(6):846-851.
4. Lewis KG, Bercovitch L, Dill SW, Robinson-Bostom L. Acquired disorders of elastic tissue: Part II. Decreased elastic tissue. J Am Acad Dermatol. 2004;51(2):165-185.