Are You Confident of the Diagnosis?

  • What you should be alert for in the history

The history is most likely to reveal an elderly or middle-aged patient with a past of significant long-term sun exposure; however anyone with a history of chronic exposure to ultraviolet (UV) radiation is at risk.

  • Characteristic findings on physical examination

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On physical exam, skin texture appears rough, thickened, and wrinkled, often with the presence of yellowish papules or plaques or overall yellow/sallow discoloration (Figure 1). The diagnosis is confirmed through light microscopy, which histologically shows a subepidermal grenz zone of horizontal collagen fibers (fragmented collagen fibers), with groups of “elastotic” deposits (fragmented elastic fibers) in the dermis.

Figure 1.

  • Expected results of diagnostic studies

Early on in actinic elastosis, histology shows elastic fiber hyperplasia, which progresses into fragmented, coarse, “elastotic” material (Figure 2). This elastotic material is composed of elastin and microfibrillar proteins, with a minimal degree of interstital collagen. This material was also found to contain amyloid P and vitronectin, with a small quantity of fibrillin (a glycoprotein).

Figure 2.

  • Diagnosis Confirmation

The differential diagnosis includes several actinic elastotic variants:

Milian’s citrine skin- wrinkled, leathery, and inelastic sun-exposed skin with a yellow hue and irregular pigmentation; the more atrophic form consists of prominent telangiectasias, hypertrophic sebaceous hyperplasia, and chronic solar erythema.

Striated beaded lines- linear groups of hypertrophic sebaceous glands and elastotic material which form parallel lines on the lateral neck and V-shaped area of chest (decolletage); juxtaclavicular beaded lines are hypertrophic sebaceous glands found over the clavicular area.

Favre-Racouchot syndrome- thickened yellow plaques and enlarged pilosebaceous orifices with comedones and kertainous cysts residing in the plaques; most often found on the periorbital and malar skin, but also seen on the nose, neck, and retroauricular area. With advanced photodamage, the elastotic plaques are studded with comedones that are presumed to be due to weakened structural support of the follicular unit yielding dilated ducts and retained keratin. Seen more frequently in males.

Actinic comedonal plaque- erythematous to blue nodular plaques; lesions most often occur on the upper extremities; histologically similar to Favre-Racouchot syndrome and is viewed as an uncommon ectopic form.

Cutis rhomboidalis nuchae- thickened, yellow, leathery skin on the posterolateral aspect of neck with etched in wrinkles; due to the repeated movement by underlying muscles yielding a geometric pattern; severe cases may also reveal the presence of small cysts and comedones.

Elastotic nodules of ear – pale, semi translucent, 4-6mm papules; located on the anterior crus of the antihelix, but can also be found on the helix, with pain mimicking chrondrodermatitis nodularis helicis. Lesions may be somewhat senstive to touch and are usually observed in elderly men with signs of solar damage. Histologically elastotic nodules do not affect underlying cartilage but rather degeneration and clumping of elastotic material.

Weathering nodules-often occur on the helix of the ear, are asymptomatic, show no signs of inflammation and histologically show cartilage metaplasia with fibrous tissue. Like elastotic nodules, weathering nodules tend to be seen in elderly men with signs of solar damage but histologically show cartilage metaplasia with fibrous tissue.

Diffuse elastoma of Dubreuilh- distinctly marginated, thickened yellow plaques most often seen on the face, neck, or chest; can occur singly or in groups.

Actinic granuloma- begins as flesh-colored or pink papules or nodules 1-6 cm in size, which enlarge to annular/serpiginous plaques with normal or atrophic centers; may resemble granuloma annulare in an elastotic skin type.

Solar elastotic bands of forearms- flesh-colored to yellow papulonodules merging into cordlike bands covering the dorsiflexural aspects of the forearms.

Keratoelastoidosis marginalis (Degenerative collagenous plaques)- occurring on sun-damaged hands of manual workers; distinguished from focal acral hyperkeratosis and acrokeratoelastoidosis (autosomal dominant condition with shiny papules at the periphery of palms and soles with extension to dorsolateral surfaces).

Digital papular calcific elastosis- histologically differentiated by degenerated elastic tissue and contained calcium.

Adult-onset colloid milium- flesh- to yellow-colored translucent small dermal papules occurring on sun-exposed areas of middle-aged adults; seen in refinery workers exposed to trauma and with phenols in gas oil; an association exists with high concentrations of hydroquinone bleaching creams and the development of ochronosis with pigmented colloid milium.

Who is at Risk for Developing this Disease?

Patients living in tropical climates, or individuals who spend many hours outside exposed to a great deal of sun (golfers, tennis players, farmers, sailors, landscapers, roofers); fair-skinned individuals are at greater risk.

What is the Cause of this Disease?

  • Etiology

The primary cause of actinic elastosis is a chronic and prolonged exposure to UV radiation, resulting in distinct histologic features. Actinic elastosis often presents histologically with elastin-like aggregate (elastotic) material in the dermis beneath the grenz zone. The subepidermal grenz zone is felt to be newly sythesized collagen that occurs as the elastotic material collects in the dermis, thus creating a crowding of the collagen fibers. The amount of elastotic material correlates to the relative amount of UVR skin exposure.

One of the earliest changes is elastic fiber hyperplasia. Later these fibers become coarse, twisted, and branched, forming dense masses that stain characteristically basophilic in the papillary and upper reticular dermis.

  • Pathophysiology

It is still controversial whether elastic material is formed from a degradative process or abnormal synthesis or both. It has been suggested that there maybe a “biphasic” nature of actinic elastosis: (1) an initial phase collection of normal elastic fibers in the papillary and reticular dermis, followed by (2) a destructive phase characterized by elastic fiber retention of basophilic stain (blue degeneration) with elastic tissue destruction and elastolysis becoming the final process.

Other investigators describe chronic heliodermatitis, which starts as an inflammatory infilitrate composed of perivenular lymphohistiocytes and several degranulated mast cells in proximity to fibroblasts. They theorize that mast cell–derived mediators in conjunction with surrounding cell released enzymes cause breakdown of collagen and elastic fibers yielding severe actinic elastosis. Thus, chronic inflammation caused by solar damage is the integral etiology in this degradative process.

Nonetheless, most studies confirm that the elastotic material is derived from elastic fibers and not collagen as demonstrated by the increased ratio of cross-linked elastic fibers to collagen in sun-exposed skin. However, the massive accumulation of elastotic material is mainly composed of elastin and microfibrillar proteins connected with fibronectin, amid a small amount of interstitial collagen. Interestingly, this elastotic material is also found to contain amyloid P and vitronectin along with some fibrillin, a glycoprotein constituent of elastic fibers.

Systemic Implications and Complications

To our knowledge, the systemic implications and complications would be similar to any UVR-induced photodamage to skin, namely metastatic squamous cell carcinoma or melanoma. Actinic elastosis is not pathognomonic for any systemic disorder, but rather a hallmark of significant photodamage.

One should examine the skin for other abnormalities from chronic sun exposure including actinic keratosis, squamous cell carcinoma, basal cell carcinoma, melanoma, and possibly dysplastic nevi. Recommendations would be judicious use of appropriate clothing, hats, sunscreens, and diligent sun avoidance.

Treatment Options

PREVENTION

Clothing, hats, sunscreens, and sun-avoidance.

MEDICAL

Topical retinoids and alpha hydroxy acids (AHAs); salicyclic acid/benzoyl peroxide may be helpful.

PHYSICAL

Dermabrasion, moderate to deep depth chemical peels, laser resurfacing (carbon dioxide, fractionated CO2, or erbium laser), Isolaz (vacuum for pore cleansing of open comedones), extractions for comedones.

Optimal Therapeutic Approach for this Disease

Start with a topical retinoid such as tretinoin cream 0.025% increasing gradually to 0.05% (evening) for the “rougher and tougher” skin. Brand names would include Refissa or Atralin. An AHA 10% (morning) can be administered up to three times a week. Increase the retinoid to every evening as tolerated and increase the AHA to 15%, then 20% as tolerated. Note that the maximum concentration of retinoids is 0.1% and the maximum concentration of AHA that can be comfortably used is 20%.

Anecdotally, AHAs of 30% has been tried, but stinging has been reported.

Intense microdermabrasion and light chemical peels can be effective, but due to their need for repetition, these treatments may be more costly, with an increased time to therapeutic improvement. However, patients may prefer such options due to no down time.

Quicker results may be found with the CO2 laser (expect at least 3 weeks of down time) and fractionated lasers (expect at least 1 week of down time). Hand pieces yielding deeper depths must be used.

Chemical peels of medium depth may be a cheaper option, but again might need repetition (expect 2 weeks of down time). Deep depth chemical peels, such as phenol peels, can be helpful, but due to the need for cardiac monitoring equipment, these peels are not as frequently employed as lasers.

Dermabrasion is another modality that is helpful and depth can be controlled. This option is often less favored over lasers because of heme splattering during treatments.

Photodynamic therapy involves the use of light plus a photosensitizer. It is used for preskin cancers such as AKs and for nonmelanoma skin cancers. It has also been used as an antiaging treatment to diminish lines and wrinkles. Its improvement of actinic elastolysis and acne scars has not been statistically measured to date, but there is a suggestion photographically of some improvement. However, when used in teenage patients, advanced education of the peeling and burning that is a natural sequelae of the treatment must be emphasized, and changes in lifestyle must be made to accomodate the recovery period.

There are studies that have documented improvement in antiaging and photoaging with Efudex (topical fluorouracil) and Aldara (topical imiqimod). These agents are typically used for preskin cancers, AKs and nonmelanoma skin cancers. The improvements show decreased fine lines and wrinkles and to some extent lentigines.

Patient Management

Encourage ongoing sun protection and sun avoidance. Have the patient examined twice a year; a helpful checkup regimen is in the spring and the fall to remind and reinforce judicial sun behavior and protection. Educate the patients on how to properly examine their own skin on a monthly basis. Stress to patients the importance of presenting back to the office in a timely fashion if any changes in their skin are noted.

Risks associated with retinoids and AHAs can be burning, stinging, redness, itching, peeling, flaking, dryness in the area being treated, as well as contact areas. Patients undergoing waxing or other hair removal processes should forgo retinoids and AHAs prior to the procedure by at least 1 week.

Risks associated with laser therapy include scarring, postprocedural inflammatory erythema, potential hyperpigmentation in susceptible individuals (higher-risk patients should consider hydroxyquinone (HQ) prophylaxis less than 5%; continuous supervision should be implemented while using HQ), as well as hypopigmentation (seen most often with phenol peels).

Bleeding with or without the presence of serosanginous fluid may occur immediately after dermabrasion or less often with laser use. Chronic bleeding would be a cause for alarm and return to the physician’s office. Patients should be made aware of all potential risks prior to the procedure.

Unusual Clinical Scenarios to Consider in Patient Management

Bullous solar elastosis is an extremely rare variant of actinic elastosis. The presentation includes the classic features of actinic elastosis, such as severe photodamage in sun-exposed areas, alongside the presence of bulla with a yellowish hue on top of erythematous papules and plaques. The most common location reported is the dorsa of the forearms.

Other less common locations include the posterior neck (described in one case). A mild burning sensation and/or pruritus of lesions has been reported by some patients, with others being asymptomatic. The diagnosis is made with a punch biopsy.

What is the Evidence?

Boyd, AS, Naylor, M, Cameron, GS. “The effects of chronic sunscreen use on the histologic changes of dermatoheliosis”. J Am Acad Dermatol. vol. 33. 1995. pp. 941-6. (A double-blind, placebo-controlled study that examines the use of UVA/UVB sunscreen on actinically damaged skin in 46 patients for 24 months. Histologic findings are reported.)

Calderone, DC , Fenske, NA. ” The clinical spectrum of actinic elastosis”. J Am Acad Dermatol. vol. 32. 1995. pp. 1016-24. (Excellent overview of actinic elastotic variants, along with a brief introduction to actinic elastosis.)

Findlay, GH, Morrison, JGL, Simson, IW. ” Exogenous ochronosis and pigmented colloid milium from hydroquinone bleaching creams”. Br J Dermatol. vol. 93. 1975. pp. 613-22. (Reviews the use of strong hydroquinone creams in South African women, which resulted in ochronosis and colloid milium.Examines the clinical, histological, histochemical, electron microscopical, and pathogenetic features from these cases.)

Greene, D, Egbert, BM, Utley, DS , Koch, RJ. ” In vivo model of histologic changes after treatment with the superpulsed CO2 laser, erbium: YAG laser, and blended lasers: A 4 to 6 month prospective histologic and clinical study”. Lasers Surg Med. vol. 27. 2000. pp. 362-72. (Comparison and overview of multiple types of lasers used in patients with facial photodamage. Of note, elastosis decreased the most with the CO2 laser and the least with erbium; collagenesis increased the most with CO2 and the least with Er:YAG.)

Heras, JA, Jimenez, ML. “Bullous solar elastosis”. Clin Exp Dermatol. vol. 32. 2007. pp. 272-4. (Report and literature review of patients diagnosed with bullous solar elastosis, along with histopathologic findings.)

Lewis, KG, Bercovitch, L, Dill, SW, Robinson-Bostom, L. ” Acquired disorders of elastic tissue: Part I. Increased elastic tissue and solar elastotic syndrome”. J Am Acad Dermatol. vol. 51. 2004. pp. 1-21. (An in-depth review of the biochemistry and physiology of elastic fibers. Also includes an overview of solar elastotic dermatoses (actinic elastosis, Favre-Racouchot syndrome, elastotic nodules of the ear and keratoelastoidosis marginalis).)

O’Brien, JP. ” A controlled hematoxylin-eosin for actinic elastosis-lysis. Its versatility and use for vascular damage (actinic arteriopathy) in the skin and orbit”. Am J Dermatolpathol. vol. 16. 1994. pp. 31-50. (Excellent pictures and descriptions of the histologic features of actinic elastosis.)

Phillips, TJ, Gottlieb, AB, Leyden, JJ. “Efficacy of 0.1% tazarotene cream for the treatment of photodamage. A 12-month multicenter, randomized trial”. Arch Dermatol. vol. 138. 2002. pp. 1486-93. (Double-blind study examining the safety and efficacy of tazarotene cream in 563 patients with facial photodamage. Significant improvement was noted in fine wrinkling, mottled hyperpigmentation, lentigines, pore size, irregular depigmentation, tactile roughness, coarse wrinkling and overall assessment of photodamage.)

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