What Are These Periorbital Changes?

A 72-year-old white man, previously employed as a truck driver, presented to the outpatient clinic for removal of nonmelanoma skin cancer from the tip of his nose.  The patient had a past medical history significant for basal cell carcinoma removed from his right upper chest. Patient was noted to have a painless, raised nodule with comedones exclusively on the left periorbital region (Figure 1A). The patient indicated the lesion was not new but rather present for many years and has slowly increased in size. He denied any other significant medical history or change in medications. Physical examination revealed unilateral left malar actinic skin, which was yellow in color and superimposed on multiple wrinkles in the left malar region, with multiple closed comedones. Right malar region had insignificant clinical findings (Figure 1B).

What is Your Diagnosis?

Diagnosis: Favre-Racouchot syndrome

Favre-Racouchot syndrome (FRS), also known as solar comedones, senile comedones, or nodular elastosis with cysts and comedones, is characterized as a group of comedones with a dark central plug. These lesions are usually symmetric and bilateral and often located on the temporal and periorbital skin, but they can also occur in other areas of the head and neck that are exposed to the sun. If unilateral lesions develop, it is often the result of asymmetric radiation or sun exposure.¹ The nodular elastosis is named after the dermal elastic tissue that results from sun exposure.^2-4 With its increasing prevalence, multiple new treatment modalities have been implicated in FRS.

Epidemiology

FRS mostly affects white men between the ages of 40 to 60 years, though cases in women have been recognized.² The prevalence of FRS is approximately 6% in patients aged 50 years and older; however, there are cases reporting this condition in patients as early as age 20 years.^2,3 Most cases are associated with a history of heavy smoking or chronic UV-A and UV-B radiation secondary to sun exposure. A few cases have also linked radiation exposure to the development of solar elastosis, a thick and disorganized cluster of elastic fibers.^2,5-7

Pathophysiology

The etiology of FRS is not well understood. This condition was discovered in 1932 and detailed in 1951 by Maurice Favre and his pupil Jean Racouchot.⁸ What is known about the condition is that lesions develop and progress over an extended period, most often years. The underlying pathophysiology has been attributed to disturbed follicular keratinization, often secondary to UV-A exposure, that results in comedone formation.⁹ It is also hypothesized that UV light exposure results in damage of the elastic network located in the papillary and upper reticular dermis, resulting in the hallmarked solar elastosis.^2,7,9 These damaged elastic fibers then lead to sebum retention and the formation of comedones and, eventually, nodules and cysts. The resulting atrophic dermis also contains keratin masses, which are associated with the follicular plugging that can result in the characteristic comedones, cysts, and nodules of FRS.^2,7,10,11 

The difference between the hallmark comedones of FRS and those seen in acne is the absence of inflammation in FRS. Cigarette smoking and UV light exposure have long been attributed to the formation of FRS^11-13 and are known to act synergistically in disease formation and progression. FRS has also been described as being associated with conditions like basal cell carcinoma, actinic keratosis, and squamous cell carcinoma.^3,14-16 However, it can be argued that these conditions are also associated with increased age, smoking, and radiation exposure. Other FRS-associated conditions include cutis rhomboidalis nuchae, trichostasis spinulosa, and keratoacanthoma.¹⁴

Clinical Presentation

Clinically, FRS lesions can be identified as atrophied, actinic skin that is often yellow in appearance with wrinkles and furrows. Cystic nodules and multiple comedones (both open and closed) are present. Lesions may also be inflamed and feel waxy or rough. Eruptions are more commonly bilateral and symmetric in sun-exposed areas, but they can present unilaterally.^11-13

Histology

On histologic exam, FRS lesions only differ from acne vulgaris by lack of inflammation and the presence of actinic keratosis in the dermis. Characteristic findings on hematoxylin-eosin staining include atrophic sebaceous glands, dilated pilosebaceous openings, and large, round cyst­-like spaces lined by a flattened epithelium and filled with layered horny material¹⁰ (Figure 2¹¹). It is not surprising that the comedones are often contaminated with Propionibacterium acnes and Corynebacterium acnes.¹¹

Differential Diagnosis

Although a clinical diagnosis, FRS can often be mistaken for other lesions such as basal cell carcinoma. This is especially true as both entities are often found in similar locations on the face. These two conditions were our primary differentials, as the patient has a known history of nonmelanoma skin cancer and overlapping risk factors (eg, sun exposure). Lesions can also be mistaken for amyloidosis and nodular chondrodermatitis helicis. Other differentials diagnoses include colloid milium, epidermoid cyst, milia, syringoma, or trichoepithelioma.^4,5 Histopathology is invariably the best differentiator, but FRS is a clinical diagnosis and does not require histopathology for accurate diagnosis.

Management  

Diagnosis of FRS is typically based on clinical findings alone and does not usually require biopsy. First-line treatment is often topical retinoids, 0.025% cream applied twice a day to help aging skin and repair collagen and elastin damage in the layers of the dermis.¹⁷ Therapies are often most effective, however, when utilized in combination. Other therapeutic options include surgical excision, cryotherapy, curettage, dermabrasion, or comedone extraction.¹² In addition, carbon dioxide laser therapy is steadily gaining value in treatment of FRS.^18,19 

It is important that patients are educated on sun-protective measures, including wearing sunscreen with sun protection factor greater than 50, avoiding sun exposure with hats, or avoiding the sun between the hours of 10 am and 4 pm.¹¹ Emphasis must also be placed on smoking cessation. Patients should follow up periodically for reoccurrence. 

Our Patient

This patient’s presentation was classical FRS, specifically the periorbital location and most likely secondary to occupational chronic sun exposure. On examination, the patient had multiple classically characterized closed comedones and papules (Figure 1A). As the patient’s lesions were unilateral, as opposed to bilateral, this was attributed to his profession. Therefore, a biopsy and histopathology were not warranted. Treatment options, including topical retinoids, dermabrasion, and excision, were described in depth to the patient. However, the patient declined all treatment options, because the lesion did not trouble him. He was advised to continue sun avoidance and protective measures as well as to follow up with the dermatologist in three months. 

Summary

FRS is often prevalent in middle aged, white men and is characterized by benign, often bilateral, lesions located in temporal and periorbital regions. Lesions are described as yellow actinic lesions with nodules and cysts and the hallmark of closed or open comedones. FRS is a clinical diagnosis associated with a history of chronic sun exposure or cigarette smoking. Histopathology will reveal solar elastosis, decreased sebaceous glands, and nodules filed with keratin. Interventions include topical retinoids, excision, and extraction of comedones and are most often effective if used simultaneously. Reoccurrence can occur despite treatment, and patients are advised to avoid sun exposure and follow up in regular intervals with their dermatologist.

Dr Ahmed is a research fellow at the Weill Cornell Medical College in New York, NY. Ms Fravor is a fourth-year medical student at West Virginia School of Osteopathic Medicine in Lewisburg, WV. Dr Khachemoune is with the department of dermatology at both Veterans Affairs Medical Center and the State University of New York Downstate, both in Brooklyn, NY.

Disclosure: The authors report no relevant financial relationships.  

References

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