What Caused These Nodules and Blackheads?

Patient Presentation A 65-year-old African-American woman presented with multiple nodules and large blackheads on her face, which she explained had gradually developed over the past 5 years. She was otherwise healthy and not taking any oral medications. She denied applying any topical medications to her face, but she mentioned spending many years living in the South where she had a fair amount of sun exposure. The patient had no history of smoking or radiation exposure, and her family history was unremarkable. On physical examination, both cheeks and periorbital areas exhibited multiple papules, large open comedones, and nodules overlying a sallow facial skin. What is your diagnosis? Read on for an answer and for more details about the condition and this case. Diagnosis: Favre-Racouchot Syndrome (FRS) Favre-Racouchot syndrome (FRS) is a dermatologic condition of multiple large open comedones and nodules of the periorbital areas. Underlying skin is often pale to yellowish and diffusely thickened with furrows. On a Historic Note M. Favre first reported the characteristic clinical findings in a patient he presented at the meeting of “Dermatology and Syphiligraphy of Lyon,” in France in 1931. Later, in 1937, J. Racouchot, one of M. Favre’s students, reported 11 other cases in a doctorate thesis. This original work was officially reported by both authors in 1951 as “L’élastéidose cutanée nodulaire à kystes et à comédons.”1 Subsequently, the word “élastéidose” was reported as “élastoidose,” and the condition was reported as Favre-Racouchot-syndrome or Favre-Racouchot disease in the French literature. In the English literature, this syndrome is known as nodular elastosis with cysts and comedones (FRS). Other synonyms include senile comedones, actinic comedonal plaques, solar comedones, and smoker’s comedones.2 Who Is Typically Affected and how? FRS occurs in approximately 6% of the middle-aged and elderly (40 to 60 year-old) population.3 It is also found predominantly in Caucasian males.4 The classic manifestation of FRS is gradual development of multiple, grouped comedones, papules and nodules, plaques, cysts and furrows in the setting of yellowish, sun-damaged skin.2,5,6 Lesions usually appear in a symmetrical distribution.2,7 The characteristic location of these lesions is on the face, particularly around the lateral canthi of the eyes, temples, cheeks, lateral aspects of the neck, and retro-auricular regions.5 However, asymmetric presentations and atypical locations have been reported. These locations include scapular girdle, arms and forearms, nose and chin, and scalp.2,5,8,9 Histopathology of FRS The histologic examination of FRS demonstrates large, non-inflammatory, open and closed comedones surrounded by extreme actinic elastosis in the dermis.10 Each comedone has an opening with associated atrophic sebaceous glands and contains a number of hair shafts, bacteria, sebum, and eosinophilic laminated horny material within the expanded infundibulum.10 The epidermis is thinned with flattened rete ridges.11 The upper dermis indicates pronounced basophilic degeneration of fibrous tissue with intermingled, large collections of the amorphous elastotic material.11,12 The cysts and comedones of FRS are both open and closed. They can be easily differentiated from an infundibular cyst by the histopathologic features rather than by surface connection.10 FRS may look similar to acne vulgaris; however, the characteristic inflammation is missing around the comedones. How FRS Develops The pathogenesis of FRS is unclear. However, several associated factors have been linked with this syndrome. As mentioned above, one of the synonyms used for FRS is “senile comedones” because there’s a strong association with aging.1,12 Other synonyms include “actinic comedonal plaques” or “solar comedones” because of the compelling connection between UV-light exposure and development of FRS.12 This relationship has been speculated based on two findings: 1. FRS histology indicates the actinic changes of dermal elastosis.12 2. There are frequent associations of FRS with other actinic cutaneous conditions such as actinic keratosis, cutis rhomboidalis nuchae and skin carcinomas.13 However, the idea of solar pathogenesis for FRS is controversial. Another proposed theory is that follicular changes in FRS are not secondary to solar elastosis but are caused by a unique reaction of follicles in predisposed individuals.6,14 There is also a report of one case of FRS associated with radiation therapy.4 Smoking has also been implicated as an important etiologic factor for developing FRS.15 Other theorized factors associated with FRS include repeated pressure,5 and chronic exposure to atmospheric agents: heat, cold and wind.13 Conditions Similar to FRS The diagnosis of FRS is usually made on clinical grounds; however, a number of skin conditions with similar distribution and appearance of FRS should be considered in the differential diagnosis. Among these are: acne vulgaris, pitch or coal-tar acne, chloracne, milia, syringomas, trichoepitheliomas, necrobiotic xanthogranulomas, xanthelasma, epidermoid sebaceous cysts, senile sebaceous hyperplasia, verrucae plana, folliculitis, rosacea, elastomas, collagenous plaques of the hand, adult colloid milium, actinic granulomas, and unusual presentation of mycosis fungoides.1-19 The history and the histologic examination are very helpful in narrowing the list of differential diagnoses. Managing this Condition The pre-eminent management of FRS is yet to be determined. Several mechanical and medical treatments have been reported with successful cosmetic results. One treatment includes topical application of retinoids (tretinoin, tazarotene, adapalene), which help loosen the comedones and assist in the their extraction.2,13,15 Oral retinoids may also be beneficial. Curettage, dermabrasion, simple surgical excision or multiple-stage excision followed by dermabrasion may also be utilized.6 Combined therapy with CO2 laser followed by extraction with forceps has been used.19 The decision on which type of treatment to employ is generally based on size and type of FRS lesions.2,13,15-19 Prevention and Prognosis for FRS Sun protection and smoking cessation are good preventative measures to decrease the risk factors for FRS.2,15 The prognosis is excellent because of the benign baseline nature of FRS and availability of treatment techniques that have good cosmetic results. Very rarely, secondary infection (folliculitis) may develop.