Erythromelanosis follicularis faciei et colli: Causes, Symptoms, Histopathology And Treatment

• Erythromelanosis follicularis faciei et colli was thought to be a rare disease, but it is increasingly being described.
• Although this disorder was said to primarily affect males, there have been many reports of this disorder in women.

• The mean age of onset of the disease was 12 years. In corroboration with earlier reports.
• Patients had classical bilateral presentation except for one male who presented with unilateral lesions. Cases with unilateral distribution have been described in the literature.

 

Etiopathogenesis of Erythromelanosis follicularis faciei et colli

• The etiopathogenesis of Erythromelanosis follicularis faciei et colli is unknown. Yañez et al. proposed an autosomal recessive mode of genetic inheritance.
• Tüzün et al. thought that Erythromelanosis follicularis faciei et colli can be a polyetiologic disorder (such as  familial, and environmental).
• A possibility of chromosomal instability syndromes has also been considered in which a hereditary component could interfere with its genesis.

Symptoms of Erythromelanosis follicularis faciei et colli

• The disease is asymptomatic. However,  some patients may complain of a burning sensation and increased redness over the affected areas. Variations in symptoms, seasonal influences, and exacerbation by sunlight have been observed in a few reported cases.
• All our patients complained of mild pruritus, burning sensation, and dryness with aggravation of symptoms after photo exposure.

Keratosis Pilaris And Erythromelanosis follicularis faciei et colli

• Keratosis pilaris is known to be frequently associated with EFFC. A family history of KP has also been reported in the literature.
• This coexistence and familial occurrence support a strong genetic relationship between the two conditions.

 

Histopathology of Erythromelanosis follicularis faciei et colli

• Histopathology revealed

1. Hyperkeratosis
2. Follicular plugging
3. Increased  pigmentation in the basal membrane
4. Dilated blood vessels in the upper dermis
5. Follicular dilatation
6. Perivascular and periadnexal inflammatory infiltrate.

• There is a correlation between the severity of the disease and the pigmentation of the basal layer and the percentage area of the inner spaces of the superficial dermal blood vessels.

Treatment of Erythromelanosis follicularis faciei et colli

• No treatment has proven satisfactory in the treatment of Erythromelanosis follicularis faciei et colli. 
• Various options have been explored but lesions reappear if the treatment is discontinued.

    1. Topical Treatment

Topicals keratolytic agents such as ammonium lactate, tretinoin cream (0.05%, 1%), salicylic acid 2%, metronidazole, hydroquinone 4%, and chemical peel have been tried.  Topical tacalcitol has been reported to be effective.

    2. Oral Treatment And Sun Protection

• Intermittent oral isotretinoin and long-pulsed dye laser are other treatment options being considered.
• Avoidance of solar exposure and the use of sunscreen is recommended.

Conclusion

• Erythromelanosis follicularis faciei et colli becomes more common than previously believed. 
• Familial cases do occur, implicating a genetic cause of the disorder. But, the exact etiologic factor of the disease needs to be clarified.
• Because the predominant symptoms of Erythromelanosis follicularis faciei are aesthetic in nature and the condition often runs silent, very few Erythromelanosis follicularis faciei et colli patients seek therapy. 
• Furthermore, clinicians are under-recognizing the condition. 
• The dermatologist's knowledge of the disease and awareness of the clinical presentation is critical for accurate diagnosis and the development of more effective therapy methods.