Erythromelanosis follicularis faciei et colli
The most common locations are the cheeks, preauricular area, and submandibular regions; however, the eyebrows, chin, and pinnae may also be involved. EFFC is typically bilateral. Onset is usually in the second decade, and males are reported to be affected more frequently than females. Most cases are described in people of Asian descent; however, there are many reports in people of Northern European descent as well.
EFFC is typically asymptomatic; however, pruritus or burning may accompany the cutaneous findings. Photosensitivity has been reported.
The etiology of EFFC is unknown. Genetic predisposition, abnormal autonomic nervous system functioning, and sun exposure have been postulated to contribute to its pathogenesis.
There is a well-established association of EFFC with keratosis pilaris (KP), and some authors have suggested that EFFC may be a variation of KP atrophicans.
L53.8 – Other specified erythematous conditions
78631004 – Erythromelanosis follicularis of face AND/OR neck
Differential Diagnosis & Pitfalls
- KP atrophicans faciei and variants (atrophoderma vermiculatum and keratosis follicularis spinulosa decalvans) – Similar findings to EFFC but no pigmentation, and atrophy or scarring are usually seen.
- Poikiloderma of Civatte
- Riehl melanosis
- Erythrose peribuccale pigmentaire of Brocq – Manifests orange-yellow-brown or red patches with associated scaling in a perioral location. It is thought by some authors to be a variant of EFFC and by others to be a variant of Riehl melanosis.
- Lichen spinulosus
- Fixed drug eruption
- Berloque dermatitis (see phytophotodermatitis)
- Nevus of Ota