1. Khalid Al Hawsawi Dermatology Consultant
Erythromelanosis Follicularis Faciei: A Case Report and Review of the Literature
Ghassan Niaz
Medical Intern
Khalid Al Hawsawi
Dermatology Consultant

2. Objectives
Case Report
Review of EFF
Literature review of EFF
Summary

3. Case Report :
History
A 16-year-old male, otherwise healthy With asymptomatic facial skin lesions since early childhood. He used various topical treatment for years but without effect . No history of predisposing factors or photosensitivity . Review of system: unremarkable PMH: unremarkable Family history : his younger brother has similar condition Parents are not contagious .

4. Skin Examination
Diffuse non-scaly reddish-brownish patches with multiple skin-colored, hypo-pigmented follicular papules on both cheeks.

5. Deferential Diagnosis:
keratosis pilaris rubra .
atrophoderma vermiculatum .
Systemic lupus erythematous .
photosensitivity syndromes: Bloom, porphyria , cockane syndrome .
pigmented peribuccal erythrosis of Brocq .
Fixed pigmented Erythema .

6. Diagnosis: Treatment : Erythromelanosis Follicularis Faciei
Patient Reassured with regular follow up

7. It was first described in Japanese patients in 1960 by Kitamura.
Background
Erythromelanosis follicularis faciei (EFF) is a rare sporadic condition
characterized by erythematous hyperpigmented patches and follicular papules on the face.
It was first described in Japanese patients in by Kitamura.
When the neck is affected, the condition is called erythromelanosis follicularis faciei et colli (EFFC)
EFF continued ….

8. Etiology
The pathogenesis is unknown. However, a combination of vasodilation and hyperpigmentation has been found in the affected areas. Some authors consider EFF as part of the spectrum of keratosis pilaris atrophicans disorders .
Mishima et al have proposed the possibility of autonomic nerve dysfunction as one of the etiology factors.
Yanez et al suggested an autosomal recessive mode of inheritance. Acay10 added two more cases of EFFC with a positive family history, and also suggested the existence of an inherited form of EFFC.
Tuzun et al suggested that EFFC may be a poly-aetiological disorder (i.e. familial and environmental) and might be considered one of the chromosomal instability syndromes .
EFF continued ….

9. Clinical presentation
Characterized by presence of red-brown patches on the lateral aspects of the cheeks, and rarely lateral aspects of the neck.
Usually asymptomatic but burning sensation has been described
Numerous pinhead-sized follicular papules are present within the involved areas that may sometimes appear relatively hypopigmented.
Bilateral distribution is characteristic, but unilateral cases have been described
Keratosis pilaris elsewhere in the body is a common association with EFF
EFF continued….

10. Histopathology there are hyperkeratosis,
slight follicular hyperkeratosis
(follicular plugging)
increased basal layer
pigmentation
dilatation of superficial dermal
blood vessels
periadnexal lymphocytic infiltrate
EFF continued…

11. Review of Literature

14. The male:female ratio is 2:1
Table Summary
Total of 130 cases were reported in the literature
the onset of the disease shows a wide range, starting from birth to as old as 43 years old .
The male:female ratio is 2:1
Most common site affect is checks then neck .
Most of the reported cases there was no family history of EFF .

15. Conclusion
Erythromelanosis follicularis faciei (EFF) is a pigmentary disease associated with erythema and follicular papules on the face. It affects all races. However, it shows a preponderance in the people of Asian ancestry The cause is unknown, but the hereditary component (autosomal recessive) seems to play a role in the pathogenesis

16. Thank You