Transcript A Case Report and Review of the Literature

Erythromelanosis Follicularis
Faciei: A Case Report and
Review of the Literature
Ghassan Niaz
Medical Intern
Khalid Al Hawsawi
Dermatology
Consultant
Objectives

Case Report

Review of EFF

Literature review of EFF

Summary
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 .
Skin Examination
Diffuse non-scaly reddish-brownish patches with multiple skin-colored,
hypo-pigmented follicular papules on both cheeks.
Deferential Diagnosis:

keratosis pilaris rubra .

atrophoderma vermiculatum .

Systemic lupus erythematous .

photosensitivity syndromes: Bloom, porphyria ,
cockane syndrome .

pigmented peribuccal erythrosis of Brocq .

Fixed pigmented Erythema .
Diagnosis:
Erythromelanosis Follicularis Faciei
Treatment :
Patient Reassured with regular follow up
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
1960 by Kitamura.

When the neck is affected, the condition is called
erythromelanosis follicularis faciei et colli (EFFC)
EFF continued ….
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 ….
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….
Histopathology


there are hyperkeratosis,
slight follicular hyperkeratosis
(follicular plugging)
increased basal layer
pigmentation

dilatation of superficial dermal
blood vessels

periadnexal lymphocytic infiltrate
EFF continued…

Review of Literature
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 .
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
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