|Year : 2017 | Volume
| Issue : 1 | Page : 53-59
Erythromelanosis follicularis faciei et colli: A first female case report from the outskirts of Libya and literature review
Private Practice (LMH), Dermatology Department, Academia Title for Health Professional (QU Affiliation), Independent Researcher, Tripoli, Libya
|Date of Web Publication||12-Dec-2016|
Private Practice (LMH), Department of Dermatology, Academia Title for Health Professional (QU Affiliation), Independent Researcher, P.O.BOX 3232 Tripoli
Source of Support: None, Conflict of Interest: None
Erythromelanosis follicularis faciei (EFF) is a rare sporadic condition of unclear etiology and is distinctive by well-demarcated reddish-brown patches and follicular papules that appear often on the face and seldom on the neck. Herein, I report a case of a 15-year-old female who had asymptomatic facial skin discoloration since early childhood. Skin examination revealed confluent nonscaly rough brownish red patches on both cheeks without telangiectasia and a barely thick scaly brownish patch over her entire forehead and both eyebrows with follicular plugging. A summary of preceding reports of EFF in the literature is presented in this report.
Keywords: Erythromelanosis follicularis faciei, erythromelanosis follicularis faciei et colli, hyperpigmentation, keratosis, keratosis pilaris
|How to cite this article:|
Elghblawi E. Erythromelanosis follicularis faciei et colli: A first female case report from the outskirts of Libya and literature review. Indian J Paediatr Dermatol 2017;18:53-9
|How to cite this URL:|
Elghblawi E. Erythromelanosis follicularis faciei et colli: A first female case report from the outskirts of Libya and literature review. Indian J Paediatr Dermatol [serial online] 2017 [cited 2021 May 6];18:53-9. Available from: https://www.ijpd.in/text.asp?2017/18/1/53/195571
| Introduction|| |
Erythromelanosis follicularis faciei is a rare sporadic condition of unknown etiology, and when the neck is affected; the condition is called erythromelanosis follicularis faciei et colli (EFFC). It is a mix between vasodilation and hyperpigmentation in the affected areas.,
EFFC is thought to be a rare asymptomatic condition of unidentified aetiology, however, a burning sensation has been described in a few patients. [1,2] The clinical findings are reddish-brown pigmentation on the face including the eyebrows, sometimes with telangiectatic vessels, and pale follicular papules all over the body. The disease is asymptomatic and has only been described in men.
Some authors consider EFF as part of the spectrum of keratosis pilaris atrophicans disorders.
The first reported case of ''erythromelanosis follicularis faciei'' was coined by Kitamura in the 1960 and collaborators in six young Japanese patients and characteristicesd by well-demarcated erythema, hyperpigmentation, and follicular papules and since its original description, it has infrequently been reported in the literature., Then in 1966, Mishima and Reuder observed this condition in four middle-aged Caucasian males with neck involvement and added the term ''et colli'' to the name of the disease- Erythromelanosis follicularis faciei et colli (EFFC).,
EFFC nosologic (classification of diseases) and aetiologic frames still remain to be clarified according to whittaker and griffiths in 1987, they state that the condition has only rarely been reported in Caucasians.
EFFC stated to start symmetrically on the preauricular cheeks and spreads onto the temples and lateral aspects of the neck and often seen in adolescent and young males, and by 2013, only 55 cases of the disease have been reported in the literature.,
Keratosis pilaris (KP) has been associated with EFFC in some cases, however synchronized EFFC and generalized KP has been only once reported by one author.
In this paper I report a unique case of female patient of EFFC and trunk, a dermatosis which was thought to affect men only.
To the best of my knowledge, this is the first report of EFFC from Libya.
| Case Report|| |
A 15-year-old healthy suburbanite Libyan girl presented to me for a localized scaly lesion on her left postauricular region. While so, I noticed her skin's peculiar finding and I offered examination. The facial pigmentation started since long time in her childhood as she affirmed, and she sought some medical advice as she come from the outskirt, a foreigner doctor told her she will get cleared off when she grown up. She never had any proper treatment or examination as she claimed. She said that her condition remained static as such as always it was. There was no history of the application of any topical medication except emollients.
On physical examination, the classical triad of erythema, pigmentation, and follicular papules was present; however, telangiectasia was not observed.
The face showed symmetrical brownish hyperpigmentation, with roughness on the malar region, preauricular regions, lateral aspects of the neck, upper lips including the entire forehead, and both eyebrows [Figure 1] and [Figure 2]. There is no associated telangiectasia.
|Figure 1: Erythema and hyperpigmentation seen in the malar area, preauricular regions (Copyright: ©2016 Elghblawi)|
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|Figure 2: Close view of the erythromelanosis follicularis faciei et colli. There are erythema, hyperpigmentation, and keratotic follicular papules on the face (Copyright: ©2016 Elghblawi)|
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Her malar patch will not increase redness on exposure to sunlight. She has no burning sensation though. There is no loss of the eyebrow hair. She denied any similar history in her family, and her parents are not consanguineous.
On examination, there are multiple follicular papules all over her body (follicular papules without erythema were present on her entire trunk and extensor surfaces of the arms and the legs), with distinctive facial brownish discoloration [Figure 3],[Figure 4],[Figure 5].
|Figure 3: Keratosis pilaris on the back with associated perifollicular erythema at many places (Copyright: ©2016 Elghblawi)|
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|Figure 4: Keratosis pilaris on the arm, shoulder, and chest (Copyright: ©2016 Elghblawi)|
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|Figure 5: Extensive keratosis pilaris involving both thighs (Copyright: ©2016 Elghblawi)|
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Patches of brownish pigmentation persisted on diascopy, over the cheeks and chin, where only the erythema disappeared. The skin texture was granular with many pale, follicular papules. Prominent KP was seen on the upper back, shoulders, tummy and the arms and legs. There was no diffuse erythema or pigmentation over these areas.
Examination of the hair, nails, and mucous membranes was normal.
The patient's lesions were not induced by light or by the use of topical or systemic photosensitizers.
There is no history of atopy, photosensitivity, and pruritus in this patient. History of seasonal variation was denied.
Furthermore, there was no atrophy, scarring, or alopecia. Systemic examination failed to divulge any significant abnormality. There was no family history of atopy or evidence of atopic signs.
Biopsy of the skin was not done as the patient denied biopsy. However, with such a history and clinical examination, a diagnosis of EFFC with generalized KP was made.
On the basis of the above classical clinical findings, the diagnosis of EFFC was made. The patient was re-assured and put under periodic follow-up.
I had advised about sunscreen and emollients and application of iso-urea cream.
| Discussion|| |
Erythromelanosis follicularis faciei et colli (EFFC) is an unusual erythematous pigmentary disease involving the hair follicles.
EFFC was thought to be a rare disease, but it is increasingly being described.
EFFC is a disorder of unknown etiology which classically presents with a triad of hyperpigmentation, erythema (with or without telangiectasia), and follicular papules, and barely fifty cases are reported in literature, most of them being males of adolescent and young adult age, and following the Kitamura's description, there have been many reports of this disorder in women.
Keratosis pilaris elsewhere in the body is a common association with EFF, and this case has shown this association. KP or keratosis follicularis on the arms and shoulders is frequently found, and it is considered by some to be a variant of keratosis rubra pilaris.
EFFC is mostly bilateral in distribution, however unilateral has been described.,
The disease might have a genetic and hereditary background with which autosomal recessive mode of inheritance was examined by Yañez et al.
Keratosis pilaris is well known to be associated with EFFC as my case has depicted. This coexistence supports a strong genetic relation for the two conditions. However its still a matter of debate. My patient did not have a relevant family history.
Tüzün et al. and other authors have suggested that EFFC might be a polyetiological disorder involving familial and environmental factors with the possibility of a chromosomal instability syndrome where hereditary component could interfere with its genesis. The likelihood of a spontaneous mutation has also been cited.
There is another prospect by Mishima et al., who have proposed the possibility of autonomic nerve dysfunction as one of the etiological factors.
An old report in 1985 by Lee and Yang had shown a cutaneous calcinosis in histological examination in a Korean patient with EFFC, however the claim can be refuted as it could be idiopathic as the patient workup did not show anything unremarkable.
The histopathology is not diagnostic but augments the diagnosis. Usually, such cases show hyperkeratosis and enlarged hair follicles, especially in the infundibular area with thick shaft. Nonetheless, the number of hair follicles are neither increased nor enlarged in EFFC according to Kim et al.'s quantitative histopathologic findings. Sebaceous glands are hypertrophic, and lymphocytic infiltration is present around the adnexa. Follicular plugging with increased pigmentation in the basal layer and mild perivascular lymphocytic infiltrate. However, Kim et al.'s study did not support the significant differences in pigmentation of the basal layer. Hyperkeratosis and dilatation of the blood vessels in the upper dermis can be major characteristic histopathological findings of EFFC. Some reports had an examination of the affected skin under Woods' light which showed increased melanin pigmentation, suggesting a correlation with superficial distribution in the affected areas.
EFFC should be differentiated from some diseases, namely, ulerythema ophryogenes, atrophoderma vermiculatum, poikiloderma of Civatte, Riehl's melanosis, acneiform eruption, pigmented peribuccal erythrosis of Brocq (erythrosis pigmentosa mediofacialis), keratosis rubra pilaris atrophicans faciei (Brocq), KP rubra, and lichen pilaris faciei, all of which should be considered to make the distinctive diagnosis.
The differential diagnosis of EFFC is discussed in [Table 1].
|Table 1: Differential diagnosis between erythromelanosis follicularis faciei et colli and similar dermatoses,,|
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KP of the face is not associated with well-defined patches of erythema and pigmentation although erythema may be seen around the papules. Unilateral forms of EFFC must be differentiated from fixed pigmented erythema, berloque dermatitis, and rarely from the nevus of Ota and Becker's nevus.
There are reports of EFFC's association with KP, but coexistent EFFC and generalized KP have rarely been reported. Even the documented histopathologic examination of skin biopsy specimens revealed hyperpigmentation of the basal layer, follicular plugging, dermal vascular dilatation and congestion, and perivascular inflammatory infiltration, which implies a genetic inheritance and a possible relationship between the two disorders.
In this article, I report a case of a 15-year-old girl with concomitant EFFC and KP in a generalized distribution involving her back, shoulders, buttocks, thighs, legs, arms, forearms, and chest, which makes this case a rare one. It is the rarity of generalized KP in association with EFFC which prompted me to report this case.
This condition is probably genetically related to KP. The exact nature of genetic transmission needs to be clarified. A diagnosis of EFFC with generalized KP was made in my case. The rarity of such an association prompted me to report this case.
Treatment is disappointing, however many trials had been employed. A combination of laser treatment (Long-pulsed dye laser) for erythema and Q-switched Nd:YAG laser for hyperpigmentation have been tried however that mandate multiple sessions to comment on a credential results.
Also topicals keratolytic, retinoids, hydroquinone, vitamin C, salicylic acid peels (20-30%), glycolic acid peels, tacalcitol ointment, and metronidazole gel have been conducted but with no avail.,
Because the primary symptoms of EFFC are of a relatively mild cosmetic nature and the disease generally takes an asymptomatic course, only very few EFFC patients actually demand treatment, and thus the disease go unrecognized as in this case who came accidentally for something else.
The occurrence of this disease appears higher than is divulged by the limited number of cases reported in the literature. It warrants more acknowledgment. Some authors consider it as a rare disease, but others consider it an underdiagnosed entity (because it was not well recognized by physicians). Awareness about the disease on the part of dermatologist and recognition of clinical presentation are important for correct diagnosis and to help find more effective therapeutic modalities. According to the existing literature, the 13th case of erythromelanosis follicularis of the face and neck was reported in 1981.
Thus, as previously thought to be a rare entity, EFFC is not so rare but has been rarely reported till date. The reason could be explained either due to lack of the disease awareness by the patient due to its general asymptomatic nature or underrecognition by the physicians.
Moreover, some authors claimed that EFFC can coexist with erythrosis pigmentosa mediofacialis (Brocq) [Table 1].
To the best of my knowledge, this is the first case of EFFC from the outskirts of Libya, with extensive KP, and I believe that EFFC could be common but infrequently reported or unrecognized condition.
I report this unique classic case for the first time in Libya.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial Support and Sponsorship
Conflicts of Interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]