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Generalized keratosis pilaris associated with erythromelanosis follicularis faciei

et colli: A rare coexistence

Article · July 2015

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Tasleem Arif
Jawaharlal Nehru Medical College (JNMC), Aligarh Muslim University (AMU), India
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CASE REPORT

Generalized keratosis pilaris associated with

erythromelanosis follicularis faciei et colli: A rare
coexistence
Tasleem Arif
Department of Dermatology, STDs and Leprosy, Jawaharlal Nehru Medical College, Aligarh Muslim University, Aligarh, India

ABSTRACT

Erythromelanosis follicularis faciei et colli (EFFC), is a very uncommon disease characterized by a triad of hyperpigmentation,
erythema and follicular papules on face and neck. There are reports of its association with keratosis pilaris (KP), but
coexistent EFFC, and generalized KP has rarely been reported. In this article, the author reports a 12‑year‑old boy with
concomitant EFFC and KP in a generalized distribution. It is the rarity of generalized KP in association with EFFC which
prompted the author to report this case.

Key words: Erythromelanosis follicularis faciei et colli, hyperpigmentation, keratosis pilaris

INTRODUCTION There is no history of the application of any topical

medication except emollients. Physical examination

E rythromelanosis follicularis faciei et colli (EFFC) is

a rare disorder of unknown etiology characterized
by the clinical triad of erythema, hyperpigmentation
and follicular plugging on the face.[1] By 2013,
some 55 cases of the disease have been reported.[2]
Though keratosis pilaris (KP) has been associated
with EFFC in some cases, but concomitant EFFC and
generalized KP has not been reported to the best of
the author’s knowledge. Here, the author describes a
unique case of EFFC in a 12‑year‑old boy associated
with generalized KP involving trunk and limbs.
CASE REPORT
A 12‑year‑old boy presented to our dermatological
outpatient department with erythema, pigmentation
and roughness of the cheeks since the age of 2 years.
There is a history of increase in the erythema and
burning sensation on exposure to sunlight. He
denied any similar history in his family members.
revealed bilaterally symmetrical erythema, brownish
hyperpigmentation, and follicular papules present
on the cheeks, preauricular and submandibular
areas [Figures 1 and 2]. There were multiple
follicular keratotic papules on the shoulders, back,
buttocks, upper arms, forearms, thighs, legs and
sparsely on the chest with perifollicular erythema at
many places [Figures 3‑5]. There was no associated
alopecia, atrophy or scarring. Examination of hair,
nails, and mucous membranes was normal. Systemic
examination was unremarkable. Biopsy of the skin
was not done. With such a history and clinical
examination, a diagnosis of EFFC with generalized KP
was made. The rarity of such an association prompted
the author to report the same.
ADDRESS FOR CORRESPONDENCE
Dr. Tasleem Arif,
Department of Dermatology, STDs and Leprosy, Jawaharlal Nehru Medical
College, Aligarh Muslim University, Aligarh, India.
E‑mail: dr_tasleem_arif@yahoo.com

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DOI: How to cite this article: Arif T. Generalized keratosis pilaris associated
10.4103/2319-7250.160665 with erythromelanosis follicularis faciei et colli: A rare coexistence. Indian J
Paediatr Dermatol 2015;16:149-51.

Copyright © 2015 Indian Journal of Paediatric Dermatology | Published by Wolters Kluwer - Medknow 149
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Arif: Generalised KP associated with EFFC

Figure 1: Erythromelanosis follicularis faciei et colli in a 12‑year‑old boy Figure 2: Close view of the erythromelanosis follicularis faciei et colli.
There is erythema, hyperpigmentation and keratotic follicular papules on
the face

Figure 3: Keratosis pilaris on the back with associated perifollicular

erythema at many places
Figure 4: Keratosis pilaris on the arm, shoulder, and chest

classically presents with hyperpigmentation, erythema

and follicular papules on the cheeks and pre‑auricular
areas. It was first described by Kitamura and others in
Japanese patients in 1960.[1] An autosomal recessive
pattern of inheritance has been proposed by some
researchers while others have reported familial cases
in few. Spontaneous mutation in EFFC has also been
reported.[3] Recently, some authors have suggested
that EFFC may be a polyetiological disorder involving
familial and environmental factors and the possibility
of a chromosomal instability syndrome.[4]

Erythromelanosis follicularis faciei et colli has been

Figure 5: Extensive keratosis pilaris involving both thighs
DISCUSSION
Erythromelanosis follicularis faciei et colli is an
uncommon disorder but rarely reported which
associated with KP. Ermertcan et al. described EFFC
in two brothers from Turkey associated with KP
involving shoulders and extensor aspects of arms.
[5]
Similarly Sardana et  al. have described a series
of five patients of EFFC from India. Two of these
patients had KP on the trunk while four of them

150 Indian Journal of Paediatric Dermatology | Vol 16| Issue 3 | Jul-Sep 2015
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Arif: Generalised KP associated with EFFC
involved legs.[6] However, the present case had KP in
a generalized distribution involving back, shoulders,
buttocks, thighs, legs, arms, forearms and sparsely
chest, which makes this case a rare one. Some
authors believe that EFFC is a clinical variant of KP
as KP on the arms has been frequently associated
and overlap with ulerythema ophryogenes has been
described.[7] However, EFFC is differentiated from
KP by the presence of pigmentation and extension
on to the neck. It is also possible that in a genetically
predisposed person and sunlight exposure over a
span of years develop pigmentation and persistent
erythema on the interfollicular skin of face and neck
in EFFC. Whether EFFC is a clinical variant of KP
or it is a separate entity with KP as one of its clinical
features is still a topic of debate.
Histopathology in EFFC is not diagnostic though it can
augment the diagnosis. The various findings on skin
biopsy, which have been reported include acanthosis,
hyperkeratosis, follicular plugging, increased
pigmentation in the basal membrane, perivascular and
periadnexal lymphocytic infiltrate.[8] However, in our
case skin biopsy was not done.
The treatment modalities for EFFC have not been
established yet. Various agents have been tried
which include topical retinoids, ammonium lactate,
hydroquinone, metronidazole, glycolic or salicylic
acid peels, oral isotretinoin and pulsed dye laser.[9,10]
However, the efficacy of these has been limited so far.
CONCLUSION
As previously thought to be a rare entity, EFFC is not so
rare but has been rarely reported till date. Generalized
KP can occur with EFFC demanding more attention
to be drawn towards the relation between the two
disease entities.
Indian Journal of Paediatric Dermatology | Vol 16| Issue 3 |Jul-Sep 2015
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Financial Support and Sponsorship
Nil.
Conflicts of Interest
There are no conflicts of interest.
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