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CASE REPORT
Year : 2015  |  Volume : 16  |  Issue : 3  |  Page : 149-151

Generalized keratosis pilaris associated with erythromelanosis follicularis faciei et colli: A rare coexistence


Department of Dermatology, STDs and Leprosy, Jawaharlal Nehru Medical College, Aligarh Muslim University, Aligarh, India

Date of Web Publication10-Jul-2015

Correspondence Address:
Tasleem Arif
Department of Dermatology, STDs and Leprosy, Jawaharlal Nehru Medical College, Aligarh Muslim University, Aligarh
India
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Source of Support: Nil., Conflict of Interest: There are no conflicts of interest.


DOI: 10.4103/2319-7250.160665

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  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.

Keywords: Erythromelanosis follicularis faciei et colli, hyperpigmentation, keratosis pilaris


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

How to cite this URL:
Arif T. Generalized keratosis pilaris associated with erythromelanosis follicularis faciei et colli: A rare coexistence. Indian J Paediatr Dermatol [serial online] 2015 [cited 2019 Dec 7];16:149-51. Available from: http://www.ijpd.in/text.asp?2015/16/3/149/160665


  Introduction Top


Erythromelanosis 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 Top


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. There is no history of the application of any topical medication except emollients. Physical examination revealed bilaterally symmetrical erythema, brownish hyperpigmentation, and follicular papules present on the cheeks, preauricular and submandibular areas [Figure 1] and [Figure 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 [Figure 3][Figure 4][Figure 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.
Figure 1: Erythromelanosis follicularis faciei et colli in a 12-year-old boy

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Figure 2: Close view of the erythromelanosis follicularis faciei et colli. There is erythema, hyperpigmentation and keratotic follicular papules on the face

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Figure 3: Keratosis pilaris on the back with associated perifollicular erythema at many places

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Figure 4: Keratosis pilaris on the arm, shoulder, and chest

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Figure 5: Extensive keratosis pilaris involving both thighs

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  Discussion Top


Erythromelanosis follicularis faciei et colli is an uncommon disorder but rarely reported which 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 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 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 Top


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.

 
  References Top

1.
Augustine M, Jayaseelan E. Erythromelanosis follicularis faciei et colli: Relationship with keratosis pilaris. Indian J Dermatol Venereol Leprol 2008;74:47-9.  Back to cited text no. 1
[PUBMED]  Medknow Journal  
2.
Thapa DP, Jha AK. Erythromelanosis follicularis faciei et colli: Report of a case and literature review. Egypt Dermatol Online J 2013;9:10.  Back to cited text no. 2
    
3.
Griffiths WA, Judge MR, Leigh IM. Disorders of keratinization. In: Champion RH, Burton JL, Burns DA, Breathnach SM, editors. Rook Textbook of Dermatology. 6th ed. Oxford: Blackwell; 1998. p. 1537-8.  Back to cited text no. 3
    
4.
Tüzün Y, Wolf R, Tüzün B, Ozdemir M, Demirkesen C, Deviren A, et al. Familial erythromelanosis follicularis and chromosomal instability. J Eur Acad Dermatol Venereol 2001;15:150-2.  Back to cited text no. 4
    
5.
Ermertcan AT, Oztürkcan S, Sahin MT, Türkdogan P, Saçar T. Erythromelanosis follicularis faciei et colli associated with keratosis pilaris in two brothers. Pediatr Dermatol 2006;23:31-4.  Back to cited text no. 5
    
6.
Sardana K, Relhan V, Garg V, Khurana N. An observational analysis of erythromelanosis follicularis faciei et colli. Clin Exp Dermatol 2008;33:333-6.  Back to cited text no. 6
    
7.
Judge MR, McLean WH, Munro CS. Disorders of keratinization. In: Burns T, Breathnach S, Cox N, Griffiths C, editors. Rook’s Textbook of Dermatology. 8th ed., Vol. 1. U.K: Wiley-Blackwell; 2010. p. 19.72-19.73.  Back to cited text no. 7
    
8.
Silva RS, Fonseca JC, Obadia D. Case for diagnosis. Erythromelanosis follicularis faciei et colli. An Bras Dermatol 2010;85:923-5.  Back to cited text no. 8
    
9.
Kurita M, Momosawa A, Ozaki M, Ban I, Harii K. Long-pulsed dye laser for the treatment of erythromelanosis follicularis faciei: Report of two clinical cases. Dermatol Surg 2006;32:1414-7.  Back to cited text no. 9
    
10.
Karakatsanis G, Patsatsi A, Kastoridou C, Chaidemenos G, Sotiriadis D. Erythromelanosis follicularis faciei et colli: Case reports of bilateral lesions in 2 females. Cutis 2007;79:459-61.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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