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Year : 2017  |  Volume : 18  |  Issue : 3  |  Page : 252-254

Atrophy in erythromelanosis follicularis faciei et colli: Is it coincidence or association?


Department of Dermatology, Jawahar Lal Nehru Medical College, Aligarh Muslim University, Aligarh, Uttar Pradesh, India

Date of Web Publication7-Jun-2017

Correspondence Address:
Tasleem Arif
Al-Rahman Apartment, ground floor, Behind Zakaria market, New Sir Syed Nagar, Civil lines, Aligarh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijpd.IJPD_114_16

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How to cite this article:
Arif T. Atrophy in erythromelanosis follicularis faciei et colli: Is it coincidence or association?. Indian J Paediatr Dermatol 2017;18:252-4

How to cite this URL:
Arif T. Atrophy in erythromelanosis follicularis faciei et colli: Is it coincidence or association?. Indian J Paediatr Dermatol [serial online] 2017 [cited 2017 Sep 25];18:252-4. Available from: http://www.ijpd.in/text.asp?2017/18/3/252/206054

Sir,

Erythromelanosis follicularis faciei et colli (EFFC) is a rare disorder of unknown etiology characterized by erythema, hyperpigmentation and follicular plugging on the face.[1] By 2013, some 55 cases of the disease have been reported.[2] Although atrophy has been mentioned in keratosis pilaris (KP), atrophic scars in EFFC have rarely been reported. In this article, a case of EFFC with atrophic scars is described.

A 15-year-old boy, product of nonconsanguineous marriage presented with erythema, pigmentation, and roughness of the cheeks since early childhood. There is a history of burning sensation and an increase in the erythema on exposure to Sun. There is no similar history in any of his family members. He denied any application of topical medications except emollients. During childhood, he developed multiple depressed scars on the left side of face spontaneously. His caregivers denied any history of trauma to the affected area of face. There is no history of loss of hair from any part of the body. There is no history of blistering on exposure to sunlight. Physical examination of the patient showed erythema, brownish hyperpigmentation and keratotic follicular papules present on the cheeks, preauricular and submandibular areas in a bilaterally symmetrical distribution [Figure 1] and [Figure 2]. On the left cheek, he had multiple atrophic scars in a linear and stellate pattern [Figure 1] and [Figure 2]. In addition, there were multiple keratotic follicular papules present on the shoulders and extensor aspects of upper arms with perifollicular erythema at places [Figure 3]. Examination of hair, nails, and mucous membranes was normal. Systemic examination was unremarkable. Biopsy of the skin was not performed. Based on history and further supported by clinical examination, a diagnosis of EFFC with atrophy was made. Since atrophy in EFFC has rarely been reported which inspired the author to report the same.
Figure 1: Erythromelanosis follicularis faciei et colli with atrophic scars in a 15-year-old boy. Note that the eyebrows are normal

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Figure 2: Close view of the erythromelanosis follicularis faciei et colli with atrophy. There is erythema, pigmentation, keratotic follicular papules associated with multiple atrophic scars

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Figure 3: Keratosis pilaris on upper arms with perifollicular erythema at places

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EFFC, though not so rare, has been rarely reported. It classically presents with well-defined hyperpigmentation, erythema and keratotic follicular papules on the cheeks and preauricular areas. This disease was first described in Japanese patients.[1] Some researchers have proposed an autosomal recessive pattern of inheritance while familial cases have been reported by others. Recently, EFFC has been believed to be a polyetiological disorder where familial and environmental factors play a role in its causation and the possibility of being a chromosomal instability syndrome.[3]

The association of EFFC with atrophy has not been reported in the literature till date. Sardana et al. have reported five patients of EFFC from India. None of them had associated atrophy.[4] Similarly, Ermertcan et al. have described EFFC in two Turkish brothers having KP involving shoulders and upper arms. There was also no associated atrophy.[5] Recently Rather et al. described 14 patients of EFFC from Kashmir valley with none of them having any associated cutaneous atrophy.[6] However, in this case, the presence of atrophic scars with EFFC makes it a unique case presentation and hence reported. Whether this coexistence is merely incidental or there really has some association between the two needs more robust reports.

The relation of EFFC with KP has been a matter of debate. Some researchers suggest that EFFC to be a clinical variant of KP as there has been a frequent association with KP on the arms and overlap with ulerythema ophryogenes has also been reported.[7] However, EFFC is differentiated from KP by the presence of pigmentation and extension onto the neck. EFFC with atrophy needs to be differentiated from two other related disorders where keratotic papules occur with atrophic scars. In ulerythema ophryogenes, there is prominent facial erythema with the involvement of scalp and eyebrows.[7] However, in our case eyebrows and scalp were normal. Atrophoderma vermiculatum affects the cheeks and preauricular regions and is characterized by honeycomb type of atrophic scars associated with keratotic papules.[7] However, a well-defined erythema and pigmentation in EFFC easily differentiates it from the former. Furthermore, the scars in the present case were not honeycomb type rather stellate or linear. Poikiloderma of civatte also comes under the differential diagnosis. It is usually seen in middle-aged women as erythema, reticulate dyschromia, and atrophy. It usually involves photoexposed areas and spares the submental region. However, the absence of follicular papules and late onset differentiates it from the EFFC with atrophy.[8] Thus, author concludes that atrophy can occur in EFFC which is distinct from simple KP with atrophy. However, more reports and studies are required to establish a definite association between the two.

Declaration of patient consent

The author certifies that he has 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

Nil.

Conflicts of Interest

There are no conflicts of interest.

 
  References Top

1.
Arif T. Generalized keratosis pilaris associated with erythromelanosis follicularis faciei et colli: A rare coexistence. Indian J Paediatr Dermatol 2015;16:149-51.  Back to cited text no. 1
  [Full text]  
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.
Tuzun Y, Wolf R, Tuzun B, Ozdemir M, Demirkesen C, Deviren A, et al. Erythromelanosis follicularis and chromosomal instability. J Eur Acad Dermatol Venereol 2001;15:150-2.  Back to cited text no. 3
    
4.
Sardana K, Relhan V, Garg V, Khurana N. An observational analysis of erythromelanosis follicularis faciei et colli. Clin Exp Dermatol 2007;33:333-6.  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.
Rather S, Yaseen A, Mukhija M. Erythromelanosis follicularis faciei et colli – A cross-sectional, descriptive study. Indian J Dermatol 2016;61:308-13.  Back to cited text no. 6
[PUBMED]  [Full text]  
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. UK: Wiley-Blackwell; 2010.P. 19.72-19.73.  Back to cited text no. 7
    
8.
Souto RS, Fonseca JC, Obadia D. Erythromelanosis follicularis “faciei et colli”: A rare or an underdiagnosed disease? An Bras Dermatol 2010;85:923-5.  Back to cited text no. 8
    


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