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On-line version ISSN 1806-4841
An. Bras. Dermatol. vol.85 no.6 Rio de Janeiro Nov./Dec. 2010
WHAT IS YOUR DIAGNOSIS?
Roberto Souto da SilvaI; João Carlos Macedo FonsecaII; Daniel ObadiaIII
IMD- - Preceptor of the Dermatological Service of the Pedro Ernesto University Hospital (HUPE- UERJ) - Rio de Janeiro (RJ), Brazil
IIPhD degree in Dermatology - Assistant Professor of Dermatology of the Pedro Ernesto University Hospital (HUPE- UERJ) - Rio de Janeiro (RJ), Brazil
IIIPost-graduation course in Dermatology from Uerj-HUPE - Preceptor of the Dermatological Service of the Pedro Ernesto University Hospital (HUPE- UERJ) - Rio de Janeiro (RJ), Brazil
Erythromelanosis follicularis faciei et colli is a rare disease of unknown etiology characterized by symmetric erythematous brownish hyperpigmentation on the frontal, malar and temporal areas associated with involvement of the pilary follicle. It is common to present pilar keratosis on the shoulders and neck. The three main clinical characteristics are: erythema (with or without telangiectasias), discrete follicular papules and brownish hyperpigmentation.
Keywords: Alopecia; Hyperpigmentation; Keratosis
HISTORY OF THE DISEASE
Eleven-year- old boy with rough skin on the facial region associated with darkening and gradual loss of the eyebrow since he was three years old. Within this period similar lesions,on the shoulders and arms, associated with pruridus and xerosis appeared. Emollients were used presenting partial improvement of xerosis and pruridus but without improvement of hyperpigmentation. Exam sho wed keratosis pilaris on the frontal region, temporal, malar and of the neck associated with brownisherythematous lesions delimited on the facial region (Figure 1). The patient also presented alopecia of the eyebrows with discreet papules without atrophy or scar on the area. (Figure 2). In the deltoid and tricipital regions there was keratosis pilaris with hyperpigmentation (Figure 3). Histopahtologic exam (Figure 4) revealed orthokeratotic hyperkeratosis, follicular "plug" and brownish pigmentation on the basal layer. On the papillary dermis there are some dilated vases, rare melanophages and superficial perivascular inflammatory infiltrate formed by lymphocytes and histiocytes.
Erythromelanosis follicularis faciei et colli is a pigmentary disease associated with erythema and envolvement of the pilary follicle. Primarily, it is observed in adolescents. However, it also affects children and young adults, with preference for the male sex. Bilateral distribution is the main characteristic but unilateral cases were described.1 It was first described in 1960, by Kitamura and collaborators in Japan. Its origin is unknown but there is an hereditary component (autossomal recessive) that seems to be interfering with its genesis.2
Clinically it presents three characteristic findings: delimited erythema, hyperpigmentation and follicular papules. Brownish delimited hyperpigmentation occurs in preauricular and maxillary areas symmetrically, affecting also the region of the neck and the temporal region. Follicular papules3 occur associated with pigmentary alteration erythema. In some cases diascopy evidences brownish colour caused by compression of telangiectasia. However, the absence of such vascular alterations does not influence the diagnosis of EFFC. Another common characteristic is alopecia in the region of the vellus hair which does not occur so frequently in the terminal hair areas such as beard and scalp. 4. Associated keratosis pilaris on the arms and shoulders can be found and to some authors the EFFC would be a variant of Keratosis pilaris rubra:4,5
Differential diagnoses include Athrophoderma Vermiculatum, "ulerythema ophryogenes", poikiloderma of Civatte, and keratosis pilaris.4,6,7 Athrophoderma Vermiculatum is characterized by atrophy with aspect of honeycomb in the malar regions. "Ulerythema ophryogenes" presents erythema with alopecia, atrophy and scar, involving the eyebrows. Both conditions differ from EFFC due to the presence of atrophy and scar.6 Poikiloderma of Civatte is observed in middle aged women as reticulated dyschromia with atrophy and erythema affecting preferably photoexposed areas and sparing the submental region. The absence of follicular papules associated with starting age and lesions distribution is different from EFFC. Keratosis pilaris faciei does not present well delimited area of hyperchromia although erythema can be seen around the follicles.
Histopathology is not diagnostic although it reveals: hyperkeratosis, plugg follicular, discreet hyperchromia of the basal membrana and dilatation of the superficial dermis vases. The pillary follicles are enlarged with perianexial inflammatory infiltrate. 1,3,4
Treatment is not well defined but it should be avoided solarexposition as well as other sorces of ultraviolet light. Sunscreen is recommended1. Various topic keratolytics are used including urea cream (1020%), ammonium lactate 12%, tretinoin cream (0,050,1%) and combinations with hydroquinone 4%. Peelings with salycylic acid (30%) can be used. Vitamin C analogues are an option due to their regulating mechanism of keratinization 8. In the most severe cases oral isotretinoin is used intermittently. Currently, the Pulsed Dyed Laser of 595nm is a therapeutic option to attenuate hyperpigmentation and erythema.9
Erythromelanosis follicularis faciei et colli (EFFC) is a rare disease for some authors but others consider it an underdiagnosed entity. Due to rarity or to underdiagnosis, less than 50 cases were described. Our case, apart from presenting clinical-pathological characteristics of EFFC, shows marked keratosis pilaris on the upper limbs and shoulders generating the discussion whether EFFC and keratosis pilaris rubra would be spectrum of the same disease.
1. Sardana K, Relhan V, Garg V, Khurana N. An observational analysis of erythromelanosis follicularis faciei et colli. Clin Exp Dermatol. 2007;33:333-6. [ Links ]
2. Acay MC. Erythromelanosis follicularis faciei et colli: A gentic disorder? Int J Dermatol. 1993;32:542. [ Links ]
3. Ermertcan AT, Oztürkcan S, Sahin MT, Türkdogan P, Saçar T. Erythrodermelanosis follicularis faciei et colli associated with keratosis piaris in two brothers. Pediatr Dermatol. 2006;23:31-34. [ Links ]
4. Augustine M, Jayaseelan E. Erythromelanosis follicularis faciei et colli: Relathionship with kertaosis pilaris. Indian J Dermatol Venereol Leprol. 2008;74:47-49. [ Links ]
5. Marqueling AL, Gilliam AE, Prendiville J, Zvulunov A, Antaya RJ, Sugarman J, et al Keratosis pilaris rubra: a common but underrecognized condition. Arch Dermatol. 2006;142:1611-16. [ Links ]
6. Luria RB, Conologue T. Athrophoderma Vermiculatum: A case report and review of the literatue on Keratosis Pilaris Atrophicans. Cutis. 2009;83:83-6. [ Links ]
7. Carvalho CV, Bastazini Jr I, Barreto JA, Duarte IB, Figueiredo RBP. Caso para diagnostico. Queratose folicular espinulosa decalvante. An Bras Dermatol. 2009;84:539-41 [ Links ]
8. Kragballe K, Steijlen PM, Ibsen HH, van de Kerkhof PC, Esmann J, Sorensen LH, et al. Efficacy, tolerability, and safety of calcipotriol ointment in disorders of keratinization. Arch Dermatol. 1995;131:556-560. [ Links ]
9. Kaune KM, Haas E, Emmert S, Schön MP, Zutt M. Successful Treatment of Severe Keratosis Pilaris Rubra with a 595-nm Pulsed Dye Laser. Dermatol Surg. 2009;35:1592-1595. [ Links ]
Mailing address: Approved by the Editorial Board and accepted for publication on 11.06.2010. * Work conducted at Pedro Ernesto University Hospital (Hupe-Uerj) - Rio de Janeiro (RJ), Brazil.
Roberto Souto da Silva
Praia João Caetano, 81, 602 Bairro Ingá
24210-405 Niterói, RJ, Brazil
Conflict of interest: None
Financial funding: None
Approved by the Editorial Board and accepted for publication on 11.06.2010.
* Work conducted at Pedro Ernesto University Hospital (Hupe-Uerj) - Rio de Janeiro (RJ), Brazil.