Erythromelanosis Follicularis Faciei et Colli Associated with Keratosis Pilaris in Two Brothers

Abstract

Erythromelanosis follicularis faciei et colli is characterized by well-demarcated erythema, hyperpigmentation, and follicular papules. Since the original description, it has seldom been reported in the literature. We present two adolescent brothers who had this disorder associated with keratosis pilaris on the shoulders and the extensor surfaces of the arms. Dermatologic examination found brown-red pigmentation, erythema, and follicular papules on both maxillary, preauricular regions, and the cheeks. The lesions of the older brother were more prominent. Histopathologic examination of skin biopsy specimens taken from both brothers revealed hyperpigmentation of the basal layer, follicular plugging, dermal vascular dilatation and congestion, and perivascular inflammatory infiltration. We suggest that the coexistence of these two conditions in brothers implies a genetic inheritance and a possible relationship between the disorders.

Keratosis Pilaris Rubra

“…Erythromelanosis follicularis faciei et colli and KPA, another KP variant, have been considered by some authors to be variants of the same condition, 12 but EFFC lacks scarring. The similarities between KPR and EFFC, however, are striking, and photographs of EFFC in some reports show findings that are virtually identical to those in our cases, 16 which suggests that they are likely part of the same disease spectrum. The hyperpigmentation noted in EFFC may, at least in part, be related to skin pigmentation type, with darker skin types showing more evidence of hyperpigmentation.…”

“…Concomitant KP on the arms has been noted in a few patients with EFFC. [14][15][16] Features that differentiate EFFC from KPR are lack of reported involvement on the torso and the presence of hyperpigmentation. Erythromelanosis follicularis faciei et colli and KPA, another KP variant, have been considered by some authors to be variants of the same condition, 12 but EFFC lacks scarring.…”

Topical tacalcitol ointment can be a good therapeutic choice in erythromelanosis follicularis faciei et colli

“…1 It predominantly affects preauricular and maxillary regions and many therapeutic options have been tried. 2 However, the results were variable and the treatment of EFFC is still challenging. 2 Tacalcitol is a synthetic analog of vitamin D 3 and its successful treatment in psoriasis and other keratinization disorders such ichthyosis, Darier disease, and keratosis pilaris has been reported.…”

“…2 However, the results were variable and the treatment of EFFC is still challenging. 2 Tacalcitol is a synthetic analog of vitamin D 3 and its successful treatment in psoriasis and other keratinization disorders such ichthyosis, Darier disease, and keratosis pilaris has been reported. 3,4 As it is known that many patients with keratosis pilaris have concomitant EFFC in previous reports, 2 we considered tacalcitol as a new therapeutic possibility for EFFC.…”

Caso para diagnóstico

“…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 recommended 1 .…”