Erythromelanosis Follicularis Faciei Et Colli Research Articles

Co-occurrence of Erythrosis Pigmentosa Mediofacialis and Erythromelanosis Follicularis Faciei et Colli Associated with Keratosis Pilaris in an Adolescent Female.

Erythromelanosis follicularis faciei et colli (EFFC) is a rare disease characterized by a triad of reddish-brown pigmentation, erythema and follicular papules localized on face and neck and is usually described in males. Erythrosis pigmentosa mediofacialis (also known as Brocq or erythrosis pigmentosa peribuccalis) is a similar disorder of the mediofacial area but with female predominance. We report a case of simultaneous occurrence of erythrosis pigmentosa peribuccalis and EFFC associated with keratosis pilaris in an adolescent female.

Erythromelanosis Follicularis Faciei et Colli - A Cross-sectional, Descriptive study.

Background:Erythromelanosis follicularis faciei et colli (EFFC) has always been reported as a rare disorder, and more data are needed to define its etiology and epidemiology.Objectives:To present a descriptive study of this disorder from Kashmir and present a review of literature on the same.Materials and Methods:A cross-sectional, descriptive study was conducted on 14 patients with clinical lesions suggestive of EFFC, presenting to our dermatology outpatient clinic between May 2013 and April 2015. After obtaining informed consent from all patients, the demographic and clinical data were collected and punch biopsies were taken which after being fixed in formalin were stained for hematoxylin and eosin. The study was approved by Institutional Review Board.Results:Age of the patients ranged from 12 to 35 years with a mean age of 24.8 years. Females outnumbered males in a ratio of 1.3:1. Family history was positive in one case, and one patient had unilateral presentation. The classical triad of erythema, pigmentation, and follicular papules were present in 100% patients. Telangiectasias were observed in 28.57% patients only. Classical sites described were involved in all the cases (100%). In addition, lesions were found to involve eyebrows (28.57%), forehead (28.57%), upper lips (14.28%), and pinna (21.42%). Keratosis pilaris was present in all patients (100%); most common sites affected were upper arms and thighs. Two (14.28%) had generalized distribution. Histopathology in all correlated well with clinical findings.Conclusion:EFFC has always been reported as a rare disorder, but we believe that it is not so. The reason could be either lack of awareness of the disease by the patient due to its generally asymptomatic nature or under-recognition by the physicians. Awareness about the disease on part of dermatologist and recognition of clinical presentation is important for correct diagnosis and to help find more effective therapeutic modalities.

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

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.

An observational analysis of erythromelanosis follicularis faciei et colli

Erythromelanosis follicularis faciei et colli (EFFC) is characterized by well-demarcated erythema, hyperpigmentation, and follicular papules. Since the orginal description, < 50 patients have been reported in the literature. Five cases of EFFC have been seen in our outpatient department in the past 5 years. All patients except one presented with the classic triad of erythema, brown pigmentation and follicular papules on the face and keratosis pilaris lesions on the trunk. In all cases, there was no family history for related disorders or history of atopy. Histopathological examination in all cases was consistent with the diagnosis of EFFC. Age at presentation ranged from 11 to 19 years, and the male female ratio was 4 : 1. Patients had the classic lesions of keratosis pilaris on the face (5/5), trunk (2/5) and legs (4/5). All patients except one had a background of erythema. One patient (patient 2) had itching and a history of photosensitivity. Of the patients who were on treatment (4/5), complete clinical regression was seen in one patient. To our knowledge, this is the first case series of EFFC from India, and we believe that EFFC is a common but infrequently reported condition.

Quantitative histopathologic findings of erythromelanosis follicularis faciei et colli

Even though only 33 cases of Erythromelanosis follicularis faciei et colli (EFFC) have been reported around the world, there have been no quantitative clinicopathological studies yet. The purpose of this study is to examine quantitatively clinical and histopathological evaluations of EFFC using image analysis system. Clinical grading of its severity was assessed. Many parameters of the horny layer, granular layer, epidermis and the dermal components were measured and compared to controls by image analysis. The correlation between the histological findings and clinical grading was assessed. Thickness and compactness of the horny layer was significantly increased in EFFC. There were no significant differences in the number of melanocytes and severity of pigmentation between EFFC and controls; however, melanization was slightly increased in EFFC. The percent area of the superficial blood vessels was significantly increased and correlated with visual grading of erythema. Diameters of hair shafts and outer root sheaths, and thickness of inner root sheaths were decreased. Basal layer pigmentation was correlated with the clinical features of pigmentation. This study supported the findings of hyperkeratosis and dilatation of superficial dermal blood vessels in EFFC. The pigmentation of the basal layer and the percent area of the inner spaces of the superficial dermal blood vessels may reflect the clinical severity more than the other parameters. Although follicular plugging is a characteristic clinical feature of EFFC, enlargement of hair follicles and hair shafts were not characteristic pathologic changes EFFC in our study.