Erythromelanosis Follicularis Faciei Research Articles

A new diagnostic paradigm for Erythromelanosis Follicularis Faciei et Colli: Dermoscopic and reflectance confocal microscopic features

Introduction: Erythromelanosis Follicularis Faciei et Colli is a rare variant of Kerastosis pilaris, characterized by a clinical triad of erythema, hyperpigmentation and follicular papules involving bilaterally cheeks, preauricular and maxillary areas of young male or, more rarely, female patients Case presentation: A 21-year-old Caucasian woman presented symmetrical patches of hyperpigmentation, erythema with microscopic telangiectasias and tiny follicular papules involving cheeks, preauricular and maxillary areas, forehead, pinna and neck since childhood. Lesions were asymptomatic but caused profound cosmetic disfigurement and had a negative impact on her social life. The patient underwent a non-invasive diagnostic procedure with epiluminescence and confocal microscopy. Dermoscopic examination revealed prominent yellowwhitish follicular keratotic plugging over a reddish background with short fine telangiectasia in the perifollicular and interfollicular areas. Furthermore, RCM features displayed the presence of enlarged the presence of enlarged follicular openings, absence of demodex mites within the follicles and slight infundibular hyperkeratosis. At the level of the epidermal-dermal junction hyperpigmentation of the epithelial basal cell layer and dilation of dermal capillaries inside dermal papillae were observed, correlating to typical histological features of EFFC. Our patient benefited from the topical use of tretinoin 0.05% cream, with attenuation of the instrumental pictures. Conclusion: Characteristic plugging over a reddish background and morphological features revealed by RCM may be helpful for assessment and monitoring in patients with EFFC. Starting from the observation of this case we would like to underline the importance of a noninvasive diagnostic procedure and an effective therapy for such a rare condition. Keywords: Erythromelanosis Follicularis Faciei et Colli; Erythromelanosis Follicularis Faciei diagnosis; Dermoscopy; In vivo reflectance confocal microscopy.

Clinicopathological and dermoscopic correlation of Erythromelanosis follicularis faciei et coli in skin of color

Erythromelanosis follicularis faciei et coli (EFFC) is a rare disorder of follicular keratinization of unknown etiology, which is first described in 1960 by Kitamura. The disease is characterized by the triad of hyperpigmentation, erythema, and follicular papules1. As EFFC can be easily mistaken for other similar pigmentary, hyperkeratotic disorders, clinico pathological correlation with dermoscopic appearance are important in preventing diagnostic errors.
 Herein we present correlation of clinicopathologic and dermoscopic appearance of EFFC in two Asian individuals.

Dermoscopy of acquired pigmentary disorders: a comprehensive review.

Dermoscopy has traditionally been used for the diagnosis of neoplasms and more recently in the evaluation of inflammatory conditions. Recent observational studies have suggested a role for dermoscopy in identifying and differentiating acquired pigmentary disorders. This comprehensive review will summarize the growing literature on the use of dermoscopy for pigmentary disorders. A literature review was performed on PubMed dating from inception to October 2020. The following pigmentary disorders were included in this study: melasma, solar lentigines, poikiloderma of Civatte, exogenous ochronosis, lichen planus pigmentosus, erythromelanosis follicularis faciei et colli, pigmented contact dermatitis, Riehl's melanosis, postinflammatory hyperpigmentation, erythema dyschromicum perstans, ashy dermatosis, confluent and reticulated papillomatosis, acanthosis nigricans, pityriasis versicolor, tinea versicolor, idiopathic guttate hypomelanosis, and vitiligo. Search terms used included each pigmentary disorder along with the terms "dermoscopy" or "dermatoscopy." Relevant case reports and case series were included. Many pigmentary disorders have unique and distinguishable features on dermoscopy. Given that these disorders can be clinically challenging for clinicians and emotionally distressing for patients, dermoscopy provides an additional, useful tool in the evaluation and assessment process.

Keratosis Pilaris and its Subtypes: Associations, New Molecular and Pharmacologic Etiologies, and Therapeutic Options.

Keratosis pilaris is a common skin disorder comprising less common variants and rare subtypes, including keratosis pilaris rubra, erythromelanosis follicularis faciei et colli, and the spectrum of keratosis pilaris atrophicans. Data, and critical analysis of existing data, are lacking, so the etiologies, pathogeneses, disease associations, and treatments of these clinical entities are poorly understood. The present article aims to fill this knowledge gap by reviewing literature in the PubMed, EMBASE, and CINAHL databases and providing a comprehensive, analytical summary of the clinical characteristics and pathophysiology of keratosis pilaris and its subtypes through the lens of disease associations, genetics, and pharmacologic etiologies. Histopathologic, genomic, and epidemiologic evidence points to keratosis pilaris as a primary disorder of the pilosebaceous unit as a result of inherited mutations or acquired disruptions in various biomolecular pathways. Recent data highlight aberrant Ras signaling as an important contributor to the pathophysiology of keratosis pilaris and its subtypes. We also evaluate data on treatments for keratosis pilaris and its subtypes, including topical, systemic, and energy-based therapies. The effectiveness of various types of lasers in treating keratosis pilaris and its subtypes deserves wider recognition.

Erythromelanosis follicularis faciei et colli: A clinicoepidemiologic study.

We describe 25 cases of erythromelanosis follicularis faciei et colli from India. The male:female ratio was 5.25:1 and the average age of onset was 12.3 years. The cheeks, preauricular area, and submandibular region were the sites most commonly affected. Keratosis pilaris was seen in 22 (88%) of the patients.

Erythromelanosis follicularis faciei et colli: A first female case report from the outskirts of Libya and literature review

Erythromelanosis follicularis faciei (EFF) is a rare sporadic condition of unclear etiology and is distinctive by well-demarcated reddish-brown patches and follicular papules that appear often on the face and seldom on the neck. Herein, I report a case of a 15-year-old female who had asymptomatic facial skin discoloration since early childhood. Skin examination revealed confluent nonscaly rough brownish red patches on both cheeks without telangiectasia and a barely thick scaly brownish patch over her entire forehead and both eyebrows with follicular plugging. A summary of preceding reports of EFF in the literature is presented in this report.

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.

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.

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 exploratory split‐face study of a dual‐wavelength laser system on erythromelanosis follicularis faciei in Chinese population

Erythromelanosis follicularis faciei (EFF) is clinically characterized by well-demarcated erythema, hyperpigmentation and follicular papules. No satisfactory therapy has been widely accepted. This study was aimed to evaluate the efficiency and complications of a dual-wavelength laser system in the treatment of EFF in Chinese population. Twelve Chinese patients with EFF were enrolled in this study. One side of the face was randomized to receive five successions of treatments at intervals of 6-8weeks, with the other side spared as control. The parameters were set as follows: energy densities of 6.5-7.5J/cm(2) with pulsed dye laser (PDL) and 35-50J/cm(2) with Nd:YAG; pulse duration of 0.5ms with PDL and 15ms with Nd:YAG; a delay time of 100ms between delivery of the two wavelengths. The efficacy was evaluated using subjective assessment and non-invasive measurement. After five sessions of treatment, over 40% patients achieved more than 50% (moderate or significant) improvement. The efficacy maintained at 3-month follow-up. The values of erythema index and melanin index on treated side were significantly less than those on untreated side after sessions of treatments. Adverse effects of treated side were limited. Using this split-face module, dual-wavelength laser system treatment is proved to be suitable for Chinese EFF. Adverse effects were minimal.

Caso para diagnóstico

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.

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.

Erythromelanosis follicularis faciei &lt;i&gt;et&lt;/i&gt; colli: Relationship with keratosis pilaris

Erythromelanosis follicularis faciei et colli (EFF) is an unusual condition characterized by the triad of hyperpigmentation, follicular plugging and erythema of face and neck. This is less common in women and familial case reports are few. We report EFF in three siblings in an Indian family, two of whom are females. The possibility of this condition being genetically related to keratosis pilaris as well as being a variant of keratosis rubra pilaris is also discussed.

Long-Pulsed Dye Laser for the Treatment of Erythromelanosis Follicularis Faciei: Report of Two Clinical Cases

Two Japanese men complained of redness of their jowls and requested treatment of those areas. Examination revealed welldemarcated reddish-brown pigmentation with granular texture on both sides of the cheeks. The redness of the lesion was mainly caused by dilation of superficial vessels and vanished when pressure was applied. Both patients were diagnosed as EFF by the classical triad, which is composed of erythema, hyperpigmentation, and follicular prominence.

Long-Pulsed Dye Laser for the Treatment of Erythromelanosis Follicularis Faciei

Long-Pulsed Dye Laser for the Treatment of Erythromelanosis Follicularis Faciei

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

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.

Erythromelanosis Follicularis Faciei: First Case Report from Saudi Arabia

Erythromelanosis Follicularis Faciei: First Case Report from Saudi Arabia

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.

Erythromelanosis follicularis faciei in women

Erythromelanosis follicularis faciei is an infrequently diagnosed disorder thought to be more common in men, with only seven reported cases in women. This condition consists of the clinical triad of well-demarcated erythema, hyperpigmentation, and follicular plugging on the face. We describe two additional female patients with this condition and present a review of the literature.

Unilateral erythromelanosis follicularis faciei et colli in a young girl

Erythromelanosis follicularis faciei et colli is uncommon. Fewer than 20 cases have been reported. Except for one case in a woman and one case of unilateral distribution, all other reported cases have been bilateral in young male patients. We report the first case of unilateral distribution in a white girl.