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Cutaneous and Ocular Signs of Childhood Rosacea
Mélanie Chamaillard, MD;
Bruno Mortemousque, MD;
Franck Boralevi, MD;
Claudia Marques da Costa, MD;
Farida Aitali, MD;
Alain Taïeb, MD;
Christine Léauté-Labrèze, MD
Arch Dermatol. 2008;144(2):167-171.
ABSTRACT
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Objectives To describe the clinical features of cutaneous and ocular manifestations of childhood rosacea, to propose diagnostic criteria, and to emphasize the possible severity of ocular complications in this age group.
Design Retrospective study.
Setting Tertiary referral center.
Patients Children aged 1 to 15 years who had received a diagnosis of cutaneous and/or ocular rosacea and were seen between January 1, 1996, and December 31, 2005.
Results Of 20 patients, 11 had ocular and cutaneous rosacea, 6 had isolated cutaneous involvement, and 3 had isolated ocular involvement. Dermatologic examination results were sufficient to diagnose rosacea in 12 of the patients (60%). The most common presentation was a papulopustular eruption on a telangiectatic background. In 11 patients (55%), ocular involvement preceded the skin eruption. Among the ophthalmologic manifestations, chalazions and blepharoconjunctivitis were the main presenting symptoms; keratitis was observed in 4 patients and corneal ulcers in 2. Ten patients were treated with oral metronidazole. Intermittent treatment for at least 3 months was used to avoid neurologic toxic effects and to achieve complete remission.
Conclusion Although rare, childhood rosacea should be recognized because of the possible severity of ocular involvement.
INTRODUCTION
Rosacea is a chronic cutaneous condition of vasomotor instability that is characterized by facial erythema of the central convex areas of the face. In adults, 1 or more of the following primary features (with distribution in the central face) is necessary for the diagnosis of rosacea1: flushing, nontransient erythema, papules and pustules, or telangiectasia. Secondary features that are supportive characteristic findings but not necessary for diagnosis include burning or stinging of the skin, red plaques, a dry appearance, facial edema, phymatous changes, and ocular manifestations.2 The relative abundance of these associated findings will dictate which of 4 subtypes is present: erythematotelangiectatic, papulopustular, phymatous, or ocular rosacea.1-3 In addition to these 4 subtypes, experts recognize only 1 variant: granulomatous rosacea.1
Rosacea is a disease of fair-skinned young to middle-aged adults; its prevalence in children is rare.4-7 However, childhood rosacea is probably underreported3 because of the lack of diagnostic criteria in this age group. We studied 20 young patients who had received a diagnosis of cutaneous and/or ocular rosacea. Our primary aim was to describe the clinical cutaneous and ocular manifestations of rosacea in childhood and adolescence; our secondary aims were to propose diagnostic criteria for childhood rosacea and to make dermatologists more aware of the possible associated ocular complications.
METHODS
We reviewed the medical records of children aged 1 to 15 years who had received a diagnosis of rosacea and were seen in the Pediatric Dermatology Unit of Bordeaux Children's Hospital between January 1, 1996, and December 31, 2005 (5000 external visits per year). All children with suspected cutaneous rosacea were examined by a pediatric ophthalmologist (B.M. and/or F.A.), and all children with suspected ocular rosacea were examined by a pediatric dermatologist (F.B., A.T., and/or C.L.L.). Two dermatologic and/or ophthalmologic criteria were needed for inclusion in our study. Possible skin symptoms included facial erythema or flushing that subsided within minutes; recurrent papular or pustular eruptions of the central convex areas of the face, including the forehead, cheeks, nose, and perioral and periocular skin; and telangiectases. Possible ocular symptoms included red eye with blepharoconjunctivitis, episcleritis or keratitis, palpebral telangiectases, meibomian gland inflammation, and burning or irritation due to ocular dryness.
Informed oral consent was obtained from the parents. Data extracted from the medical records included the patient's age, sex, and skin phototype; family history of rosacea; patient history of cutaneous and ocular manifestations; the progression and characteristics of the disease; the natural history of each manifestation; and the topical or systemic treatment regimen, especially the use of topical corticosteroids.
RESULTS
Eleven girls and 9 boys were identified as having cutaneous and/or ocular rosacea (eTable). The mean age at presentation was 4.6 years (median age, 42 months). Of the 20 patients, 17 were white. Six patients had a familial history of rosacea and 13 had skin phototypes I or II. One of our patients with severe ocular rosacea had a history of idiopathic facial aseptic granuloma.8-9 Among these 20 patients, 11 had both ocular and cutaneous rosacea, 3 had isolated ocular rosacea, and 6 had isolated cutaneous rosacea.
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eTable. Summary of Patient Characteristics
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Three dermatologic presentations were found: papulopustular rosacea, telangiectasia, and granulomatous rosacea. Papulopustular rosacea was observed in most of the children (14 of 17 patients) (Figure 1). The lesions developed on a background of associated erythema and telangiectasia on the cheeks and chin. The telangiectatic form, with or without flushing, was present in 4 of 17 patients (Figure 2). Facial flushing was observed in response to various stimuli, such as exposure to heat, and episodes of erythema recurred and lasted longer than normal physiologic flushing, which typically subsides within minutes. Granulomatous rosacea was seen in 2 patients and consisted of brown infiltrated papules of the perioral and periocular areas (Figure 3). Five patients had received topical corticosteroids as first-line treatment for atopic dermatitis.
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Figure 1. Typical papulopustular form of rosacea on a telangiectatic background in an 8-year-old girl.
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Figure 2. Boy with idiopathic facial aseptic granuloma at 3 years of age (A) and with the telangiectatic form of rosacea with flushing at 5 years of age (B). The boy's eyes were closed in the later photograph because of severe photophobia due to keratoconjunctivitis.
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Figure 3. Granulomatous form of rosacea in a 9-year-old boy associated with severe ocular rosacea (keratoconjunctivitis with corneal ulcerations and visual impairment) before (A) and after (B) 2 months of treatment with oral metronidazole. In the earlier photograph, the facial dermatosis was modified by the application of topical corticosteroids for several months. Skin pitting is visible in the later photograph.
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In 12 of the patients (60%), a rosacea diagnosis was made initially because of cutaneous signs. In 5 cases, systematic ophthalmologic examination results helped to diagnose ocular rosacea. Nevertheless, the parents indicated that the clinical signs of eye involvement had preceded the cutaneous involvement (ie, stinging in an eye, photophobia, and bilateral chalazions). Among the 11 patients with both cutaneous and ocular rosacea, only 3 had cutaneous manifestations before the ocular involvement.
The manifestations in the 14 children with ocular rosacea are given in the following tabulation:
The most common ophthalmologic finding was meibomian gland inflammation, which manifested as chalazions (Figure 4) and ocular hyperemia (Figure 5). Ten patients had minor ocular involvement, but 4 had severe ocular manifestations: 2 patients had keratitis complicated by a corneal ulcer, and 1 of the 2 patients with granulomatous rosacea, who also had keratitis, had severe blepharoconjunctivitis and a corneal ulcer (the patient in Figure 3).
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Figure 4. Ocular rosacea in a 3-year-old girl with relapsing bilateral chalazions.
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Figure 5. Ocular and eyelid involvement by rosacea is manifested as ophthalmic hyperemia associated with inferior limbic erythema, corneal neovascularization, and eyelid telangiectases.
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Children with minor cutaneous involvement were treated with topical metronidazole or topical niacinamide (9 patients). In cases of benign ocular involvement, eyelid care included massage with lukewarm water and administration of isotonic sodium chloride solution 3 or 4 times a day. Children with severe ocular involvement and/or both cutaneous and ocular rosacea received systemic therapy associated with topical care. Tetracyclines (eg, doxycycline) were used for patients older than 12 years; in younger children, systemic metronidazole was given. Use of erythromycin produced only partial remission when given as a first-line treatment in our initial cases; thus, we chose to use metronidazole, at least 20 mg/kg/d, in 10 children, with good efficacy and tolerance. To avoid neurologic toxic effects, short courses of treatment were preferred to continuous administration of the drug. We usually treated the patient for 1 month at the initial dosage (30 mg/kg/d; if not well tolerated, then 20 mg/kg/d). If complete remission was achieved, the treatment was continued for 2 more months at half the initial dosage. Nevertheless, treatment for at least 3 months at the full dosage was necessary in 3 of the children because of relapsing or persistent disease.
COMMENT
An association of ocular symptoms and facial inflammatory dermatosis in a child should lead to a suspicion of rosacea.4-7,10 Despite the absence of validated diagnostic criteria in children, the clinical features of childhood rosacea seem mostly similar to those found in adults.5 We clearly identified children with facial flushing, persistent telangiectasia, and/or papulopustular eruptions on the convex areas of the face, but no phymatous rosacea was found in this age group. The most frequent papulopustular form can be difficult to differentiate from juvenile acne; however, comedones are lacking in rosacea and both telangiectasia and flushing are absent in acne. Of course, in teenagers, acne and rosacea may coexist.4 Clinically, demodicosis can mimic rosacea; the facial eruption is characterized by erythema associated with papulopustules.11 However, demodicosis is rare in immunocompetent children, and skin scrapings usually show Demodex mites in abundance. Telangiectatic rosacea should be differentiated from other causes of telangiectasia, which range from rare diseases such as hereditary poikilodermas to more common disorders such as poststeroidal skin atrophy. Granulomatous perioral dermatitis12-13 is similar to granulomatous rosacea in its semiology, chronic evolution, and sequelae (skin pitting). One of our patients (case 1, seen in Figure 3) had extensive granulomatous perioral dermatitis and associated ocular rosacea, which suggested merging these entities. Another common dermatosis of childhood should also be discussed in the differential diagnosis because one of our patients with severe ocular rosacea had a history of idiopathic facial aseptic granuloma8-9 (case 10, seen in Figure 2). This observation suggests that idiopathic facial aseptic granuloma, which is usually seen in younger patients,9 may belong to the spectrum of rosacea.
The clinical characteristics of childhood ophthalmologic rosacea have rarely been addressed.6-7,10 A study published in 1969 found that 58% of the patients with rosacea had ocular manifestations.14 Ocular manifestations of pediatric rosacea are frequently underdiagnosed or misdiagnosed, leading to ophthalmologic complications.4, 10 Ocular involvement includes blepharitis6 with meibomian gland inflammation and relapsing chalazions, ocular redness, photophobia, episcleritis or keratoconjunctivitis, and, in rare cases, corneal ulcers.10, 15-16 In clinical practice, the diagnosis of rosacea is often based on cutaneous findings, even if ocular involvement is present earlier.4 Ocular symptoms antedated skin symptoms in 11 of the patients in our series (55%) vs only 20% in previous studies.14-16 In our series, most of the children had minor ocular involvement, but 2 patients had corneal ulcers, 4 had keratitis, and 1 had severe blepharoconjunctivitis. If not diagnosed, corneal ulcers can lead to severe ocular infection and visual impairment. Ophthalmologic examination should thus be proposed for all children with suspected cutaneous rosacea.
The pathophysiologic mechanisms of rosacea remain unclear, but various factors have been implicated in both ocular and cutaneous rosacea, such as climatic exposures, vascular changes, matrix degeneration, pilosebaceous unit abnormalities or microbial organisms, and, more recently, inflammatory mediators.1-3 A recent study17 has demonstrated that patients with stye during childhood had a predisposition for the development of rosacea in adulthood, underlying the close relationship between ocular and cutaneous inflammation. In ocular rosacea, a meibomian gland dysfunction leads to thickened secretions, glandular dropout, and thickened eyelid margins.10 This dysfunction could be secondary to hyperemia consecutive to facial and angular venous dilation or secondary to increased glandular production of free fatty acid due to bacterial lipases. Another recent study18 has shown that increasing facial temperature due to vasodilation could switch the secreted protein profiles of bacterial commensal agents, such as Staphylococcus epidermidis. In addition to these factors, topical corticosteroids, even at the lowest dosage, may favor the development of rosacea in susceptible children.19
Clinically, childhood rosacea is an inflammatory disease.4 Tetracyclines, which are commonly used in the treatment of adults, are contraindicated for children younger than 8 years. Several authors have reported success using oral erythromycin.4-7 However, in our experience, the therapeutic results were not good, with early relapses. Kligman16 reported that metronidazole was as effective as tetracycline and achieved longer-lasting remissions. We also noted a frank success in 10 patients with severe cutaneous rosacea and/or severe ocular manifestations after we administered a dosage of at least 20 mg/kg/d. Treatment was maintained for 1 month until clinical remission, but, in 8 cases, a 3- to 6-month treatment was required at a lower dosage to achieve long-term remission. To avoid the neurologic adverse effects of metronidazole, intermittent treatment is recommended. Topical treatments can be used in mild rosacea. Topical metronidazole, azelaic acid, or niacinamide is helpful, with good clinical tolerance. Local treatment of ocular rosacea consisted of eyelid hygiene and topical erythromycin or metronidazole ophthalmic gel.4, 10
In conclusion, the most frequent presentation of childhood rosacea consists of a papulopustular eruption of the facial convex areas, associated with facial flushing or erythema in a light-skinned child. The clinical criteria used in adults can be applied to children. However, if only 1 criterion is sufficient for a diagnosis of rosacea in adults,1 we believe that at least 2 criteria should be present in children (Table). If a diagnosis of cutaneous rosacea is suspected, an ophthalmologic follow-up is necessary to detect ocular involvement and to prevent complications such as keratitis and corneal ulcers.
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Table. Proposed Dermatologic and Ophthalmologic Criteria for Childhood Rosaceaa
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AUTHOR INFORMATION
Correspondence: Christine Léauté-Labrèze, MD, Unité de dermatologie pédiatrique, Place Amélie Raba-Léon, 33 076 Bordeaux CEDEX, France (christine.labreze{at}chu-bordeaux.fr).
Accepted for Publication: May 5, 2007.
Author Contributions: Dr Léauté-Labrèze had full access to all of the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis. Study concept and design: Chamaillard and Léauté-Labrèze. Acquisition of data: Chamaillard, Mortemousque, Boralevi, Marques da Costa, Taïeb, and Léauté-Labrèze. Analysis and interpretation of data: Chamaillard, Marques da Costa, Aitali, and Léauté-Labrèze. Drafting of the manuscript: Chamaillard, Boralevi, and Léauté-Labrèze. Critical revision of the manuscript for important intellectual content: Chamaillard, Mortemousque, Marques da Costa, Taïeb. and Léauté-Labrèze. Statistical analysis: Chamaillard. Administrative, technical, and material support: Chamaillard. Study supervision: Taïeb and Léauté-Labrèze.
Financial Disclosure: None reported.
Additional Contributions: We are indebted to our patients and their families.
Author Affiliations: Pediatric Dermatology Unit, National Reference Center for Rare Skin Disorders, Bordeaux Children's Hospital (Drs Chamaillard, Boralevi, Marques da Costa, Taïeb, and Léauté-Labrèze), and Department of Ophthalmology, National Reference Center for Keratoconus (Drs Mortemousque and Aitali), Bordeaux, France.
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