Rosacea is a chronic skin disease characterized by flushing, erythema, telangiectasia, papules, and pustules in the central face region.1 It most often affects middle-aged women (age range, 30–50 years).2 Rosacea is rare in the pediatric population, especially before puberty.3 There are 3 subtypes of pediatric rosacea: vascular, papulopustular, and ocular. Phymatous/rhinophymatous rosacea is only seen in the adult population.3 Recommendations for the management of pediatric rosacea heavily rely on data from retrospective chart reviews and case series.
Etiology of Pediatric Rosacea
Rosacea is thought to be a consequence of vasomotor instability in both adults and children. A family history of rosacea is sometimes reported in patients with pediatric rosacea.4 Patients often are sensitive to heat, sunlight, topical corticosteroids, spicy foods, hot liquids, and certain soaps and cleansers.1,3,4 In a review of the literature by Vemuri et al,5 the various reported triggers of rosacea include harsh climates that damage the blood vessels and dermal connective tissue, defects in the endothelium and dermal matrix, perivascular inflammation, orally ingested chemicals, changes in the flora of the hair follicles, excessive antimicrobial peptides, and the presence of free radicals. Overall, it is unclear which of these factors are triggers of pediatric rosacea.
The molecular basis of rosacea has been elucidated. It is well known that rosacea patients have higher levels of cathelicidins in the facial skin. Furthermore, they appear to have different processed forms of cathelicidin peptides compared to adults without rosacea, possibly due to changes in posttranslational processing.6 One such peptide, cathelicidin LL-37, also has been implicated in atopic dermatitis7 and psoriasis.8 Its role in rosacea appears to be multifaceted. Cathelicidin LL-37 helps to attract neutrophils, monocytes, and T lymphocytes, and also has antimicrobial properties; therefore, it plays a role in both the innate and adaptive immune systems.9 Cathelicidin LL-37 also has been implicated in inducing angiogenesis10 and suppressing dermal fibroblasts.11
Muto et al12 found that there is an increased number of mast cells in the dermis of patients with rosacea. Mast cells contribute to vasodilation, angiogenesis, and the recruitment of other inflammatory cells.12 Importantly, human mast cells are a source of cathelicidins including cathelicidin LL-37; these proteins play a vital role in the antimicrobial capabilities of mast cells.13
Clinical Presentation and Comorbidities
Vascular rosacea presents with characteristic flushing and erythema, which lasts more than a few minutes as compared to physiologic erythema,1 and sometimes telangiectasia is seen.3 The cheeks, chin, and nasolabial folds are most commonly involved.2 In papulopustular rosacea, papules and pustules are seen overlying the erythema.1,3 Open and closed comedones also have been documented in case reports but are not commonly seen.2 Pediatric rosacea often begins with flushing of the face and then progresses to the development of papules and pustules.4
Ocular rosacea can occur with or without cutaneous findings. In a retrospective study of 20 pediatric patients (aged 1–15 years), 11 (55%) patients had both ocular and cutaneous rosacea, 3 (15%) only had ocular symptoms, and 6 (30%) only had cutaneous symptoms. The most common form of rosacea in this study was papulopustular rosacea.14 Ocular symptoms often are bilateral15 and can include blepharitis, conjunctival injection, recurrent chalazion, conjunctivitis,2 and less commonly corneal ulceration and scarring.16 Patients also may report photophobia or a foreign body sensation.17 Importantly, ocular symptoms often precede the cutaneous symptoms and can delay the diagnosis of rosacea,14,16,18 as these symptoms often are misdiagnosed as viral or bacterial infections.15 Fortunately, ocular disease responds well to treatment if diagnosed early.
Weston and Morelli19 conducted a retrospective study of 106 children (46 males; 60 females) 13 years and younger with steroid rosacea; 29 children were younger than 3 years. A family history of rosacea was present in 20% of participants, and prior use of class 7 steroids was reported in 54%, whereas only 3% had used class 1 topical steroids. Ninety-eight participants had perinasal involvement, 94 had perioral involvement, and 44 had periorbital involvement of the lower eyelids.19
Rosacea fulminans (also known as pyoderma faciale) is a rare acute-onset eruption typically found in young women in their 20s and 30s.20 Rosacea fulminans is characterized by papules, pustules, nodules, cysts, draining sinuses, communicating sinus tracts, and less commonly comedones.20,21 The skin can appear erythematous, cyanotic, or dull red.21 Most of the lesions are found on the face, particularly on the forehead, cheeks, nose, and chin,21 but lesions on the chest and back have been documented in adult patients.20 In an examination of prior case series, most patients were otherwise healthy. There are case reports documenting rosacea fulminans in teenagers,20 but the youngest patient recorded was an otherwise healthy 3-year-old girl who developed a sudden onset of erythematous papules, pustules, cysts, and purulent discharging sinuses on the cheeks that spread to the chin, perioral, and paranasal areas.21