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Rosacea fulminans in pregnancy with ocular perforation

Rosacea fulminans is a rare disease of unknown etiology characterized by the sudden onset, over weeks to months, of nodules, papules and pustules with intense erythema in the central portion of the face of young adult women [1–4]. It was first described by O'Leary and Kierland in 1940 as ‘pyoderma faciale’ and in 1991, Plewig et al. who considered it an extreme form of rosacea suggested the name rosacea fulminans [1, 3–4]. Constitutional symptoms are rare. Most patients with rosacea fulminans suffer only one episode of severe disease but do have an underlying rosacea with telangiectases and erythema. [1, 3–4]. Rosacea fulminans may occur in pregnancy. Massa and Su found that six of their 29 cases were associated with pregnancy, while Plewig et al. observed that three of their 20 cases occurred during pregnancy. This connection suggests a possible role for hormonal changes in the pathogenesis of rosacea fulminans [3]. In conventional rosacea, ocular involvement is seen in 3–58 % of cases, but it is not a feature of rosacea fulminans [5]. In fact, there is no report of severe ocular rosacea in association with rosacea fulminans. We present a case of rosacea fulminans in pregnancy with severe ocular disease culminating in ocular perforation. A 26-year-old woman presented in week 21 of her second pregnancy; she complained of facial changes for five months. The lesions had appeared abruptly without associated previous symptoms. She also complained of blurred vision. She denied fever, joint symptoms, bowel symptoms or vulvovaginitis. There was no history of medication use. Dermatological examination revealed facial erythema with papules, nodules, pustules, primarily in the center of the face with absence of comedones and telangiectasia (Figure 1a). On ophthalmologic evaluation severe blepharitis was observed with advanced keratitis and ocular perforation in both eyes (Figures 2a, b). Laboratory tests revealed increased C-reactive protein and an elevated sedimentation rate. Other tests were normal. A skin biopsy from the nose showed intense focal neutrophilic inflammation in the dermis along with mild lymphocytic Rosacea fulminans in pregnancy with ocular perforation