Background Infantile SLE is an extremely rare disease as underlined by the fact that there are only few publications in the literature (only 13 patients have been published until now).
Objectives Case report of a rare disease.
Methods We report on a female patient who developed infantile systemic lupus erythematosus (iSLE) during the first year of life. At the age of 8 months the child presented with dry cough, strained breathing and agitation at night. 2 months later she developed fever attacks and a skin rash localized on the cheeks in a butterfly shape as well as on the extremities.
Based on the clinical symptoms, serological findings and the skin biopsy we diagnosed infantile systemic lupus erythematosus.
Results Because the skin rash was unresponsive to steroid therapy, we decided to start a systemic treatment with hydroxychloroquine which dramatically improved the skin symptoms and fever attacks. Since the beginning of the therapy the patient has not yet developed a relapse of disease or any other systemic symptoms.
Conclusions In conclusion, we present a rare case of infantile systemic lupus erythematosus. To our best knowledge we are the first to describe a successful treatment with systemic hydroxychloroquine in a patient with iSLE. Since the induced therapy with hydroxychloroquine the patient did not suffer from skin rash or other systemic symptoms any more.
Zulian F, Pluchinotta F, Martini G et al. Severe clinical course of systemic lupus erythematosus in the first year of life. Lupus 2008; 17: 780-6.
Pluchinotta FR, Schiavo B, Vittadello F et al. Distinctive clinical features of pediatric systemic lupus erythematosus in three different age classes. Lupus 2007; 16: 550-5.
Disclosure of Interest None declared
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