Abstract
Description Childhood-onset systemic lupus erythematosus is considered a multisystemic, inflammatory autoimmune disease with a wide spectrum of organs involvement. The clinical presentation can vary from cutaneous involvement to nephritis, hematological, neuropsychiatric or macrophage activation syndrome and generally require a more aggressive treatment than for adult population.
We present a series of two cases of pediatric systemic lupus erythematosus.
First case is of a girl 17 years old who presented with malar rash and discoid lesions on photo-exposed aria with histopathological picture relevant for chronic discoid lupus erythematosus. The immunological screening revealed positive antinuclear antibodies, positive anti DNAdc antibodies and decreased complement (C3 and C4). The investigations also showed lymphocytopenia but no other organ involvement (no proteinuria and no echographic cardiac changes). She also complained about different skin rashes, non-specific for lupus, which were resolved only by oral corticosteroids administration. Currently she is on hydroxychloroquine with a favorable course but we close monitoring her by rheum-derma team.
The second case is also a girl 13 years old who is known with a single discoid lesion on the right cheek for about 3 years treated with dermato-corticosteroids and lasers with no improvement. Furthermore, she developed also atrophic aria on the same spot and another two round lesions on the malar area and on the scalp. She also had photosensitivity together with positive antinuclear antibodies and anti DNAdc antibodies. We performed a biopsy relevant for discoid lupus and begun hydroxychloroquine treatment(200mg/day). Despite the treatment the lesions did not show significative improvement. We added mycophenolate mofetil and local calcineurin inhibitor with a good cutaneous and immunological response.
Conclusions The two cases presented prove that childhood-onset systemic lupus erythematosus need an intense multidisciplinary approach because of its aggressive course and disease flares associated with higher morbidity.
