Abstract
Background The co-existence of SJS/TEN in context of childhood onset lupus is distinctly unusual and only a handful of cases have been reported in the literature. As the presentation of both illnesses can be heterogenous, simultaneous occurrence of both diseases in the same patient can pose diagnostic difficulties for the treating pediatrician.
Methods we report an 11 year old girl who presented with an SJS-TEN rash and had other features consistent with SLE.
Results An 11-year-old girl presented with history of alopecia, intermittent fever, a maculopapular rash progressing into patchy bullous and desquamating lesions, red scaly eyes and 2 episodes of generalized tonic clonic seizures. On examination, she had pallor, edema, oral ulcers and alopecia. She had distinct atypical targetoid lesions, diffuse erythematous rashes with denuded skin all over the face, trunk and extremities (involving >65% body surface area). There were bullae with positive Nikolsy sign. Deep tendon reflexes were brisk. Rest of the systemic examination was unremarkable.
Hemogram showed severe anemia, thrombocytopenia, lymphopenia and high erythrocyte sedimentation rate. Liver and kidney function tests were normal. Urinalysis showed subnephrotic range proteinuria. Antiphospholipid antibody workup (anti-cardiolipin IgG and IgM, 2 Glycoprotein I IgG and IgM, and lupus anticoagulant) was negative. Direct Coombs test was positive. Antinuclear antibody test by immunofluorescence was positive (4+homogeneous with rim enhancement). Anti-DsDNA antibodies tested positive by both enzyme linked immunosorbent assay as well as by immunofluorescence.
Skin biopsy showed full thickness epidermal necrosis. Magnetic resonance imaging (MRI) brain revealed presence of cerebral atrophy with multiple small infarcts suggestive of vasculitic changes. On the basis of clinical and laboratory findings, a diagnosis of SJS/TEN in context of childhood onset lupus was proffered. She was treated with intravenous immunoglobulin, pulse methylprednisolone, antimicrobials and anticonvulsants along with supportive care. The child, however, succumbed to her illness.
Conclusions SJS/TEN may, at times, be the presenting feature of childhood lupus. The etiology of SJS/TEN in this patient is conjectural but is unlikely to be drug induced given the time course of events.
Funding Source(s): NIL
