Childhood-onset bullous systemic lupus erythematosus

Bullous systemic lupus erythematosus has rarely been described in pediatric lupus population and the real prevalence of childhood-onset bullous systemic lupus erythematosus has not been reported. From January 1983 to November 2013, 303 childhood-onset SLE (c-SLE) patients were followed at the Pediatric Rheumatology Unit of the Childreńs Institute of Hospital das Clínicas da Faculdade de Medicina Universidade da Universidade de São Paulo, three of them (1%) diagnosed as childhood-onset bullous systemic lupus erythematosus. All three cases presented tense vesiculobullous lesions unassociated with lupus erythematosus lesions, with the median duration of 60 days (30–60). All patients fulfilled bullous systemic lupus erythematosus criteria. Two had nephritis and serositis and presented specific autoantibodies. The histological pattern demonstrated subepidermal blisters with neutrophils-predominant infiltrates within the upper dermis. Direct immunofluorescence (DIF) showed deposits of IgG and complement along the epidermal basement membrane, in the presence or absence of IgA and/or IgM. A positive indirect immunofluorescence on salt-split skin demonstrating dermal binding was observed in two cases. All of them had moderate/severe disease activity at diagnosis with median Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) of 18 (14–24). Two patients received dapsone and one with severe nephritis received immunosuppressive drugs. In conclusion, in the last 30 years the prevalence of bullous lupus in childhood-onset lupus population was low (1%) in our tertiary University Hospital. A diagnosis of SLE should always be considered in children with recurrent tense vesiculobullous lesions with or without systemic manifestations.


Introduction
Bullous systemic lupus erythematosus (BSLE) has rarely been described in pediatric lupus population and to our knowledge the prevalence of childhood BSLE has not been reported.

Objectives
To evaluate the prevalence and describe cases of BSLE in childhood-onset SLE (c-SLE) patients.

Methods
From 1983 to 2013, 303 c-SLE patients were followed at the Pediatric Rheumatology Unit of the Children´s Institute of Hospital das Clínicas da Faculdade de Medicina da Universidade de Sao Paulo. Three of them (1%) presented BSLE and are described herein.

Case 3
A 6 years girl, presented with fever and vesicles and bullous lesions on the neck, lower limbs, and oral mucosa for 2 months. Skin biopsy showed subepidermal blister with neutrophil inflammation in the upper dermis and DIF showed deposition of IgA, IgM and IgG. Two months later, the patient developed eyelid edema and oral ulcers. Hemoglobin was 7.9g/dL, ANA 1:200 and positive anti-Sm and proteinuria was 0.6g/day. Renal biopsy revealed mesangial proliferative lupus nephritis. She received methylprednisolone pulses, prednisone, http://www.ped-rheum.com/content/12/S1/P335 hydroxychloroquine and dapsone. After 4 months, she presented progressive improvement of the bullous lesions.

Conclusion
In the last 30 years, the prevalence of BSLE in childhood was 1% in our tertiary University Hospital. Vesiculobullous skin involvement was the first disease manifestation in all three cases. A diagnosis of SLE should always be considered in children with recurrent tense vesiculobullous lesions especially with systemic manifestations and specific autoantibodies.

None declared
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