Childhood-onset bullous systemic lupus erythematosus

Abstract

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.

Impact of chronic urticaria on systemic lupus erythematosus: A nationwide population‐based study in Taiwan

“…Chronic urticaria is caused by degranulation of skin mast cells and a perivascular leukocyte infiltrate composed of lymphocytes, eosinophils, neutrophils and basophils, characterized by the recurrence of wheals, angioedema or both for more than 6 weeks . Patients with CU having a high degree of comorbidity for autoimmune diseases respond to immunosuppression, such as antihistamines, cyclosporin and corticosteroids …”

“…2 Systemic lupus erythematosus (SLE) is a chronic and heterogeneous systemic autoimmune disease characterized by various clinical manifestations involving multiple organ systems, such as skin, joints, kidneys, central and peripheral nervous system, and cardiovascular system. 4,5 Early diagnosis and appropriate treatment involving clinicians across many different specialties are crucial for preventing further manifestations and achieving beneficial long-term outcomes for patients. 6,7 Urticaria may present as the initial symptom in these patients before the more classical form of SLE is confirmed.…”

Childhood-onset systemic lupus erythematosus: early disease manifestations that the paediatrician must know

“…Discoid lupus and lupus erythematosus panniculitis (or lupus erythematosus profundus) were rare forms of chronic cutaneous manifestations reported in 4% and 0.7% at cSLE onset, respectively [10,15]. A diagnosis of bullous cSLE should also be considered in patients with recurrent tense vesiculobullous lesions and usually not associated with classic SLE lesions [16].…”

Bullous systemic lupus erythematosus in a 6‐year‐old boy

“…BSLE can involve any part of the body, including the mucous membranes, but the eruption most commonly involves photodistributed areas, including the face, neck, upper extremities, and upper trunk. BSLE has been rarely reported as the initial presentation of SLE in children and adolescents . We report a case of BSLE as the initial presentation of SLE in a 6‐year‐old boy.…”