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Author: Dr Sophie Rolls, Specialist Registrar in Dermatology, Glamorgan House, University Hospital of Wales, Cardiff and Vale University Health Board, Cardiff, United Kingdom. DermNet NZ Editor in Chief: Adjunct A/Prof Amanda Oakley, Dermatologist, Hamilton, New Zealand. Copy edited by Gus Mitchell. October 2019.
Bullous SLE is also called bullous eruption of SLE and vesiculobullous SLE.
Bullous systemic lupus erythematosus
The incidence of bullous SLE was estimated to be 0.22 and 0.26 cases per million per year in France and Singapore. In a large cohort of sera taken from patients with immunobullous disorders, 1–2% were identified as bullous SLE [1,2].
Like SLE, bullous SLE typically occurs in women of African descent in their thirties. It can, however, occur in all ages, sexes, and ethnicities.
Bullous SLE is classified into type 1 and type 2.
Predisposing factors include adverse drug reactions to hydralazine, penicillamine, methimazole; and exposure to ultraviolet (UV) B radiation.
Bullous SLE is immuno-genetically related to epidermolysis bullous acquista (EBA). In both, the expression of autoimmunity to type VII collagen is associated with human leukocyte antigen class II allele .
In bullous SLE, tense vesicles, bullae and erosions arise on normal or erythematous skin, usually in sun-exposed sites.
No complications have been described.
Most patients with bullous SLE have already been diagnosed with SLE. Investigations are necessary to distinguish bullous SLE from SLE with a concomitant blistering disorder. Rarely, bullous SLE can be the initial clinical manifestation of SLE.
Histopathological findings resemble dermatitis herpetiformis, in that they both possess:
Basal layer vacuolation, characteristic for other forms of cutaneous LE, is not present.
The characteristic direct immunofluorescence (DIF) finding in a perilesional skin biopsy in bullous SLE is linear or granular immunoglobulin (Ig)G, IgM, IgA or C3 at the dermoepidermal junction (DEJ).
Serum autoantibodies, detected via the indirect immunofluorescence test or enzyme-linked immunosorbent assay (ELISA), are against type VII collagen (type I bullous SLE). BP 230, BP 180, or lamina 322 can also be targeted (type II bullous SLE).
Serration pattern analysis of DIF microscopy differentiates tissue bound auto-antibodies against type VII collagen, where a u-serrated pattern is seen, from all other anti-DEJ antibodies where an n-serrated pattern is seen .
Diagnostic criteria for bullous SLE requires:
Other blistering conditions that may resemble bullous SLE include:
Dapsone, at a dose of 1.0–1.5 mg/kg/day, is considered the treatment of choice. It is effective in the vast majority of patients and leads to rapid clinical improvement within days or a few weeks. This rapid response differentiates bullous SLE from SLE with a concomitant autoimmune blistering disease.
Bullous SLE is transient in the majority of cases and usually completely regresses with no further flares, irrespective of the activity of the systemic disease.
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