Clinical and immunopathological evaluation of epidermolysis bullosa acquisita.
BACKGROUND
Epidermolysis bullosa acquisita (EBA) is a subepidermal blistering disease with IgG antibodies against collagen VII. The disease is heterogeneous and can lead to significant morbidity.


OBJECTIVE
To characterize the clinical and laboratory profile of patients with EBA from Sao Paulo, Brazil.


METHODS
In total, 12 patients (mean age 24 years) were analysed for cutaneous and mucosal involvement, laboratory data and response to treatment.


RESULTS
Mucosal involvement occurred in 11 of the 12 patients (eyes in 4/12, nose in 4/9, pharynx-larynx in 5/9 and oesophagus in 4/10; 3 patients did not undergo nasopharyngeal examination and 2 paediatric patients did not undergo endoscopy). Using direct immunofluorescence, different patterns of deposits were found at the basement membrane zone: IgG (12/12), IgA (6/12), IgM (4/12), C3 (11/12). Indirect immunofluorescence (IIF) was positive in 6 of 12 patients, and IIF on salt-split skin detected dermal deposition in 10 of 12 patients. Antinuclear antibodies were found in 3 of 12 patients, but none of them fulfilled the criteria for systemic lupus erythematosus. After treatment, total remission was achieved in three patients and partial remission in five (three were maintained on minimal treatment, one on the full treatment and one was able to come off treatment). Two patients were lost to follow-up and the remaining two had disease flares. Complications were mainly mucosal (oesophageal stenosis, laryngeal synechia, symblephara and trichiasis).


CONCLUSIONS
Mucosal involvement in EBA is a determining factor for disease morbidity. Complete evaluation of the patient, focusing on both cutaneous and extracutaneous sites is essential, as EBA may evolve to refractory disease, severely compromising its outcome.
Delgado
L
L
Department of Dermatology, Hospital das Clinicas Sao Paulo, Sao Paulo, Brazil.
Aoki
V
V
Santi
C
C
Gabbi
T
T
Sotto
M
M
Maruta
C
C
D000328:Adult; D000368:Aged; D001379:Azathioprine / Q000627:therapeutic use; D001938:Brazil; D002648:Child; D002675:Child, Preschool; D003622:Dapsone / Q000627:therapeutic use; D016107:Epidermolysis Bullosa Acquisita / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007223:Infant; D008297:Male; D008875:Middle Aged; D009173:Mycophenolic Acid / Q000031:analogs & derivatives / Q000627:therapeutic use; D011241:Prednisone / Q000627:therapeutic use; D016896:Treatment Outcome; D055815:Young Adult
D007166:Immunosuppressive Agents; D003622:Dapsone; D009173:Mycophenolic Acid; D001379:Azathioprine; D011241:Prednisone