Identification of factors associated with treatment refractoriness of oral lesions in pemphigus vulgaris

Br J Dermatol. 2017 Dec;177(6):1583-1589. doi: 10.1111/bjd.15658. Epub 2017 Nov 15.

Abstract

Background: The oral mucosal lesions of patients with pemphigus vulgaris are known to show more treatment refractoriness than skin lesions.

Objectives: To identify which clinical and laboratory parameters may indicate treatment refractoriness of oral lesions in pemphigus vulgaris.

Methods: This was a prospective study of 50 adults with pemphigus vulgaris and oral lesions; patients were given treatment appropriate for overall disease severity. Treatment refractoriness was defined arbitrarily as less than 75% reduction in oral objective Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) after treatment for 6 months.

Results: Of 46 patients who completed the study, 17 (37%) were treatment refractory whereas 29 (63%) were treatment responsive. At baseline, the treatment refractory group had a significantly longer mean duration of disease (P = 0·02) and mean duration of oral lesions (P = 0·01), a higher percentage of lesions in the retromolar trigone (P = 0·05) and on the occlusion line along the buccal mucosa (P = 0·04), a higher percentage of deep/crateriform ulcers (P < 0·001) and erosions with a lichenoid hue (P < 0·001). Herpes simplex virus (HSV) DNA positivity, assessed by polymerase chain reaction in oral tissue scrapings (P = 0·02), was also significantly higher in the treatment refractory group. No other factors we tested for were statistically significant.

Conclusions: Treatment refractoriness of oral lesions was significantly associated with duration of disease/oral lesions; specific morphology and location of oral lesions; and the presence of HSV DNA in the oral cavity. These factors may forewarn the treating physician about a refractory course of oral lesions that may help with counselling patients.

MeSH terms

  • Adult
  • Antibodies / metabolism
  • Azathioprine / therapeutic use
  • Cyclophosphamide / therapeutic use
  • DNA, Viral / metabolism
  • Dermatologic Agents / therapeutic use*
  • Desmoglein 1 / immunology
  • Desmoglein 3 / immunology
  • Drug Resistance
  • Drug Therapy, Combination
  • Female
  • Humans
  • Male
  • Mouth Diseases / drug therapy*
  • Mouth Mucosa / drug effects
  • Pemphigus / drug therapy*
  • Prednisolone / therapeutic use
  • Prospective Studies
  • Regression Analysis
  • Rituximab / therapeutic use
  • Simplexvirus / genetics
  • Treatment Outcome

Substances

  • Antibodies
  • DNA, Viral
  • Dermatologic Agents
  • Desmoglein 1
  • Desmoglein 3
  • Rituximab
  • Cyclophosphamide
  • Prednisolone
  • Azathioprine