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TuulaSalo
MariaSiponen

Oral Blistering Diseases

Essentials

  • Oral blistering diseases include vesicle-forming diseases caused by viruses Viral Infections of the Oral Mucosa as well as immunological or hereditary blistering diseases that usually are associated with easily ulcerating larger blisters.

Pemphigoid

  • A group of chronic, autoimmune bullous diseases with antibodies forming against one or more basement membrane antigens. Two main forms: bullous pemphigoid and mucous membrane pemphigoid Chronic Bullous Diseases (Dermatitis Herpetiformis, Pemphigoid).
  • Oral lesions are found in approximately 10-20% of patients with bullous pemphigoid and in 85% of patients with mucous membrane pemphigoid.
  • Most commonly seen in 60-70-year-olds.

Symptoms and findings

  • The blisters in the mouth are filled with clear fluid or blood, and rupture readily. The mucous membrane surrounding the blisters is erythematous. After the blisters rupture the wound surface is slow to heal and may leave a scar.
  • The most common clinical manifestation of mucous membrane pemphigoid in the mouth appears in the attached and marginal gingiva as erythema, desquamation, erosion, blisters or ulceration. Sometimes the most marginal area of the gingiva may be intact. Changes are typically also seen on the palate, the buccal mucous membrane and the tongue.
  • Before the cutaneous bullae appear there may be itching of the skin, excoriations, urticaria, eczema and papular lesions.

Diagnosis

  • If the clinical picture and/or histopathology (subepithelial vesicle) raise suspicion of pemphigoid, the diagnosis is confirmed through examination of a tissue sample (fresh sample) taken from the area surrounding the lesion by direct immunofluorescence. In addition, a fresh tissue sample can be taken also from the buccal mucosa, even if there is no change there.
  • In mucous membrane pemphigoid, serologigal examination will reveal circulating antibodies in 50-80% of patients.
  • Investigations that confirm the diagnosis are usually carried out in specialized care.
  • The Nikolsky's test may be positive (see pemphigus vulgaris).

Treatment

  • Treatment of lesions on oral mucosa in a mild or localized disease consists of topical glucocorticoids, and, as necessary, medicinal spoons are prepared (see treatment of oral lichen planus Oral Lichen Planus).
  • More severe cases and all patients with ocular manifestations should be treated with systemic glucocorticoids and/or with other immunosuppressive medication. The patient should also be advised to avoid any trauma to the skin and mucous membranes which may provoke bullae formation (e.g. food of hard consistency) and to use toothpaste for a sensitive mouth.
  • Maintaining good oral hygiene is important.
  • All patients with mucous membrane pemphigoid should be examined by an ophthalmologist.

Pemphigus vulgaris

  • Rare, chronic autoimmune bullous disease in which antibodies are forming against proteins (desmoglein 1 and 3) in intercellular junctions between epithelial cells.
  • In approximately 60% of cases the disease first manifests itself as blistering in the oral mucosa. The blisters rupture easily causing painful erosions. Intact blisters are rarely encountered in the oral cavity.
  • Most common at middle age, more common in women than in men

Symptoms and findings

  • Typical sites for oral changes are the soft palate, buccal mucosa, the floor of the mouth, the lips and the alveolar ridge.
  • Skin lesions appear as thin walled flaccid blisters filled with clear fluid. They burst quickly and leave redness and erosion in the area.
  • Changes may also occur in the mucous membranes of the larynx, oesophagus, nose, genital or anal area, or of the eye, as well as on the nails.

Diagnosis

  • If the clinical picture and/or histopathology (intraepithelial vesicle) raise suspicion of the disease, the diagnosis is confirmed through examination of a tissue sample (fresh sample) taken from the area surrounding the lesion by direct immunofluorescence. Also serology may confirm the diagnosis. Investigations that confirm the diagnosis are usually carried out in specialized care.
  • Nikolsky's test can be used in clinical diagnostics: pressing uninvolved mucosa around a blister with a blunt instrument for one minute will cause blister formation or the epithelium will separate from the lower layers (as opposed to e.g. viral infections). The use of this test should be considered on a case-by-case basis as it may increase the patient's symptoms.

TreatmentInterventions for Pemphigus

    References

    • Du G, Patzelt S, van Beek N, et al. Mucous membrane pemphigoid. Autoimmun Rev 2022;21(4):103036. [PubMed]
    • Rashid H, Lamberts A, Borradori L, et al. European guidelines (S3) on diagnosis and management of mucous membrane pemphigoid, initiated by the European Academy of Dermatology and Venereology - Part I. J Eur Acad Dermatol Venereol 2021;35(9):1750-1764. [PubMed]
    • Schmidt E, Rashid H, Marzano AV, et al. European Guidelines (S3) on diagnosis and management of mucous membrane pemphigoid, initiated by the European Academy of Dermatology and Venereology - Part II. J Eur Acad Dermatol Venereol 2021;35(10):1926-1948. [PubMed]
    • Carey B, Joshi S, Abdelghani A, et al. The optimal oral biopsy site for diagnosis of mucous membrane pemphigoid and pemphigus vulgaris. Br J Dermatol 2020;182(3):747-753. [PubMed]
    • Joly P, Horvath B, Patsatsi, et al. Updated S2K guidelines on the management of pemphigus vulgaris and foliaceus initiated by the European academy of dermatology and venereology (EADV). J Eur Acad Dermatol Venereol 2020;34(9):1900-1913. [PubMed]
    • Carey B, Setterfield J. Mucous membrane pemphigoid and oral blistering diseases. Clin Exp Dermatol 2019;44(7):732-739. [PubMed]
    • Schmidt E, Kasperkiewicz M, Joly P. Pemphigus. Lancet 2019;394(10201):882-894. [PubMed]
    • McMillan R, Taylor J, Shephard M, et al. World Workshop on Oral Medicine VI: a systematic review of the treatment of mucocutaneous pemphigus vulgaris. Oral Surg Oral Med Oral Pathol Oral Radiol 2015;120(2):132-42.e61. [PubMed]
    • Sultan AS, Villa A, Saavedra AP, et al. Oral mucous membrane pemphigoid and pemphigus vulgaris-a retrospective two-center cohort study. Oral Dis 2017;23(4):498-504. [PubMed]