Oral Ulceration

Involvement of the conjunctivae is relatively common and may lead to blindness. Bullous pemphigoid affects older patients and cutaneous lesions are more common than mucosal lesions. This condition is often associated with the prescribing of various medications. The lesions are similar but may heal with less scarring. Both lesions are histologically similar and show direct immunofluorescence to IgG in the basement membrane, supporting an autoimmune etiology. Biopsy shows subepithelial clefting with dissolution of the basement membrane but no degenerative intraepithelial changes. Treatment is often difficult and multiple sites are involved but potent topical steroids or intralesional steroids can be effective. Systemic steroids may be required for severe cases for ocular involvement. Steroids combined with immunosuppressants maybe needed in refractory cases.

Pemphigus Vulgaris:

Pemphigus vulgaris is a more severe, potentially fatal form characterized by intraepithelial bullae and acantholysis. The disease is more common in Jewish and Italian people and males are more often affected than females. The lesions almost invariably initially involve the oral mucosa and the bullae are so easily ruptured that painful irregular ulcers are often the presenting lesions. The most common sites include the buccal mucosa, palate and gingiva. Microscopically, the lesions show early intercellular edema and loss of intercellular bridges. The lack of cohesion allows cell separation and rounding (acantholysis). Intraepithelial clefting (as opposed to subepithelial clefting in bullous pemphigoid) occurs, and a gentle rubbing of adjacent uninvolved skin may denude the epithelium, producing an ulcer or vesicle (positive Nikolsky’s sign). As this causes a lesion, this test is no longer considered appropriate. The basal cells remain attached to the lamina propria creating a “tombstone” affect and free acantholytic cells assume a spherical form (Tzanck cells) which are considered pathognomonic for pemphigus vulgaris. Direct immunofluorescence shows antibodies against intercellular bridges, more specifically against the desmoglein 3 protein in the desmosomes. Serum levels of intercellular antibodies have been shown to correlate with the severity of the disease. Treatment with high dose steroids has greatly reduced the mortality and morbidity of pemphigus but significant morbidity from steroids has been reported as doses of 100 mg/day are often required for initial control. The dose can be tapered down to 20 mg/day over three months. Intramuscular gold has been used with some success and plasmaphoresis is currently under investigation.

Pemphigoid Variants

There are a number of variants of pemphigoid, with autoantibodies directed against different basement membrane proteins. Oral involvement is also probably more common in bullous pemphigoid than was formerly supposed: bullae and/or other erosions are found in up to 50% of patients.

Linear IgA Disease

Linear IgA disease is a rare disorder, the lesional IgA deposits are linear at the epithelial basement membrane zone.

Linear IgA disease is usually idiopathic i.e. it arises spontaneously. However, it sometimes follows infection and is rarely caused by drug allergy. Vancomycin is the most frequently associated drug, although diclofenac, cotrimoxazole, cyclosporin, lithium, penicillin, phenytoin, and sodium hypochlorite have been implicated in case reports. Drug-induced disease resolves with withdrawal of the offending agent. Linear IgA disease has also been rarely associated with lymphoma, haematological conditions, rheumatological conditions, ulcerative colitis and solid tumours.

As Linear IgA disease is a subepidermal blistering disorder, skin biopsy reports blistering just under the epidermis as opposed to some blistering disorders that result in blistering within the epidermis e.g. pemphigus.

A special skin biopsy antibody test, direct immunofluorescence, reveals the immunoglobulin IgA along the basement membrane of the epidermis in a linear pattern. Sometimes these IgA antibodies can be detected by a blood test (indirect immunofluorescence). Research indicates the antibodies are directed against various basement membrane components (target antigens).

Most patients with Linear IgA disease improve or clear with Dapsone 50: 100mg daily. Although the condition may eventually be cured, many patients require long-term treatment as a reduction in dose of medication results in further blistering.

Localized Oral Purpura (ABH or Angina Bullosa Haemorrhagica)

Blood blisters in the mouth are not uncommon in elderly people.

Aetiology

Unclear. No bleeding tendency appears to underlie this condition. Corticosteroid inhalers may sometimes predispose.

Clinical features

Blood blisters are seen in the mouth or pharynx, mainly on the soft palate (sometimes termed angina bullosa haemorrhagica) and occasionally on the lateral border of the tongue in elderly people. There is rapid onset, with breakdown in a day or two to a large round ulcer.

Diagnosis and management

It is necessary to differentiate this condition from pemphigoid and other vesiculobullous disorders, trauma, and purpura. Confirm that haemostasis is normal first and then perform biopsy to exclude pemphigoid if that is likely.

There is no specific treatment other than reassurance. The blisters should be carefully burst. Topical analgesics may provide symptomatic relief.