Angina bullosa haemorrhagica: a disorder to keep in mind

ARTICLE

Auteur(s) : David Serra, Hugo Schönenberger De Oliveira, José Pedro Reis, Ricardo Vieira, Américo Figueiredo

University Hospital of Coimbra, Dermatology Department, Praceta Prof Mota Pinto, 3000-075 Coimbra, Portugal

Angina bullosa haemorrhagica (ABH) is a blistering disorder of the oropharyngeal mucosa that has not received much attention from the dermatological community.

We studied 4 patients (2 male and 2 female; mean age: 59.5 years; range: 44-66 years) with ABH, all presenting a similar history of a suddenly-appearing, dark red, tense blister that grew rapidly and ruptured spontaneously in most instances. Lesions were found in the soft palate mucosa or in the lateral lingual mucosa. The past medical history was significant in one patient, who had been treated with inhaled steroids (budesonide inhaler and mometasone furoate nasal spray) for asthma and nasal polyposis for two years, prior to developing ABH. Furthermore, 2 patients suffered from hypertension, both of them receiving anti-hypertensive treatment. Family history was negative for mucocutaneous disorders.

Recurrences were seen in two patients. One of them had new lesions over a 3-month period and the other has been experiencing a protracted course, with recurrent monthly or twice-monthly episodes for the last 5 years. Laboratory evaluation, including full blood count and coagulation profile, failed to disclose any underlying illness. We performed a more extensive evaluation – complete metabolic panel, ESR and C-reactive protein, serum protein electrophoresis, autoimmune panel and direct immunofluorescence (DIF) studies – in our patient with a chronic relapsing course. Treatment consisted of rupturing large blisters. Furthermore, we advised our patient taking inhaled steroids to rinse her mouth after using her inhaler.

Badham has been recognized as the first to describe ABH, in 1967 [1]. Other designations for the same condition include stomatopompholyx haemorrhagica [2], benign hemorrhagic bullous stomatitis [3] and recurrent or traumatic oral hemophlyctenosis [4]. The incidence remains unknown; however, it is probably more common than previously thought, as significant series have already been published [4]. Its aetiology remains obscure. Blisters could result from a combination of mild trauma associated with a constitutional predisposition, such as loose cohesion between the epithelium and the corium of the mucosa, or a weak anchorage of mucosal vessels, allowing sub-epithelial haemorrhages to occur easily. Lesions usually appear during or right after meals and are probably related to masticatory trauma. Dental and anaesthetic procedures are other recognized precipitants. Arterial hypertension [5], diabetes mellitus [4] and the long-term use of steroid inhalers [6] have also been linked to this condition, as potential etiological factors. The differential diagnosis of mucosal blisters is broad, including pemphigus, mucosal pemphigoid, cicatricial pemphigoid, epidermolysis bullosa acquisita, linear IgA dermatosis, bullous lichen planus, amyloidosis and fixed drug eruption. Haemorrhagic blisters can also appear in the setting of leukemia, vasculitis and other haematological and haemostatic disorders. Nevertheless, ABH can easily be recognized in most cases due to its peculiar presentation and evolution: blood-filled, painless blisters (figure 1A) that usually rupture spontaneously and heal normally, without any treatment. Histology reveals blistering at the subepithelial level without any inflammatory infiltrate (figure 1B) and DIF is negative.

Recurrences are common (around 30% of cases [4]) and large blisters can cause upper airway obstruction. Although the risk of asphyxia is probably remote, palatal or pharyngeal blisters should be ruptured in order to prevent this potential complication. In general, the prognosis is good and this should also be stressed when advising patients.

Acknowledgements

References

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3 Antoni-Bach N, Couilliet D, Garnier J, Tortel MC, et al. Case for diagnosis. Benign hemorrhagic bullous stomatitis. Ann Dermatol Venereol 1999; 126: 525-6.

4 Grinspan D, Abulafia J, Lanfranchi H. Angina bullosa hemorrhagica. Int J Dermatol 1999; 38: 525-8.

5 Horie N, Kawano R, Inaba J, et al. Angina bullosa hemorrhagica of the soft palate: a clinical study of 16 cases. J Oral Sci 2008; 50: 33-6.

6 High AS, Main DMG. Angina bullosa haemorrhagica: a complication of long-term steroid inhaler use. Br Dent J 1988; 165: 176-9.