Angina Bullosa Hemorrhagica 

Updated: Dec 15, 2017
Author: Kara Melissa T Torres, MD, DPDS; Chief Editor: Dirk M Elston, MD 

Overview

Background

Angina bullosa hemorrhagica (ABH) is the term used to describe acute, benign, and generally subepithelial oral mucosal blisters filled with blood that are not attributable to a systemic disorder or hemostatic defect.[1, 2, 3, 4]

This condition was first described in 1933 as traumatic oral hemophlyctenosis. Badham first used the currently accepted term angina bullosa hemorrhagica in 1967.

The lesions may be confused with other more serious disorders (eg, mucous membrane pemphigoid, epidermolysis bullosa, linear IgA, dermatitis herpetiformis); however, the isolated nature, rapid healing, and rare recurrence of angina bullosa hemorrhagica blisters generally are sufficient findings to rule out the previously mentioned conditions.

The lesions of angina bullosa hemorrhagica may be indistinguishable from blood blisters related to thrombocytopenia; however, blood tests and the absence of areas of ecchymosis, epistaxis, or gingival bleeding are helpful signs to rule it out.

Some authors suggest mild trauma as the causative agent in angina bullosa hemorrhagica to break the epithelial–connective-tissue junction, causing bleeding of superficial capillaries and resulting in the formation of a subepithelial hemorrhagic bullae.

Also see the Medscape articles Bullous Pemphigoid, Epidermolysis Bullosa, Linear IgA Dermatosis, and Dermatitis Herpetiformis.

Pathophysiology

A genetic predisposition of loose adhesion between the epithelium and corium of the mucosa or a weak anchorage of the mucosal vessels may result in subepithelial hemorrhage.[5]

Angina bullosa hemorrhagica (ABH) may also represent an acute or chronic injury to soft tissue. It is an example of an oral mucosal traumatic lesion.[6, 7] Angina bullosa hemorrhagica most commonly occurs in the soft palate where the covering squamous epithelium of the nonkeratinized type is thin and friable.[8] The break in the epithelial-connective–tissue junction causes bleeding of the superficial capillaries, resulting in the formation of the subepithelial hemorrhagic bullae.[8]

Angina bullosa hemorrhagica may also be a rare and underestimated adverse effect of inhalational corticosteroid therapy. The resulting collagen synthesis modifications lead to mucosal atrophy and a decrease in submucosal elastic fibers, especially in the elderly population over the long term (>5 y). Long-term use of such inhalers may induce capillary breakdown.[8, 9] Similarly, vascular fragility has also been implicated, in view of the association of angina bullosa hemorrhagica with diabetes mellitus.[5]

Etiology

The described cases of angina bullosa hemorrhagica (ABH) had spontaneous onset or were related to minor trauma of ingestion of hot drinks[10] ; hard, rough, and crispy foods[11, 8] ; restorative dentistry[12] ; or periodontal therapy.[13]

Other potential causes of angina bullosa hemorrhagica mentioned in the literature are dental injections of anesthetics[11, 14] ; steroid inhalers[15, 14] ; endoscopy[10] ; trauma from the sharp edges of adjacent teeth, metal crowns, and prosthetic use[5, 16] ; tobacco consumption[16] ; anticoagulant intake[17] ; and coughing, sneezing, and shouting.[18, 8]

A report of 16 cases affecting the soft palate described hypertension as the most frequent underlying systemic condition (6 of 16 subjects); however, its relationship remains speculative, as hypertension is common in adults.[8] Other reported associated systemic factors were diabetes mellitus, chronic kidney injury on hemodialysis, asthma, rheumatoid arthritis, gastrointestinal disturbances, hyperuricemia, and systemic lupus erythematosus.[18, 17, 13, 5, 19, 20]

In general, angina bullosa hemorrhagica is not attributable to blood dyscrasia, vesiculobullous disorders, or systemic diseases. A causative factor is not identified in approximately 47% of patients with angina bullosa hemorrhagica.[18]

Epidemiology

Sex

No sex predilection is reported for angina bullosa hemorrhagica (ABH).

Age

Angina bullosa hemorrhagica (ABH) predominantly affects middle-aged or elderly people. The median age at presentation is 54 years, with 60% of the patients in the range of 45-70 years. Lesions have not been documented in children younger than 10 years.

Prognosis

The prognosis for angina bullosa hemorrhagica (ABH) is good. Recurrences have been reported to occur once or twice a year in 30% of the affected population at the same or different site, with no specific precipitating factor (eg, trauma) or seasonal predilection.[21, 19]

ABH is a benign condition; however, some authors have reported acute upper airway obstruction associated with rapidly enlarging bulla of the posterior pharynx and epiglottic region.[18] Rarely, tracheal intubation and surgical tracheostomy are required in such patients.

 

Presentation

History

Angina bullosa hemorrhagica (ABH) is characterized by its sudden onset during or just after eating.[8] The lesions may be preceded by stinging pain or a burning sensation, but they are essentially asymptomatic.[5] A large bullae in the palatal region has been reported to give rise to a feeling of suffocation.[22] Occasionally, patients may present with hoarseness or blood-tinged sialorrhea.[5]

The blisters may last from a few minutes to 24-48 hours and then spontaneously rupture, typically during meals, releasing blood into the mouth. It usually leaves an erosion covered by epithelial slough that heals without scarring, discomfort, or pain within a week’s time.[6] The lesions may appear intermittently or at regular intervals. The cases reported had a duration ranging from 4 months[21] to 25 years.[19]

Patients do not report a tendency to bleed at other sites.

Family history generally is noncontributory to angina bullosa hemorrhagica. Grinspan et al[23] reported that 44% of his patients in a series of 24 cases published in 1999 had from type II diabetes, hyperglycemia, or family history of diabetes. No conclusive evidence of a cause-and-effect relationship exists between the presence of angina bullosa hemorrhagica and glucose metabolism.

Physical Examination

The blister of angina bullosa hemorrhagica (ABH) appears tense, dark red to purple in color, and blood-filled surrounded by an ecchymotic halo. It has an average size of 1-3 cm in diameter.[5] They are often solitary, but multiple lesions have been described.[21]

Note the images below.

A 42-year-old man with the sudden appearance of an A 42-year-old man with the sudden appearance of angina bullosa hemorrhagica. The patient experienced pain a few minutes before the bulla appeared. He had a history of 3 similar previous episodes at the same site. Because the lesions only stay intact for a short duration, the patient took the picture using his own cellular phone.
A 42-year-old man with the sudden appearance of an A 42-year-old man with the sudden appearance of angina bullosa hemorrhagica. The patient experienced pain a few minutes before the bulla appeared. He had a history of 3 similar previous episodes at the same site. Because the lesions only stay intact for a short duration, the patient took the picture using his own cellular phone. Image shows 24 hours after the initial presentation of angina bullosa hemorrhagica.

The soft palate is the most commonly affected site in angina bullosa hemorrhagica.[17] Occasional lesions have been reported in the buccal mucosa, alveolar ridge,[19] tongue, hard palate,[18] and, rarely, the gingiva.[13] If located on the tongue, the anterior third is most commonly affected. The vermillion border of the lips are almost always spared.[23] Angina bullosa hemorrhagica also may involve the pharynx and the esophagus.[18] Approximately one third of the patients exhibit lesions in more than one location.

Similar lesions in other mucous membranes or the skin have not been reported.

Complications

There is a remote risk of asphyxia with palatal or pharyngeal lesions.[5]

 

DDx

 

Workup

Laboratory Studies

In angina bullosa hemorrhagica (ABH), platelet counts and coagulation tests are within normal limits.

Procedures

Performing a biopsy of an intact bulla is difficult because of the short duration of the lesion.

Histologic Findings

The diagnosis of angina bullosa hemorrhagica (ABH) essentially is clinical; however, in cases in which biopsies have been performed, microscopic examination reveals a subepithelial bulla containing RBCs and an underlying mild and nonspecific chronic lymphocytic inflammatory cell infiltrate that generally is limited to the region of the lamina propria. The surface epithelium may be intact or show nonspecific ulceration. Fibrinoid material within the blister and/or beneath the basement membrane may also be present.[13] In one report, angina bullosa hemorrhagica was reported to mimic a neutrophil-rich subepithelial blistering disorder.[21]

Performing a biopsy of an intact bulla is difficult because of the short duration the lesion remains intact. Otherwise, a biopsy of a ruptured bulla exhibits a nonspecific ulceration.

Direct immunostaining for immunoglobulin G, immunoglobulin A, immunoglobulin M, and fibrin is usually negative; however, there may be equivocal staining for immunoglobulin G and C3 along the basement membrane zone.[18, 24]

 

Treatment

Medical Care

No treatment is generally required for angina bullosa hemorrhagica (ABH). Palliative treatment may include benzydamine hydrochloride and chlorhexidine gluconate 0.12%-0.20% mouthwashes to prevent infection and short courses of topical steroids.[6, 18]

Coagulation tests and platelet count may be indicated to rule out a blood dyscrasia.

Any large, intact blood blister should be incised to prevent further enlargement that could cause airway obstruction.

Prevention

Avoidance of mechanical stimuli (eg, metal crown, dental prosthesis) may prevent trauma. The intake of ascorbic acid/citrus flavonoids (200 mg 1 tab bid) may be helpful in decreasing/preventing recurrence.[23] As an adverse event of inhaled corticosteroid therapy, rinsing the mouth with water after inhalation may be preventive.[9] In patients with systemic diseases, antibiotics and/or nonsteroidal anti-inflammatory drugs have been used to prevent secondary infection.[17]