Giant Angina Bullosa Hemorrhagica of Hard Palate: A Case Report

Abstract

Angina Bullosa Hemorrhagica is characterized with rapid appearance of soft, blood-filled blisters on the mucosa of oral cavity and oropharynx. It is a benign condition, and is not associated with any systemic hematological disorder. No etiological factors have been identified and there are no established guidelines for its management. The current case report describes the sudden appearance of large, bluish, soft lesion on the soft palate which was managed with incision and drainage, and healed completely over the next three weeks.

Introduction

Angina Bullosa Hemorrhagica (ABH) is benign, usually self-limiting disorder, characterized by sudden appearance of a hemorrhagic bullous lesion in oral or oropharyngeal mucosa. Although it is considered a rare disorder, exact incidence is difficult to be estimated. It is also often misdiagnosed, underdiagnosed or diagnosed with one of the many names suggested for it [1]. Nonetheless, despite benign nature and spontaneous resolution, it causes anxiety to the patient and poses diagnostic dilemma for the health care providers.

We describe an interesting such case who presented with sudden and spontaneous onset of a large hemorrhagic bullous lesion on the hard palate mucosa. The patient was managed with incision and drainage of the lesion to provide relief to the patient. The lesion healed completely over the next 03 weeks without any sequelae.

Case Report

A 35 years old female, with no comorbidities presented to emergency department with history of sudden onset discomfort in the throat. She had a history of having meals two hours back, with no specific history of consuming any hard or hot food. She noticed a reddish blister on the soft palate which gradually increased in size within two hours. She had minimal difficulty on swallowing with no pain or discomfort. There was no history of similar lesions or rashes in any other part of body presently or in the past. She was not on any medications including antiplatelet/anticoagulants, steroids or hormonal therapies. On examination, her blood pressure and other vital signs were normal and remained so during entire duration of admission. Examination of the oral cavity and oropharynx revealed a 04 × 03 cm, soft, non-tender, fluctuating bullous lesion involving soft palate, uvula and extending to the posterior part of hard palate (Fig. 1[a]). Rest of the oral cavity, pharynx, larynx and nasal cavity were normal. A consultation was taken from dermatologist and rheumatologist to identify any systemic illnesses, but was normal. The patient had hematological and biochemical investigations, including coagulation profile, direct & indirect Coombs test and D-dimer levels, all of which were normal.

Fig. 1.

Angina bullosa haemorrhagica: Lesion at presentation (a) and complete resolution of lesion at day 10 (b)

The patient was diagnosed as a case of ABH based on the clinical features and as per the diagnostic criteria proposed by Ordioni U et al. (Table 1) [2]. To alleviate her discomfort due to large bullous lesion in the oropharynx, incision and drainage was performed under local anesthesia. A small incision was given with a number 11 blade and about 10 ml of blood was drained. The incision site was left open to allow any residual hematoma to drain and prevent any recurrence. Following this the patient was discharged and received analgesics (Paracetamol 650 mg tablets) on as and when required basis and diluted betadine mouth wash. She continued intake of normal food without any restrictions. A follow up examination after 10 days revealed a healing lesion with erythema and after 21 days, the palatal lesion healed without any scar (Fig. 1[b]). The patient has not developed any recurrence during a follow up period of two years.

Table 1.

Diagnostic criteria for Angina Bullosa Haemorrhagica as per Ordioni et al. [Six criteria (including I & II) out of nine are sufficient for diagnosis]

		Applicable to current patient
I	Clinically notable haemorrhagic bulla or erosion with a history of bleeding of the oral mucosa	Yes
II	Exclusively oral or oropharyngeal localisation	Yes
III	Palate localization	Yes
IV	Triggering event event or promoting factor (food intake)	Yes
V	Recurrent lesion	No
VI	Favourable evolution without a scar within a few days	Yes
VII	Painless lesion, tingling or burning sensation	Yes
VIII	Normal platelet count and coagulation tests	Yes
IX	Negative direct immunofluorescence	Not done

Discussion

ABH is a rare disease of the oropharyngeal mucosa. A retrospective study has estimated the prevalence to be 0.18%. The disease has been stated to effect mainly the elderly in 6th to 8th decade of life [3]. The disease has been shown to have a slight female preponderance [2]. Numerous case reports exist in literature, and it has been stated to be an underdiagnosed condition due to its misdiagnosis and often spontaneous resolution. The disease has also been described by numerous other synonyms in literature, such as Angina Ulcerosa Benigna, localized oral purpura, Hemorrhagic Bullous Stomatitis, and Stomatopompholyx Hemorrhagica [2, 4].

The exact etiopathogenesis of ABH is yet unknown. Its occurrence is most commonly related to local mechanical or thermal trauma from consumption of hard or hot food [1–3]. The use of topical steroid medications has been postulated to predispose to ABH formation, by causing epithelial atrophy and alteration of elastic fibers of lamina propria [4, 5]. Even though many systemic diseases such as diabetes mellitus, hypertension and chronic renal failure have been implicated as potential casual factors, an association has still not been established [3, 4, 6].

ABH manifests as rapid formation of a blood filled, soft, reddish-blue blister within the mucosa of oral cavity and oropharynx. It usually manifests as a solitary lesion, but multiple lesions may also be present. The lesions are usually reported to range from 0.3 to four cm in size [3, 4, 6]. The lesions most commonly affect the soft palate, followed by lateral border of tongue [1–3]. The term ABH is also a misnomer as ABH lesions are usually painless, but can be associated with discomfort. Large lesions affecting the soft palate and uvula can lead to symptoms of odynophagia and airway compromise. The patient in this case report had reported with an unusually large lesion affecting the soft palate, uvula, and extending to posterior part of hard palate too.

The disease is not associated with any hematological illnesses or coagulopathies [3]. Routine hematological investigations including bleeding and clotting profile are still recommended to rule out any systemic hematological illness. Biopsy of the lesions has been reported to demonstrate an infiltrate of non-specific mononuclear inflammatory cells limited to lamina propria [2, 4]. The findings are non-specific and routine biopsy is therefore not recommended [2].

The diagnosis is usually based on clinical features and lack of systemic illnesses. Ordioni et al. have proposed an acceptable diagnostic criterion based on clinical findings, thus avoiding elaborate investigations like biopsy for this benign, self-limiting condition with most favorable outcome (Table 1) [2]. However, a differential diagnosis of hematological and mucocutaneous manifestations of immunological illnesses should be considered and evaluated appropriately [3].

There are no standard guidelines for management of ABH. Previous published articles have reported management with analgesics and antiseptic mouthwash, aspiration, systemic steroids, and observation for spontaneous rupture and healing [1–3, 6–8]. Surgical management involving incision and drainage has been recommended for large lesions affecting the soft palate with potential for airway obstruction [2]. Interestingly, Kiwi consumption was reported to help in accelerated healing of this lesion [9].

Recurrence has been reported to occur in about 62% of cases [1, 2]. The disease therefore requires due follow-up of the patient for detection and management of any recurrences. The use of ascorbic acid and citroflavinoids to prevent recurrence has been reported, but without significant results [3, 4]. The current case has been followed up for past two years and has not shown any signs of recurrence.

Conclusion

This case report presents an interesting case of an unusually large ABH of soft palate, which progressed rapidly as blood filled bulla and subsided without any sequelae or recurrence during two years follow up. The current case report highlights the need of strong clinical suspicion, optimal workup to exclude other hematological and rheumatological illnesses, and management including surgery to alleviate the patient symptoms. The lesions subside and heal completely in a few weeks without any sequelae.