Angina bullosa hemorrhagica-like lesions: An underrecognized mucosal manifestation in pemphigus vulgaris

Sir, Pemphigus vulgaris is an autoimmune vesiculobullous disorder affecting the skin and mucous membrane, in which the oral cavity is affected in more than 70% of patients at the time of presentation. It can have varied clinical manifestations in the oral cavity including vesicles and bullae, erosions, desquamative gingivitis, sialorrhea, halitosis, continuous formation of brown, or blackish crusts at the vermillion border and transient or abortive aphthous-like lesions.[12] Angina bullosa hemorrhagica (ABH) are asymptomatic lesions that occur in oral cavity of otherwise healthy individuals and are considered to be “idiopathic” in origin, whereas its mimicker, ABH-like lesions are specific lesions rarely encountered in pemphigus vulgaris. The best way to differentiate both is based on biopsy where ABH shows subepidermal cleft, whereas ABH-like lesions show suprabasal cleft with or without acantholysis, similar to that of pemphigus vulgaris. We report a known case of pemphigus vulgaris presenting with ABH-like lesions in the oral cavity. A 42-year-old male, a known case of pemphigus vulgaris for 5 years with no other comorbidities and on irregular treatment, presented with painful erosions over the scalp and oral cavity for the duration of 10 days. He was on 10 mg prednisolone and 50 mg cyclophosphamide at the time of presentation. General examination, vitals, and systemic examination were normal. Cutaneous examination showed multiple crusted erosions over the parietal and occipital scalp with slough and few erosions over the trunk and upper limb. Both perilesional and distant Nikolsky's signs were negative. No petechiae or ecchymoses were noted anywhere on the body. Examination of the oral cavity showed two hemorrhagic blisters on buccal mucosa opposite to lower first molars over both sides [Figure 1]. Differential diagnosis of ABH and ABH-like lesions in pemphigus was considered. The coagulation profile of the patient including platelet count, bleeding time, and clotting time was normal. Biopsy was done from one of the hemorrhagic blisters and histopathological examination showed suprabasal cleft with a row of tombstone appearance of basal cells with collection of erythrocytes, confirming the diagnosis of ABH-like lesion in pemphigus [Figure 2a and b]. Direct immunofluorescence was not done assuming it to be positive irrespective of the lesion as there were erosions over adjacent buccal mucosa. These hemorrhagic lesions disappeared after 48 h, and the patient is currently under follow-up.Figure 1: Hemorrhagic blister over right buccal mucosaFigure 2: (a) Suprabasal cleft with intraepidermal collection of erythrocytes (H and E, ×4) (b) Acantholysis with row of tombstone appearance of basal cells (H and E, ×40)ABH refers to sudden onset blood-filled bullae over oropharyngeal mucosa in a person with no known bleeding diathesis or immunobullous disorders. Lesions are usually single or less than 3 or 4 in number and can range in size from 2 mm to 3 cm in diameter. They are mostly asymptomatic, although there are few reports of hoarseness of voice and blood-tinged sialorrhea associated with lesions over pharynx.[3] They usually rupture within 48 h and heal without scarring. Few studies have looked into possible associations for this condition. Masticatory trauma, intake of hot food, and the use of long-term steroid inhalers have been proven to have some role. It has also been reported following dental procedures, endoscopy, and even violent sneezing.[4] There are reports of documenting sparse elastic fibers in the mucosa of patients with ABH, resulting in weak anchorage and fragility of vessel walls.[5] It has also been reported to be associated with diabetes and hypertension.[3] However, no such predisposition to develop ABH has been documented in patients on systemic steroids. Both intraepidermal and subepidermal blistering have been reported in histopathology across various reports.[6] Balighi et al. in 2019 reported ABH-like lesions to be a previously unreported but not so uncommon abortive presentation of transient oral pemphigus vulgaris; it was documented in 25.8% of their study population.[7] They found that the majority of patients developed these lesions during partial remission of the disease, and the use of intraoral steroids was another association that was documented. Nevertheless, there was no significant difference in the use of systemic steroids between those with and without ABH-like lesions. Furthermore, since intraoral steroid was used in only 19.5% of those with ABH-like lesions, mucosal friability induced by this alone could not explain the presence of these lesions. Just like in the case of ABH, masticatory trauma may be considered in the pathogenesis of ABH-like lesions in pemphigus, since most of these patients were in partial remission. During the active stage or relapse of pemphigus, patients will usually be on soft and bland or liquid diet because of the painful erosions in oral cavity. Once the disease activity comes down and erosions start healing, they start taking their usual diet and minor trauma caused by the hard food on the healing friable oral mucosa can result in the formation of hemorrhagic bulla. However, it can be assumed that the occurrence of ABH-like lesions in pemphigus patients is probably due to multiple contributory factors since they were present in few patients without other oral mucosal lesions of pemphigus also. Our patient was not a diabetic or hypertensive, had no history of receiving intralesional steroids in the oral cavity, was on systemic steroids for long duration, and presumably did not have high disease activity since his Nikolsky's sign was negative. This case highlights the occurrence of ABH-like lesions as one of the rare and underreported oral manifestations of pemphigus vulgaris which may represent a transient or abortive form of oral pemphigus during the remission period. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

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