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eMedicine - Angina Bullosa Hemorrhagica : Article by

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AUTHOR AND EDITOR INFORMATION

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Author: Roman Carlos, DDS, Co-Director, Professor, Oral and Maxillofacial Pathology, Centro de Medicina Oral de Guatemala; Past President, International Association of Oral Pathologists; Chairman, Oral and Maxillofacial Pathology, Centro Clínico de Cabeza y Cuello, Guatemala

Roman Carlos is a member of the following medical societies: American Academy of Oral and Maxillofacial Pathology and International Association of Oral Pathologists

Editors: Terry L Barrett, MD, Director, Associate Professor, Department of Dermatology, Division of Dermatopathology and Oral Pathology, Johns Hopkins University School of Medicine; David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Director, Division of Dermatology, Scott and White Clinic; Director Dermatology Residency Training Program, Scott and White Clinic; Drore Eisen, MD, DDS, Consulting Staff, Department of Dermatology, Dermatology Research Associates of Cincinnati; Glen H Crawford, MD, Assistant Clinical Professor, Department of Dermatology, University of Pennsylvania School of Medicine; Chief, Division of Dermatology, The Pennsylvania Hospital; William D James, MD, Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System

Author and Editor Disclosure

Synonyms and related keywords: traumatic oral hemophlyctenosis, angina bullosa hemorrhagica, angina bullosa haemorrhagica, ABH, oral blisters, localized oral purpura

INTRODUCTION

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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.

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 ABH blisters generally are sufficient findings to rule out the previously mentioned conditions.

The lesions maybe 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 to break the epithelial–connective-tissue junction, causing bleeding of superficial capillaries and resulting in the formation of a subepithelial hemorrhagic bullae.

The following related eMedicine articles may be of interest:

Mortality/Morbidity

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.1 Rarely, tracheal intubation and surgical tracheostomy are required.

Sex

No sex predilection is reported.

Age

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.


CLINICAL

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History

  • The blisters predominantly affect middle-aged or elderly people without sex predilection.
  • Some patients describe a stinging pain or burning sensation immediately before the appearance of the blood blister.
  • The blisters last only few minutes and then spontaneously rupture, leaving a shallow ulcer that heals without scarring, discomfort, or pain.
  • Patients do not report a tendency to bleed at other sites.
  • Family history generally is noncontributory.
    • Grinspan et al2 reported that 44% of his patients in a series of 24 cases published in 1999 suffered from type II diabetes, hyperglycemia, or family history of diabetes.
    • No conclusive evidence of a cause-and-effect relationship between the presence of ABH and glucose metabolism exists.

Physical

  • The lesions reach an average size of 1-3 cm in diameter.
  • The soft palate is the most commonly affected site.3
  • Occasional lesions have been reported in the buccal mucosa and tongue.
  • The anterior third is most commonly affected in the tongue.
  • Approximately one third of the patients exhibit lesions in more than one location.
  • The oral mucosa attached to bone (ie, masticatory mucosa represented by the hard palate and gingiva) is not affected.
  • Similar lesions in other mucous membranes or the skin have not been reported.

Causes

  • Slightly more than 50% of the cases described are related to the minor trauma of hot foods,4 restorative dentistry,5 or periodontal therapy.6
  • Other potential causes that have been mentioned in the literature are dental injections of anesthetics,4 steroid inhalers,7 and chlorhexidine gluconate mouthrinse.
  • A causative factor is not identified in approximately 47% of patients.


DIFFERENTIALS

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Dermatitis Herpetiformis
Epidermolysis Bullosa
Linear IgA Dermatosis

Other Problems to be Considered

Mucous membrane pemphigoid
Thrombocytopenia


WORKUP

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Lab Studies

  • 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 is essentially clinical; however, in the cases in which a biopsy has being taken, the microscopic examination reveals a subepithelial bulla filled with blood and an underlying mild and nonspecific mononuclear inflammatory cell infiltrate that generally is limited to the region of the lamina propria. Occasionally, neutrophils may be seen.8

Performing a biopsy of an intact bulla is difficult because of the short duration the lesion stays intact. Otherwise, a biopsy of a ruptured bulla exhibits just a nonspecific ulceration. According to Stephenson et al,9 direct immunostaining for immunoglobulin G, immunoglobulin A, or C3 consistently are noncontributory.


TREATMENT

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Medical Care

No treatment is required because the blood blisters spontaneously rupture and heal. 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.


FOLLOW-UP

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Prognosis

ABH is a benign condition.


MULTIMEDIA

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Media file 1:  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.
Click to see larger pictureClick to see detailView Full Size Image
Media type:  Photo

Media file 2:  The same patient as in Media File 1 twenty-four hours after the initial presentation of angina bullosa hemorrhagica.
Click to see larger pictureClick to see detailView Full Size Image
Media type:  Photo


REFERENCES

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  1. Pahl C, Yarrow S, Steventon N, Saeed NR, Dyar O. Angina bullosa haemorrhagica presenting as acute upper airway obstruction. Br J Anaesth. Feb 2004;92(2):283-6. [Medline].
  2. Grinspan D, Abulafia J, Lanfranchi H. Angina bullosa hemorrhagica. Int J Dermatol. Jul 1999;38(7):525-8. [Medline].
  3. Yamamoto K, Fujimoto M, Inoue M, Maeda M, Yamakawa N, Kirita T. Angina bullosa hemorrhagica of the soft palate: report of 11 cases and literature review. J Oral Maxillofac Surg. Sep 2006;64(9):1433-6. [Medline].
  4. Garlick JA, Calderon S. Oral blood blisters in angina bullosa haemorrhagica secondary to trauma of eating and dental injection. Br Dent J. Oct 22 1988;165(8):286-7. [Medline].
  5. Corson MA, Sloan P. Angina bullosa haemorrhagica: an unusual complication following crown preparation. Br Dent J. Jan 6 1996;180(1):24-5. [Medline].
  6. Curran AE, Rives RW. Angina bullosa hemorrhagica: an unusual problem following periodontal therapy. J Periodontol. Nov 2000;71(11):1770-3. [Medline].
  7. Higgins EM, du Vivier AW. Angina bullosa haemorrhagica--a possible relation to steroid inhalers. Clin Exp Dermatol. Jul 1991;16(4):244-6. [Medline].
  8. Kurban M, Kibbi AG, Ghosn S. Expanding the histologic spectrum of angina bullosa hemorrhagica: report of one case. Am J Dermatopathol. Oct 2007;29(5):477-9. [Medline].
  9. Stephenson P, Scully C, Prime SS, Daly HM. Angina bullosa haemorrhagica: lesional immunostaining and haematological findings. Br J Oral Maxillofac Surg. Dec 1987;25(6):488-91. [Medline].
  10. de las Heras ME, Moreno R, Núñez M, Gómez MI, Ledo A. Angina bullosa hemorrhagica. J Dermatol. Jul 1996;23(7):507-9. [Medline].
  11. Deblauwe BM, van der Waal I. Blood blisters of the oral mucosa (angina bullosa haemorrhagica). J Am Acad Dermatol. Aug 1994;31(2 Pt 2):341-4. [Medline].
  12. Edwards S, Wilkinson JD, Wojnarowska F. Angina bullosa haemorrhagica--a report of three cases and review of the literature. Clin Exp Dermatol. Nov 1990;15(6):422-4. [Medline].
  13. Giuliani M, Favia GF, Lajolo C, Miani CM. Angina bullosa haemorrhagica: presentation of eight new cases and a review of the literature. Oral Dis. Jan 2002;8(1):54-8. [Medline].
  14. Hopkins R, Walker DM. Oral blood blisters: angina bullosa haemorrhagica. Br J Oral Maxillofac Surg. Feb 1985;23(1):9-16. [Medline].
  15. Stephenson P, Lamey PJ, Scully C, Prime SS. Angina bullosa haemorrhagica: clinical and laboratory features in 30 patients. Oral Surg Oral Med Oral Pathol. May 1987;63(5):560-5. [Medline].

Angina Bullosa Hemorrhagica excerpt

Article Last Updated: May 22, 2008