Angina Bullosa Hemorrhagica: A Case Report

Case
Report
JOMP
pISSN 2288-9272 eISSN 2383-8493
J Oral Med Pain 2016;41(2):76-79
http://dx.doi.org/10.14476/jomp.2016.41.2.76
Journal of Oral Medicine and Pain
Angina Bullosa Hemorrhagica: A Case Report
Jun-Hyong Park1, Jung-Hoon Yoon2,3, Jin-Kyu Kang1,3
1
Department of Orofacial Pain and Oral Medicine, Wonkwang University Daejeon Dental Hospital, Daejeon, Korea
2
Department of Oral Maxillofacial Pathology, Wonkwang University Daejeon Dental Hospital, Daejeon, Korea
3
Wonkwang Dental Research Institute, Wonkwang University, Iksan, Korea
Received May 20, 2016
Revised May 28, 2016
Accepted May 28, 2016
Correspondence to:
Jin-Kyu Kang
Department of Orofacial Pain and
Oral Medicine, Wonkwang University
Daejeon Dental Hospital, 77 Dunsanro, Seo-gu, Daejeon 35233, Korea
Tel: +82-42-366-1127
Fax: +82-42-366-1115
E-mail: orofacial@wku.ac.kr
Angina bullosa hemorrhagica (ABH) is a rare oral mucosal disorder characterized by blood
blisters which is not related to hematologic or immunopathologic abnormalities. ABH is most
common in middle-aged and elderly people and associated with local trauma, diabetes and
long-term use of inhaled steroids. Diagnosis is generally based on reviewing history and clinical presentation and it is important to distinguish it from other serious disorders. Usually, ABH
is benign condition which requires no treatment. We present a case of 81-year-old female with
ABH.
Key Words: Angina bullosa hemorrhagica; Blood blister; Oral mucosa
This paper was supported by
Wonkwang University in 2015.
INTRODUCTION
pre­­­sentation and it is important to distinguish ABH from
other serious disorders.3)
Angina bullosa hemorrhagica (ABH) is a rare oral muco-
Usually, ABH is benign condition which requires no treat-
sal disorder characterized by blood-filled vesicles or bullae
ment9) except for the lesion associated with upper airway
which is not related to hematologic or immunopathologic
obstruction.10)
abnormalities.1,2) Clinically, ABH has a single or multiple
We present a case of 81-year-old female with ABH.
dark red blisters of 1 to 3 cm in diameter. Soft palate is the
CASE REPORT
most common site, but other oral mucosa including lateral
border of tongue, buccal mucosa and throat may also be affected.3) ABH usually appears suddenly, accompanying vari-
An 81-year-old female presented to the Department of
ous degrees of pain and burst within some minutes or hours
Orofacial Pain and Oral Medicine of Wonkwang University
1,4)
leaving ulcer which heals without scar.
ABH is most common in middle-aged and elderly peo1,5-7)
Daejeon Dental Hospital (Daejeon, Korea) complaining of
a lesion on tongue which had appeared a month ago af-
And it has no sexual predilection. Although the pre-
ter taking a meal. In clinical examination, an ulcerative le-
cise etiology of ABH is unknown, it is thought that ABH is
sion, which measured around 8 mm in diameter, was ob-
associated with local trauma, diabetes and long-term use of
served on the right lateral border of tongue (Fig. 1). She
ple.
8)
inhaled steroids and trauma seems to be the major provok1)
ing factor.
Diagnosis is generally based on history and clinical
was taking medications for diabetes and hyperlipidemia. A
biopsy was taken due to recurrence in the same site and delayed healing. In the result of biopsy, subepithelial cleft and
Copyright Ⓒ 2016 Korean Academy of Orofacial Pain and Oral Medicine. All rights reserved.
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77
Jun-Hyong Park et al. Angina Bullosa Hemorrhagica
Fig. 1. Clinical photograph shows shallow ulcerative lesion on right
lateral border of tongue.
Fig. 3. Microscopic examination reveals a subepithelial cleft filled
with blood and fibrinous materials. Underlying lymphoplasmacytic
inflammatory cell infiltration was observed in lamina propria (H&E
staining, ×200).
Fig. 2. Microscopic examination shows ulceration with chronic
inflammatory cell infiltration in the submucosa (H&E staining, ×
200).
Fig. 4. Two blisters of bright red color and 8 mm each in diameter
on right lateral border of tongue.
infiltration of chronic inflammatory cells were observed (Fig.
biopsy and blood test including herpes simplex virus an-
2, 3).
tibody test were done. Aspiration biopsy showed hemor-
After a month, the patient revisited complaining of black
rhagic fluid with a few inflammatory cells (Fig. 5). Except
discoloration and ulceration in the same site after a pea-
for decreased white blood cell count and increased glucose,
sized blister had broken, which had appeared a week before
blood test results were within normal range. Through these
her visit. The ulcerative lesion was in healing state at clini-
clinical and laboratory results, she was diagnosed as ABH.
cal examination. Lesions occurred twice during a month of
follow-up. When she came the Department of Orofacial Pain
and Oral Medicine, clinical examination showed two intact
DISCUSSION
blisters of bright red color and 8 mm each in diameter at
Clinically, ABH presents a single or multiple dark red blis-
the same site (Fig. 4). She stated that there was no pain on
ters of 1 to 3 cm in diameter. The majority of cases reported
blisters. However, she reported mild pain as the ulcerative
previously were single lesion.3,5,7,9,11-13) Usually, multiple blis-
lesion appeared after the breakdown of blister. Aspiration
ters present two or three small-sized blisters8) but there was
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J Oral Med Pain Vol. 41 No. 2, June 2016
Present report showed the similar lesions recurred three
times with 1 to 3 weeks intervals during a month.
Until now, the etiology of ABH has been unclear; however, mucosal alteration due to local trauma and systemic
condition is a suspected cause. The weakened attachment
between oral epithelium and corium may induce separation
in non-keratinized mucosa, even in subclinical trauma. This
results in bleeding from capillary vessels and the consequent formation of subepithelial blood blisters.9,11,18)
Trauma seems to be a major provoking factor of ABH,
and many cases were reported after various intraoral traumas. Especially, many studies reported ABH cases related to
Fig. 5. Aspiration biopsy shows many extravasated red blood cells
with some inflammatory cells (H&E staining, ×200).
the ingestion of crispy, hard and hot food.1,3,4,8,9) In this case,
the patient mentioned that recurred lesion had appeared after meal.
14)
This report presents
Long-term use of inhaled steroids is referred to another
two blisters of 8 mm in diameter with bright red color and
common cause of ABH as well. It is thought that long-term
it is different from the previous reports in terms of color.
use of inhaled steroids is associated with alterations in col-
a report of numerous blisters in ABH.
4)
Soft palate is the most common site. Stephenson et al.
lagen synthesis, atrophy of mucosa and decrease in sub-
reported that the lesions were found on soft palate in 93%
mucosal elastic fiber.9,12) Senile atrophy occurred in middle-
of patients. Involvement on non-keratinized oral mucosa
aged people is similar as these alterations. This similarity
including buccal mucosa, tongue, lips, mouth floor, and
can be a reason of why ABH is common in middle-aged
4,5,7,9,11-13)
pharynx was also reported by several researchers.
and elderly people3) and relatively rare in young people.
There has been no reported case with ABH in keratinized
Grinspan et al.8) assumed that diabetes, hyperglycemia
mucosa4,11) and other mucosal surfaces or skin.4,6,11,12,15,16) In
and family history of diabetes were associated with ABH
this case, lesions were recurred only on right lateral border
but further study would be needed. In this case, the patient
of the tongue.
had history of diabetes. Hyperglycemia was often observed
1)
during follow up visits. Especially on the visit with exis-
pain was evoked in 36.1% of
tence of intact blisters, 200 mg/dL of blood glucose level
patients. Burning sensation was frequently reported in pa-
was measured. Based on this, uncontrolled hyperglycemia
The degree of pain is different according to patients.
17)
According to da Rosa et al.,
1,8,9,12)
tients who suffered pain.
18)
al.,
In cases reported by Rai et
the researchers found lesions by coincidence while the
might be associated with frequent recurrence although additional research is needed.
patient had not been recognizing. Generally, ABH breaks
Generally, ABH is diagnosed by excluding other diseases
spontaneously within some minutes or hours and heals
with the history and clinical symptoms.6,12,14,15) Disorders
without scarring within 1 to 2 weeks.1) Due to these clinical
which need differential diagnosis from ABH are bullous
features, ABH is considered to be more common than previ-
diseases including pemphigus, pemphigoid, bullous lichen
7,11,13,16,17)
planus, erythema multiforme, linear IgA disease, amyloi-
ously reported.
8)
Grinspan et al. reported recurrence rate of 30% and da
dosis and dermatitis herpetiformis and hematological dis-
Rosa et al. reported recurrence of lesion within 12 months
orders such as thrombocytopenia.5,12) In ABH, the blisters
in 27.6% of patients. Persistent recurrence over 15 years
occur only in intraoral non-keratinized mucosa and the
17)
15)
Recurrence in-
Nikolsky sign is negative. These clinical features are distinct
terval was varied from a few weeks to several months and
from other bullous diseases. Biopsy can be used if neces-
8)
sary. Blood-filled subepithelial bulla and ulcerative lesion
was observed in a report by Edwards et al.
recurred condition was similar but not same as first lesion.
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Jun-Hyong Park et al. Angina Bullosa Hemorrhagica
with chronic inflammation are observed in the histological
8,11)
appearance, respectively before
and after the breaking of
blister.9) Generally, non-specific results appear in the immunofluorescence tests.10)
ABH generates blood-filled blister, however, hematologic abnormalities may exhibit similar lesion as well.
Hematological disorders might exhibit epistaxis, ecchymosis and other signs. Thus clinicians should pay attention to
these signs. In case of ABH, evaluation of complete blood
count and hemostatic function result within normal range.8)
In the patient of our report, the biopsy of ulcerative lesion
was done and subepithelial cleft with chronic inflammation
was observed in the results. Hemorrhagic fluid with a few
inflammatory cells was observed in the aspiration biopsy of
intact blister before breaking. In combination of thorough
review of medical history, lab test and biopsy, we diagnosed
the patient as ABH.
Management of ABH includes reassuring patient, relief
of pain, promotion of healing, and preventive treatment of
secondary infection. Use of chlorohexidine gluconate was
recommended as well.6,9,12) In this case, ABH was managed
by topical steroid mouthwash.
CONFLICT OF INTEREST
No potential conflict of interest relevant to this article
was reported.
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