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Atypical presentation of bullous pemphigoid in a young adult male: a case report.

Author

Julie Mae T. Lim,
Natividad Sandra D. Chua

Related Institution

Department of Dermatology - St. Luke's Medical Center

Publication Information

Publication Type
Journal
Title
St. Luke
Frequency
Quarterly
Publication Date
January-March 2014
Volume
9
Issue
1
Page(s)
79-84

Abstract

Introduction. Bullous pemphigoid (BP) is one of the most common autoimmune blistering diseases worldwide. It is primarily a disease of the elderly with a peak incidence in the 80s. There is no ethnic, racial, or sexual predilection. BP results from an immune response against adhesion molecules of the epidermis and basement membrane zone. Two BP antigens are identified, namely BP Ag1 and BP Ag2. BP lesions are large tense blisters that may occur anywhere on the body. Diagnosis is made clinically, histopathologically and by immunofluorescence. Corticosteroid is the mainstay of the treatment. The disease generally has a good prognosis except among those associated with comorbidities.


Case summary. A 34-year-old male presented with an 8-month history of multiple discrete and grouped tense vesicles and bullae, some grouped annularly, on an erythematous base on the nape, trunk and extremities. Prior intake of low-dose oral steroids and antihistamines only led to slight improvement of lesions. Progression of lesions prompted consult at this institution, where the initial impression was linear  IgA bullous dermatosis (LABD) versus bullous pemphigoid. Skin punch biopsy with direct immunofluorescence was performed showing results consistent with BP. Patient was started with oral prednisone 60mg daily tapering doses and topical steroids. Follow-up after 1 week showed marked decrease in number of lesions. Appropriate management and advise was given. Unfortunately, patient was eventually lost to follow-up.


Conclusion. BP may occur in young patients with a long history of blister formation, autoimmune diseases should be considered and proper referral to specialists should be done. Prompt diagnosis and early institution of treatment is vital to improve quality of life of patient and to rule out other more serious causes of blistering diseases.

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