Diagnose It: Hive-Like Blisters & High Blood Pressure

NEWS/ QUIZ

2 MIN QUIZ

A 78-year-old fair-skinned woman presents to her dermatologist with chronic pruritis which, after 2 months, has turned into itchy, hive-like blisters. She has a history of high blood pressure and rheumatoid arthritis and is currently taking a diuretic once daily and an anti-inflammatory twice a day. The patient believed she had eczema and self-treated with topical steroids, which provided no relief. As soon as her blisters clear, new blisters occur accompanied by papules, resembling lesions of lichen planus.

The hive-like blisters appear all over the body, but particularly on her arms, legs, abdomen, groin, armpits, and inner thigh. The dermatologist decides to take a skin biopsy, which shows subepidermal blistering and eosinophil infiltration. Direct immunofluorescence finds IgG along the dermal-epidermal junction.

What is the diagnosis?

Select the correct answer

A) Bullous pemphigoid

B) Psoriasis

C) Vulgaris pemphigoid

D) Bullous lupus erythematous

CORRECT

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INCORRECT

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Answer is: A

Bullous pemphigoid (BP) is an autoimmune bullous disease, also known as an autoimmune blistering disease, that effects the elderly population.^1,2A typical characteristic of PB is papules accompanied by blisters.1 This disease can present with localized bullous lesions or nonbullous and widespread lesions.² Its classic manifestation is blisters combined with urticarial plaques on the trunk and extremities accompanied by intense pruritus.³ It is also one of the most common subepidermal autoimmune diseases.²

BP is associated with autoantigens that play a role in maintaining dermo-epidermal structural integrity.³ A dysregulated T cell immune response is commonly seen along with synthesis of IgG and IgE autoantibodies against hemidesmosomal proteins (BP180 and BP230), which degrades the basement membrane zone.^1,3An additional correlation with polymorphism in the mitochondrial encoded ATP synthesis gene relates to developing BP.² The autoimmune nature of this disease can cause infection complications that can lead to death.³ During the acute phase, there is a 15-fold risk of developing thromboembolic disease, which can also lead to death.

A skin biopsy is used to diagnose the disease, along with a histopathological evaluation, detection of IgG or C3 deposition in the basement membrane, and quantification of circulation antibodies against BP180.^1,2 This condition is also often associated with many comorbidities and has a 1-year mortality rate of 13% to 38%.² Autoimmune disease, such as rheumatoid arthritis, along with varying factors, including medication such as furosemide and penicillin and mechanical and physical trauma, is seen in BP patients.^2,3

Treatment is focused on prevention of infection and symptom relief, as BP is normally self-limiting, and once it clears up, treatments can be discontinued.⁴ High potency topical or systemic steroids can be used for rapid relief but need to be monitored for undesirable side effects and should be slowly tapered off once infection clears.² Antibiotics such as tetracycline can be used to clear infection but only work directly on the immune system and do not prevent bacteria buildup.^1,5 Immunosuppressants can also be useful for patients with autoimmune disease.² However, if the case is severe, the patient should be treated in the hospital because of the high mortality rate and to prevent worsening infection.² Flare-ups can occur, but patients can be symptom free for many years with proper management.⁵

References:

1. Miyamoto D, Santi CG, Aoki V, Maruta CW. Bullous pemphigoid. An Bras Dermatol. 2019;94(2):133-146. doi:10.1590/abd1806-4841.20199007 [Pg 133/ Para 4/ Ln 1-4; Pg 135/ Para 4/ Ln 1-6; Pg 133/ Para 1/ Ln 3-5; Pg 139/ Para 11/ Ln 4-7; Pg 141/ Para 4/ Ln 1-6; Pg 141/ Para 4/ Ln 1-6]

2. Amber KT, Murrell DF, Schmidt E, Joly P, Borradori L. Autoimmune subepidermal bullous diseases of the skin and mucosae: clinical features, diagnosis, and management. Clin Rev Allergy Immunol. 2018;54(1):26-51. doi:10.1007/s12016-017-8633-4 [ Pg 26/ Para 1/ Ln 1-4; Pg 27/ Para 5/ Ln 4-11; Pg 32/ Para 6/ Ln 1-7; Pg 26/ Para 1/ Ln 11-12; Pg 26/ Para 1/ Ln 9-11; Pg 27/ Para 3/ Ln 3-7; Pg 28/ Para 4/ Ln 2-7; Pg 28/ Para 1/ Ln 2-4; Pg 27/ Para 8/ Ln 1-4; Pg 27/ Para 8/ Ln 7-9; Pg 40/ Para 4/ Ln 2-4 + Ln 8-11; Pg 27/ Para 7/ Ln 14-16; Pg 31/ Table 1; Pg 30/ Para 7/ Ln 1-17; Pg 31/ Para 5/ Ln 1-6; Pg 27/ Para 7/ Ln 1-4]

3. Moro F, Fania L, Sinagra JLM, Salemme A, Di Zenzo G. Bullous pemphigoid: trigger and predisposing factors. Biomolecules. 2020;10(10):1432. doi:10.3390/biom10101432 [ Pg 11/ Para 2/ table 2]

4. Han A. A practical approach to treating autoimmune bullous disorders with systemic medications. J Clin Aesthet Dermatol. 2009;2(5):19-28. [Pg 25/ Para 4/ Ln 1-4; Pg 25/ Para 8/ Ln 12-16]

5. American Osteopathic College of Dermatology. Bullous pemphigoid. Accessed April 26, 2022. https://cdn.ymaws.com/www.aocd.org/resource/resmgr/patientresources/1/BULLOUS_
PEMPHIGOID.pdf [Pg 1/ Para 8/ Ln 3-4/ Para 9/ Ln 1-2; Pg 1/ Para 5 Ln 1-3]

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