Pemphigus vulgaris

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Pemphigus vulgaris


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Pemphigus vulgaris

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A 70-year-old man comes to the office because of severe generalized pruritus and blisters. The pruritus began a month ago, followed by multiple tense blisters over his body two weeks later. Some of the blisters ruptured, causing moist erosions. Past medical history is significant for Parkinson disease and hypertension. Current medications include levodopa/carbidopa and lisinopril. Vitals are within normal limits. Physical examination shows multiple 1 to 3 cm tense bullae as shown:  

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Which of the following histological findings will be seen in this patient?  

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Acantholysis p. 487

pemphigus vulgaris and p. 493

Bullae p. 487

pemphigus vulgaris p. 493

IgG antibodies p. 103

pemphigus vulgaris p. 493

Nikolsky sign

pemphigus vulgaris p. 493

Pemphigus vulgaris p. 493

acantholysis and p. 487

autoantibody p. 113

labs/findings p. 718

type II hypersensitivity p. 110


Pemphigus vulgaris is a rare autoimmune skin disorder that causes blisters or bullae because there’s separation of skin cells.

Now, the skin is divided into three layers--the epidermis, dermis, and hypodermis.

The epidermis forms the thin outermost layer of skin.

Underneath, is the thicker dermis layer, and finally, there’s the hypodermis that anchors the skin to the underlying muscle.

The epidermis itself is made of multiple layers of developing keratinocytes - which are flat pancake-shaped cells that are named for the keratin protein that they’re filled with.

Keratinocytes start their life at the lowest layer of the epidermis called the stratum basale, or basal layer which is made of a single layer of stem cells, called basal cells that continually divide and produce new keratinocytes.

Below the epidermis is the basement membrane which is a thin layer of delicate tissue containing collagen, laminins, and other proteins.

Hemidesmosomes are a protein complex that attach basal cells to the basement membrane.

As keratinocytes in the stratum basale mature and lose the ability to divide, they migrate into the next layer, called the stratum spinosum.

The next layer up is the stratum granulosum, then stratum lucidum, and finally stratum corneum.

The cells of the epidermis are bound together by protein complexes called desmosomes, most of which are in stratum spinosum.

Similar to how the skin lines the outside of the body, mucosa lines the inside of the body, and it’s named for the surface it covers.

So there’s oral mucosa, nasal mucosa, bronchial mucosa, gastric mucosa, intestinal mucosa, and so forth.

Mucosa is made up of one or more layers of epithelial cells that sits on top of a layer of connective tissue called lamina propria.

Just like with the skin, the mucosal cells are attached to each other by protein complexes called desmosomes, and the basal cells are attached to the basal membrane by protein complexes called hemidesmosomes.

Pemphigus vulgaris is a type II hypersensitivity reaction, which is when the immune system produces antibodies that bind to the body's own cells.


Pemphigus Vulgaris is an autoimmune skin condition caused by antibodies that attack the epithelial cells in the skin and mucous membranes and form blisters. Symptoms include mucus membrane and skin blisters, and fever. Treatment usually involves corticosteroids and other immunosuppressive medications to suppress the immune system and reduce inflammation.


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  2. "Harrison's Principles of Internal Medicine, Twentieth Edition (Vol.1 & Vol.2)" McGraw-Hill Education / Medical (2018)
  3. "Pathophysiology of Disease: An Introduction to Clinical Medicine 8E" McGraw-Hill Education / Medical (2018)
  4. "CURRENT Medical Diagnosis and Treatment 2020" McGraw-Hill Education / Medical (2019)
  5. "Diagnosis and classification of pemphigus and bullous pemphigoid" Autoimmunity Reviews (2014)
  6. "Pemphigus: Etiology, pathogenesis, and inducing or triggering factors: Facts and controversies" Clinics in Dermatology (2013)
  7. "Pemphigus" Dental Clinics of North America (2013)
  8. "Diagnosis and Clinical Features of Pemphigus Vulgaris" Immunology and Allergy Clinics of North America (2012)

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