A 43-year-old lady presents to the clinic with blistering swellings in the mouth, which have ruptured and caused pain. On examination there are ruptured blisters in the oral mucosa, which have ulcerated. The ulcers have erythematous base and are tender to palpation. The scalp has small blisters, which are felt on palpation. She is given steroid lozenges for the painful ulcers in the mouth and is sent home on non steroidal anti inflammatory agents for her pain. She is asked to review if the symptoms worsen or if there is any change. The very next day she presents to the emergency room with extensive bullae all over her trunk. Rubbing a cotton swab laterally on the uninvolved skin near the blisters causes separation of the epidermis.

  1. What is the clinical diagnosis in this lady?
    1. Pemphigus vulgaris
    2. Bullous pemphigoid
    3. Behcet’s Disease
    4. Systemic Lupus Erythematosus

    Answer: The answer is (a). This lady has the classical picture of pemphigus vulgaris. Pemphigus vulgaris is characterized by blistering lesions on the skin, which exhibit Nikolsky’s sign. Nikolsky’s sign is the easy separation of the dermis when a cotton swab is applied laterally to the uninvolved portion of the skin. The skin blisters are usually preceded by oral mucosal blisters which rupture and cause ulceration. The scalp is one of the earliest areas of involvement.

  2. The pathophysiology of this condition includes all the following except:
    1. Autoantibodies against the adhesion molecules in the desmosomal complex of skin and mucus membrane.
    2. Acantholysis is a characteristic feature
    3. About 95% of the patients are positive for HLA DR4/DQw3
    4. Dietary factors have been shown to exacerbate the disease

    Answer: The answer is (d). Pemphigus vulgaris is caused by autoantibodies against the adhesion molecules in the desmosomal complex of the skin and mucous membrane. Acantholysis, which stands for separation of the epidermal cells from each other, is a characteristic feature of pemphigus vulgaris. About 95% of patients are positive for HLA DR4/DQw3 or HLA DRw6/DQw1. Dietary factors are not known to have any influence on the disease.

  3. Which of the following statements regarding the outcome of the disease in this lady is true?
    1. The most important and common cause of morbidity and morality in these patients is hemodynamic instability.
    2. The course of the disease is mostly chronic with some remissions and exacerbations.
    3. Infection with streptococcal species is the most common cause of death.
    4. Disturbances of fluid and electrolyte balance are not common.

    Answer: The answer is (b). Hemodynamic instability is not the common cause of morbidity and mortality in pemphigus vulgaris. In fact the most important cause of mortality and morbidity is secondary infection of the ulcers and ruptured bullae. Staphylococcus is the commonest organism in the community setting and pseudomonas in the hospital environment. Disturbances of fluid and electrolyte balances due to fluid and electrolyte losses are common.

  4. What is the treatment for this lady’s condition?
    1. Topical steroids, systemic steroids and antibiotics
    2. Systemic steroids, azathioprine and dapsone
    3. Topical steroids, Systemic steroids and steroid sparing agents.
    4. Systemic steroids, tetracycline and dapsone

    Answer: The answer is (c). The correct treatment of pemphigus vulgaris includes systemic treatment and topical treatment. Topically steroids may be applied. Systemic treatment includes steroids and steroid sparing agents such as Azathioprine, dapsone etc. Antibiotics are indicated of superadded infection is suspected.

  5. The following diagnostic features of pemphigus vulgaris are true except
    1. Acantholysis is a hallmark of pemphigus vulgaris
    2. Immunoflourescence microscopy demonstrates IgG deposits intercellularly in the epidermis.
    3. Indirect immunofluorescence microscopy to detect circulating antibodies is the gold standard for diagnosis
    4. Some patients demonstrate eosinophilic spongiosis early in the disease.

Answer: The answer is (c). Indirect immunofluorescence microscopy to detect circulating pemphigus antibodies is not necessary for the diagnosis. Antibody titers in some patients may correspond with disease activity and might help in management.