Review Question - QID 210000

This patient's clinical presentation is consistent with pemphigus vulgaris.

Pemphigus vulgaris typically presents in elderly patients with flaccid bullae, mucosal erosions, and a positive Nikolsky sign (separation of the blister/skin with minimal pressure) on physical exam. The pathophysiology is related to IgG against desmosomes between cells, which result in a reticular pattern on immunofluorescence (rather than linear which would be seen in bullous pemphigoid). Steroids are the treatment of choice for this condition.

Figure A demonstrates the flaccid bullae of pemphigus vulgaris which separate easily with minimal pressure (Nikolsky sign). Illustration A demonstrates immunofluorescence with a netlike intercellular pattern which is unique to pemphigus vulgaris. This is in contrast to the linear pattern in bullous pemphigus.

Incorrect Answers:
Answer 1: Acute drug reaction would either present with a targetoid lesion (which could represent an acute drug reaction or erythema multiforme) or hives depending on the type of reaction. This patient's flaccid bullae suggest a diagnosis of pemphigus vulgaris.

Answer 2: Bullous pemphigoid presents with thick/tough bullae which do not separate/break easily when pressure is applied. This pathology occurs secondary to antibodies to hemidesmosomes resulting in a linear staining pattern on immunofluorescence.

Answer 4: Stevens-Johnson Syndrome presents with fever, malaise, and mucosal ulceration. This is often secondary to infection (Mycoplasma) or sulfa drugs.

Answer 5: Type IV hypersensitivity reflects exposure to poison ivy or poison sumac which causes pruritic vesicular lesions typically in hikers/rangers.

Bullet Summary:
Pemphigus vulgaris presents with flaccid bullae, mucosal erosions, and a positive Nikolsky sign (separation of the blister/skin with minimal pressure).