Case history
Case history #1
A 55-year-old man seeks medical care for superficial erosions and blisters involving the skin of his head, neck, and trunk. Significant involvement of his oral mucosa has made eating difficult. A year ago, he developed tender sores on the buccal mucosa and soft palate of his mouth that were exacerbated by certain foods. His oral disease was initially treated as herpetic stomatitis. He was prescribed multiple courses of antibiotics for a presumed superficial skin infection. His condition deteriorated, resulting in the development of eroded areas over his trunk and extremities, with persistent involvement of his scalp and a 10 kg weight loss. The patient notes that the top layer of his skin could be easily removed when firm horizontal pressure was applied. He also reports involvement of his nasal mucosa.
Case history #2
A 74-year-old woman with a recent history of non-Hodgkin's lymphoma (NHL) developed severe stomatitis with pronounced involvement of the lips. Her NHL was in remission. She was initially thought to have a variant of erythema multiforme, but an underlying cause such as a drug or upper respiratory infection was not found. The eruption persisted >1 month, which is not typical of erythema multiforme. Over the next few months, she developed superficial skin erosions. Some of the lesions are tender, persistent, target-shaped plaques. She also noted increasing shortness of breath and had recently received antibiotics for a presumed case of pneumonia. Her most significant complaint is intractable stomatitis that was unresponsive to prednisolone, oral antibiotics, and anti-herpetic medications.
Other presentations
Less-common presentations include disease confined exclusively to the scalp, in pemphigus foliaceus (PF), or to the oesophagus or conjunctival mucosa, in pemphigus vulgaris (PV). Vegetative lesions (pemphigus vegetans) are less-common variants of PV in which persistent lesions develop into large fungating nodules. In pemphigus erythematosus (PE), typical PF lesions occur in sun-exposed areas in patients with underlying lupus. Unusual variants of paraneoplastic pemphigus (PNP) include seronegative lichenoid PNP.
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