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• Pemphigus foliaceus
• Drug-induced pemphigus (penicillamine, captopril, thiol-containing compounds)
• IgG/IgA pemphigus – A rarely reported condition. It is unclear whether it represents a separate entity or pemphigus vulgaris that it transitioning to IgA pemphigus. Clinical findings in a recently reported series resembled pemphigus vulgaris, pemphigus foliaceus, and IgA pemphigus. In IgG/IgA pemphigus, dapsone can be used as an ancillary treatment.
• Paraneoplastic pemphigus – Associated with underlying neoplasms (non-Hodgkin lymphoma, chronic lymphocytic leukemia, Castleman disease, thymomas, sarcomas, Waldenström macroglobulinemia).
• Erythema multiforme – This condition may appear clinically identical to paraneoplastic pemphigus. Patients may have a history of previous episodes that resolved within 2-4 weeks. The presence of classic targetoid lesions of the skin is more consistent with erythema multiforme.
• Bullous pemphigoid – Tense vesicles and bullae are the predominant feature of this condition, and only 20% of patients have oral involvement.
• Stevens-Johnson syndrome – Usually drug induced and accompanied by high fever, skin tenderness, mucosal erosions, and desquamation 1-3 weeks after starting the inciting medication.
• Reactive infectious mucocutaneous eruption (RIME) – Hemorrhagic crusting of lips is accompanied by a sparse macular, papular, or vesicular cutaneous eruption.
• Staphylococcal and streptococcal toxic shock syndrome and toxic shock-like syndrome – Look for sudden onset of exanthematous eruption.
• Epidermolysis bullosa acquisita
• Linear IgA dermatosis
• Acute graft-versus-host disease – Clinical history of bone marrow transplantation.
• Drug-induced hypersensitivity syndrome (drug reaction with eosinophilia and systemic symptoms [DRESS]) – Look for facial edema (hallmark of DRESS), eosinophilia, hepatitis, and other visceral involvement.
• Subcorneal pustular dermatosis (Sneddon-Wilkinson syndrome)
• Hailey-Hailey disease
• Dermatitis herpetiformis
• Drug-induced erythroderma
• Erythrodermic psoriasis
• Atopic dermatitis with erythroderma
• Contact dermatitis
• Sézary syndrome (see cutaneous T-cell lymphoma)
• Phototoxic reaction
• Photoallergic reaction

• Lichen planus – The erosive oral lesions of lichen planus are usually surrounded by characteristic radiating keratotic striae.
• Oral ulcers of systemic lupus erythematosus
• Mucous membrane pemphigoid – This condition mostly affects the mucosa, most commonly the gingiva and palatal mucosa, with only 20% of patients showing cutaneous lesions.
• Primary herpes gingivostomatitis – The oral ulcerations may be diffuse but are composed of smaller lesions (1-2 mm) that coalesce.
• Candidiasis
• Aphthous stomatitis
• Chemotherapy-induced mucositis
• Plasma cell gingivitis – This hypersensitivity reaction presents as marked erythema of the gingiva (desquamative gingivitis) without blister formation.
• Epidermolysis bullosa acquisita – This almost never presents intraorally without skin involvement.
• Linear IgA disease – This almost never presents intraorally without skin involvement.
• Chronic oral erythema multiforme – These ulcers remit completely and then recur and are associated with recurrent herpes simplex virus infection.
• Bullous pemphigoid – This autoimmune bullous dermatosis is less likely to have oral involvement (20% of cases) and can be distinguished by histological and immunofluorescence studies.

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