Blistering disorders with mucous membrane involvement

Disorder	Associated conditions	Clinical features	Pathology	Laboratory findings
Herpes simplex virus (HSV)	None	Recurrent, grouped vesicles; most commonly found on lips or genitalia	H&E: intraepidermal blister; ballooning of keratinocyte cytoplasm and margination of chromatin to form intranuclear inclusion bodies; mixed inflammatory infiltrate	Viral culture, Tzanck smear, HSV DFA, PCR
Erythema multiforme	HSV or Mycoplasma pneumoniae infection, occasionally drug exposure	Mucosal erythema and erosions, most frequently involving oral cavity; target lesions on skin	H&E: vacuolar interface dermatitis, necrotic keratinocytes, subepidermal clefts and vesiculation	HSV testing on active lesions, serology, or biopsy specimen; testing for M. pneumoniae infection
Pemphigus vulgaris	None	Vesicles, erosions and desquamative gingivitis	H&E: intraepidermal vesicle, acantholysis DIF: intercellular epidermal IgG deposition	IIF: intercellular IgG staining of skin substrate, IgG antidesmoglein 3
Mucous membrane pemphigoid*	Reflects a diverse group of subepidermal blistering disorders with mucous membranes as the primary site of involvement	Vesicles and erosions, desquamative gingivitis; can have ocular involvement and symblepharon	H&E: subepidermal blisters; inflammatory infiltrate and DIF findings vary with subtype	IIF: varies with subtype
Stevens-Johnson syndrome and toxic epidermal necrolysis	Medication, Mycoplasma infection	Exudative erosions of lips, oral mucosa, eyes, genital mucosa; targetoid papulovesicles of skin, skin sloughing, skin pain	H&E: vacuolar interface dermatitis or epidermal necrosis	None
Paraneoplastic pemphigus	Underlying malignancy	Targetoid papulovesicles; erythema multiforme-like cutaneous lesions; exudative erosions of lips, oral mucosal, eyes, genital mucosa	H&E: mixed histology; overlapping features of pemphigus vulgaris, erythema multiforme, and lichen planus DIF: intercellular IgG deposition; basement membrane zone IgG deposition also may be present	IIF: intercellular IgG deposition on transitional epithelium (rodent bladder)

HSV: herpes simplex virus; H&E: hematoxylin and eosin pathology stain; DFA: direct fluorescent antibody; PCR: polymerase chain reaction; DIF: direct immunofluorescence; IgG: immunoglobulin G; IIF: indirect immunofluorescence.
* Skin-predominant forms of bullous pemphigoid, linear IgA bullous dermatosis, and epidermolysis bullosa acquisita may also present with mucosal involvement.

Graphic 67630 Version 2.0

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Blistering disorders with mucous membrane involvement