Cause |
Antibodies to collagen VII |
No clear drug association, may have history of recent LE exacerbation |
Drug-induced |
>90% caused by infections, most commonly HSV |
Drugs cause in 80%, infections are rare |
Distribution |
Any cutaneous site with predilection for the face, upper trunk, and proximal extremities, can have mucosal involvement |
Photodistribution, No or limited mucosal involvement |
May occur on any site on skin/mucosa, most commonly palms, soles, lips, and glans penis16
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Typically lesions on extremities and face |
Typically start on the trunk and can spread to the neck/face and proximal upper extremities, generally spare distal extremities |
Lesion morphology |
Tense bullae on an erythematous or urticated base |
Preceded by characteristic cutaneous LE lesions. Develop over weeks to months |
Sharply marginated round- or oval-shaped lesions that can have bullae. Usually few and localized, but can be multiple and disseminated |
Typical target (well-defined border and three distinct zones). Atypical target lesions are palpable and have only two zones and/or a poorly defined border |
Atypical target lesions if present have only two zones or a poorly defined border, but are macular. Develop over hours to days |
Recurrent cases |
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Minutes-hours after re-exposure to medication. Occur in the same location as previous episodes |
More frequently recurrent compared to SJS/TEN, recurrences due to HSV common |
As early as 2 days. No predilection for previously affected sites |
H&E |
Subepidermal blister with neutrophilic infiltrate in upper dermis (papillary microabscesses or band-like infiltrate) |
Junctional vacuolar alteration, presence of solitary necrotic keratinocytes at lower epidermal levels, moderate-to-dense periadnexal and perivascular lymphocytic infiltrate, thickened BMZ, presence of plasma cells, melanophages, mucin |
Superficial and deep perivascular mixed infiltrate with lymphocytes, histiocytes, neutrophils, and eosinophils (eosinophils may predominate) |
Superficial perivascular infiltrate with lymphocytes and histiocytes |
Superficial perivascular infiltrate with lymphocytes and histiocytes. Typically characterized by little or no inflammation, but drug-related cases may have a substantial number of eosinophils |
DIF |
IgG, IgM, IgA, and C3 at the BMZ |
Most characteristic— IgG and/or IgM (IgA less commonly) granular deposits at dermal-epidermal junction and around hair follicles, (neg DIF does not rule out LE) |
Not well described (reports of linear IgG and C3 deposition along the BMZ, perivascular and stippled band of C3 at the BMZ, intercellular IgG and C3 reported.)25
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Not specific (C3 and IgM in blood vessels in the upper dermis, C3 may be found along BMZ and focally in Civatte bodies)26
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Not specific (C3 around blood vessels, IgM, and C3 at the dermoepidermal junction)26
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