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. 2011 Nov 28;3(3):e38. doi: 10.4081/dr.2011.e38

Table 1. Comparison of the clinical and immunopathological features of subepidermal blistering diseases.

Subepidermal immunobullous Disease Clinical Characteristic features DIF Binding on salt split skin Serration pattern
Bullous Pemphigoid (BP) Elderly, most common autoimmune blistering disease. Tense blisters on inflammed or non inflamed skin. Pruritus common, variable severity. More common in patients with multiple sclerosis. IgG and C3 or C3 alone (± weaker staining IgM, IgA) at the dermal-epidermal junction. Epidermal (few dermal) n-serrated9
Predilection sites: the inner or anterior thighs, groins, flexor surfaces of the upper extremities and lower abdomen. Oral mucosal lesions are rare.
Pemphigoid Gestationis During pregnancy and/or puerperium; urticarial plaques and/or tense blisters. Pruritus typically severe. Linear deposition of C3 ± IgG at the dermal-epidermal junction. Epidermal As BP; n-serrated
Predilection sites: umbilical and periumbilical regions; trunk and extremities.
Lichen planus Pemphigoides Usually benign, tense blisters and lesions of lichen planus (usually persistent) on top of lichen planus lesions or on clinically normal skin. Linear deposits of IgG at the dermal-epidermal junction. Epidermal Unknown
Predilection sites: the extremities, trunk and oral mucosa.
Mucous membrane Pemphigoid Elderly (female) patients. Tense blisters and erosions with scar formation. In anti-BP180 MMP and antilamin 332 MMP10: linear IgG, ±C3 occasionally IgA. Epidermal in anti-BP 180 MMP and ocular MMP n-serrated9
Predilection sites: mucosa of the mouth, eyes, nose, larynx, eosohagus or anogenital regions. In ocular MMP:11 linear IgA ±IgG. Dermal in antilamin 332 MMP
Dermatitis herpetiformis Adult patients. Erythematous papules, urticarial plaques, papulovesicles, vesicles and rarely bullae, isolated or in herpetiform grouping often healing with scar formation. Intensely pruritic. Granular papillary and basement membrane IgA. Negative Irrelevant
Predilection sites: symmetrically distributed lesions on extensor surface of the extremities, scalp, nape, shoulders, sacral region and buttocks.
Linear IgA disease Papulovesicular eruption in cluster of jewels configuration. Pruritic(ranging from mild to severe). Linear IgA (rarerly granular) at the dermal-epidermal junction. Epidermal (few dermal) n-serrated9
Predilection sites: trunk, extremitites, the face, abdomen and perineum. Frequent mucous membrane involvement (may induce severe complications).
Linear IgA/IgG bullous dermatosis12 Resemble the lesions of BP, annular vesicobullous lesions with frequent involvement of the oral mucosa. Linear IgA and IgG ± C3 at the dermal-epidermal junction. Epidermal (few dermal or both epidermal and dermal) Probably n-serrated
Predilection sites: no specific
Anti-p450-pemphigoid13 Only one case in literature, Fujiwara et al.14 Only one case in literature, Fujiwara et al.14 Epidermal13 Unknown
Anti-p200 pemphigoid32 Often resembles BP, could resemble dermatitis herpetiformis, linear IgA disease or EBA. Could involve mucous membranes. Often coexisting psoriasis. (rapid response to treatment) Linear IgG and C3 at the dermal-epidermal junction. Dermal n-serrated9
Epidermolysis bullosa acquisita Classical EBA: skin fragility, trauma- induced blisters and erosions. Predilection sites: extensor surfaces of the extremities. ± mucous membrane lesion. Inflammatory EBA: widespread eruptions of tense blisters on erythematous or normal appearing skin. Linear IgG (± IgA, IgM), C3 at the dermal-epidermal junction. Dermal u-serrated9
Predilection sites: usually on flexural and/orc intertriginous areas. ± mucous membrane lesions
Bullous SLE Mainly adult patients. Tense blisters on normal or erythematous skin, eruptions usually in a herpetiform arrangement in patients with SLE. Pruritus may be severe. Linear or granular depositions of IgG (± IgM, IgA, C3) at the dermal-epidermal junction. Dermal (rarely epidermal or combined binding) u-serrated9
Predilection sites: trunk and flexural surfaces. Frequently oral lesions.
Anti-p105-pemphigoid16 Bullae and erosions on mucous membrane and skin, resembling toxic epidermal necrolysis or pemphigus vulgaris13 Linear IgG and C3 deposition at the skin basement membrane zone13 Dermal13 Unknown