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
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Predilection sites: the inner or anterior thighs, groins, flexor surfaces of the upper extremities and lower abdomen. Oral mucosal lesions are rare. |
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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 |
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Predilection sites: umbilical and periumbilical regions; trunk and extremities. |
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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 |
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Predilection sites: the extremities, trunk and oral mucosa. |
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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 |
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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 |
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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 |
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Predilection sites: symmetrically distributed lesions on extensor surface of the extremities, scalp, nape, shoulders, sacral region and buttocks. |
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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) |
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Predilection sites: trunk, extremitites, the face, abdomen and perineum. Frequent mucous membrane involvement (may induce severe complications). |
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Linear IgA/IgG bullous dermatosis12
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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 |
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Predilection sites: no specific |
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Anti-p450-pemphigoid13
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Only one case in literature, Fujiwara et al.14
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Only one case in literature, Fujiwara et al.14
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Epidermal13
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Unknown |
Anti-p200 pemphigoid32
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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 |
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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 |
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Predilection sites: usually on flexural and/orc intertriginous areas. ± mucous membrane lesions |
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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
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Predilection sites: trunk and flexural surfaces. Frequently oral lesions. |
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Anti-p105-pemphigoid16
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Bullae and erosions on mucous membrane and skin, resembling toxic epidermal necrolysis or pemphigus vulgaris13
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Linear IgG and C3 deposition at the skin basement membrane zone13
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Dermal13
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Unknown |