| Clinical features |
Systemic lupus erythematosus (fulfil American Rheumatism Association criteria for SLE)
Symmetrical vesiculobullous eruption on trunk, proximal upper extremities, neck and face
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Blistering on lichen planus affected skin of life
Tense bullae on skin surface with predilectioin on flexural areas
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Affects elderly in fifth to seventh decades papules and plaques over the extremities and trunk
Wickham's striae
Mucosal involvement
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Shiny, flat-topped violaceous to erythmatous
Subepidermal blisters affecting lichen planus and non-affected skin
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Violaceous, planar papules and plaques plaques with overlying silvery scales affecting the buttocks, elbows, lower back, scalp and knees
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Symmetrically distributed, well demarcated erythematous
Nail dystrophy
Arthropathy
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| Histology |
Subepidermal blister withpredominant neutrophilic dermal infiltrate DIF – IgG (+/− IgA and IgM at BMZ)
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Intense, and exaggerated basal vacuolar alteration resulting in a subepidermal cleft
Other features of LP DIF – Shaggy fibrinogen along the basement membrane and IgM ovoid bodies. Negative for IgG and C3 along the BMZ.
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Subepidermal blisters with predominant eosinophil infiltration. DIF – linear deposition of IgG and C3 at BMZ IIF - IgG autoantibodies that bind to the epidermal(roof) of salt-split skin substrate
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Epidermal acanthosis with hypergranulosis
Saw-toothed appearance of rete ridges
Band-like infiltrate of lymphocytes in the upper dermis
Colloid bodies and apoptotic keratinocytes DIF – Shaggy fibrinogen along the BMZ and IgM ovoid bodies
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Subepidermal blister
May occur with or without features of lichen planus DIF – IgG and C3 along the BMZ IIF – IgG autoantibodies that bind to the epidermal (roof) side of salt-split skin substrate
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Parakeratosis
Psoriasiform hyperplasia
Neutrophilic and lymphocytic exocytosis
+/− Munro's microabscesses
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| Antibodies |
Type VII collagen, bullous pemphigoid antigen 1, laminin-5 and laminin -611
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Associated with HLA- B7, DR1, DR10 |
IgG autoantibodies to bullous pemphigoid antigens |
Associated with HLA- B7, DR1, DR10 BP230 and BP180 |
Anticollagen XVII (directed against BP180), BP antigens against 200kDa, and 230-kDa |
Elevated levels of TNF-α HLA-Cw6 |
| Treatment |
Corticosteroids
Dapsone
Azathioprine, methotrexate, mycophenolate mofetil
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Corticosteroids (topical and systemic)
NBUVB, Psoralen with UV-A
Acitretin, sulfasalazine
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Corticosteroids (topical and systemic)
Tetracycline, nicontinamide, and erythromycin
Azathioprine, methotrexate, mycophenolate mofetil, cyclophosphamide
Rituximab
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Corticosteroids (topical and systemic)
NBUVB, Psoralen with UV-A
Acitretin, sulfasalazine
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Corticosteroids (topical and systemic)
Tetracycline and nicontinamide
Isotretinoin, dapsone, azathioprine, methotrexate, mycophenolate mofetil
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Topical corticosteroids
Vitamin D analogues, topical retinoids
Phototherapy
Methotrexate, acitretin, ciclosporin, hydroxyurea
Biologics
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