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Bullous pemphigoid,Clinical Manifestation(s) and Treatment

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Bullous pemphigoid refers to an autoimmune, subepidermal blistering disease
that is most commonly seen in the elderly. It is the most common of the autoimmune bullous dermatoses.

Etiology
• Bullous pemphigoid is an autoimmune disease with IgG and/or C3 complement
component reacting with antigens located in the basement membrane zone.
• Drug-induced pemphigoid, although rare, can occur in patients taking penicillamine, furosemide, captopril, penicillin, and sulfasalazine

Clinical Manifestation(s)
• Bullous pemphigoid typically starts as an eczematous or urticarial lesion, with the
lower extremities being the most common location. Blisters form between one
week and several months.
• Anatomic distribution involves the flexor surfaces of the arms, legs, groin, axilla,
and lower abdomen  The head and neck are generally spared. The lesions are irregularly grouped but sometimes can be serpiginous. Oral lesions can
be found occasionally.

Physical Examination
• The typical blistering bullae measure from 5 mm to 2 cm in diameter and contain
clear or bloody fluid . They may arise from normal skin or from an
erythematous base and heal without scarring if denuded.

Diagnostic Tests
• Skin biopsy staining with hematoxylin and eosin reveals subepidermal blisters.
• Direct and indirect immunofluorescence studies can be done to detect the presence of IgG and C3 immune complexes .
• Immunoelectron microscopy also reveals immune deposits on the basement membrane zone.

DIFFERENTIAL DIAGNOSIS
• Cicatricial pemphigoid
• Epidermolysis bullosa acquisita
• Pemphigus
• Pemphigoid nodularis
• Bullous lupus erythematosus
• Herpes gestationis
• Erythema multiforme

TREATMENT

First Line
• Treatment of bullous pemphigoid is based on the degree of involvement and rate of
disease’s progression.
• Topical steroids in general have been used in patients with localized bullous pemphigoid.
• Systemic corticosteroids are considered the standard treatment for more advanced
bullous pemphigoid.

Second Line
• Azathioprine
• Mycophenolate mofetil
• Methotrexate

Third Line
• Cyclophosphamide
• Dapsone

• Plasmapheresis
• IVIG

CLINICAL PEARL(S)
• Antibodies to the basement membrane zone are detected in the serum in 70% of
patients with bullous pemphigoid.

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