pemphigus Presentation

Pemphigus

Epidemiology

Definition

Aetiopathogenesis

Clinical picture

Diagnosis

Treatment

Definition

an autoimmune intra- epidermal bullous skin disorder which usually affects the middle-aged. It can be life- threatening and difficult to control.

Aetiopathogenesis

Intraepithelial blister formation results from breakdown of intercellular adhesion, thus producing epithelial cell separation known as "acantholysis". Caused by antibody-mediated autoimmune reaction to desmogleins (Dsg), desmosomal transmembrane glycoproteins, leading to acantholysis. It is characterized by IgG autoantibodies against desmoglein 3,1 (Dsg 3,1).

Three major types of pemphigus

Pemphigus vulgaris

Pemphigus vulgaris is by far the most common of the three types.

Pemphigus folaceus

Paraneoplastic pemphigus

Epidemiology

• Most common age group initially diagnosed with pemphigus is middle age.
• No sex predilection.

• Children are very rarely affected.

• People of Jewish orand Indians than in other races, presumably for genetic reasons are most commonly diagnosed

Clinical Presentation

Pemphigus presents with painful vesicles and flaccid bulla on skin and mucous membranes which vary in size from 1-3 cm in diameter. These rupture quickly and more appear as painful ulcerations. The mucous membranes of the mouth are the most common site for pemphigus lesions.

Intra epithelial vesicles or bulla and cleft like spaces are produced by acantolysis .

Diagnosis

Skin biopsy

Electron microscopy

Diagnosis

immunofluorescence fishnet appearance with IgG deposits in the epidermis

ELISA

Treatment

Goals : Reduce inflammatory response -decrease blister formation -promote healing of blisters and erosions Reduce autoantibody production Use minimal dose of medication needed to control the disease

Treatment

Conventional Therapy

Systemic corticosteroids -1 mg/kg prednisone initially used with gradual tapering Immunosuppressive and anti-inflammatory agents -Used in combo with corticosteroids to provide apotential corticosteroid-sparing effect (minimize steroid use)

Other Therapies

• Methotrexate
• Mycophenolate mofetil
• Cyclophosphamide.
• Plasmapheresis
• Rituximab
• Intravenous Immune Globulin
• Plasmapheresis

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