PEMPHIGUS GRANDROUND Prof. Munyao

1 GRANDROUND : 1/14/2015 Unit 7cProf of Clinical Medicine and Vulgaris Outline Summary: Case presentation Background: Definition, Blistering diseases. presentation . Complications. Diagnosis. Differential diagnoses Management. : Case presentation . 44 year old patient , diagnosed in 2009. Good control on follow up for 3 years (2012). Absconded, presented with a flare in term pregnancy after 2 years July 2014.

2 Uneventful pregnancy, controlled on prednisone 10 mg od. Treatment stopped 28/12/14. Admitted in flare on 5 Jan : PEMPHIGUS vulgaris. Common potentially life threatening immunobullous disorder characterised by flaccid vesicles and bullae and erosions due to suprabasal epidermal (Vesiculobullous) diseases. Clinical Syndrome. Disorders of cohesion failure of structural proteins. Hereditary due to genetic mutations.

3 Acquired: Autoimmunity, drug induced or paraneoplastic. PEMPHIGUS Circulating IgG autoantibodies. Bind to desmoglein 3 in suprabasal keratinocytes desmosomes. Non inflammatory stereotactic clefts. Acantholysis presentation . Adults, no gender predisposition, 1- 2/1m population. Primary lesions: spontaneous Skin: Flaccid bullae and vesicles Mucous membranes- erosions Positive Nikolsky and Asboe Hansen sign positive. Secondary lesions Skin: erosions, crusting, scaling collarettes, Lesions are frequently secondarily infected.

4 Oral lesions may preceed skin lesion by months or years. Causes impairment of feeding due to pain. Erosional lesional surface area of >30% body surface area deemed cutaneous failure syndrome Further clinical history: Explore exposure to drugs: captopril, frusemide etc Consider malignancy as per risk and Sepsis. Cutaneous failure. Exfoliative erythroderma. Diagnosis. Clinical features Histology Deep seated Suprabasal clefts with acantholytic keratinocytes.

5 Immunofluorescence Direct- Non lesional tissue reticulate pattern Indirect: IgG4. Immunoprecipitation, immunoblotting Differential diagnoses. PEMPHIGUS foliaceous Pemphigoid group. SSSS yndrome Fixed drug eruption. Management. Holistic diagnosis and management. Cutaneous failure management Skin lesional care Systemic therapy: Prednisone 1mg/kg bwt, <21 days, scale down gradually initially by 1/3rd and less subsequently.

6 Maintainace dose 15- 30 mg/day. Follow pt. Combination: Steroid sparing: Azathioprine, Cyclophosphamide, Methotrexate Rituximab. IVIG, Plasmaphresis. Prognosis. Undiagnosed/unmanaged cutaneous failure>70% Counselling, Control and follow up. Least immunomodulation dose for PEMPHIGUS is a common and serious immunobullous disease. Adverse outcomes are preventable by prompt holisic diagnosis and management. Thank you.