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• Summary: Case presentation • Background: Definition, Blistering diseases. • Presentation. • Complications. • Diagnosis. • Differential diagnoses • Management. • Conclusion.
Summary: 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.
• Uneventful pregnancy, controlled on prednisone 10 mg od.
• Treatment stopped 28/12/14. Admitted in flare on 5 Jan 2015.
Definition:
Pemphigus vulgaris.
Common potentially life threatening immunobullous disorder characterised by flaccid vesicles and bullae and erosions due to suprabasal epidermal clefts.
Blistering (Vesiculobullous) diseases.
• Clinical Syndrome.
• Disorders of cohesion failure of structural proteins.
• Hereditary due to genetic mutations.
• Acquired: Autoimmunity, drug induced or paraneoplastic.
Pemphigus vulgaris.
Pathophysiology.
• 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, dyspigmentation.
Pemphigus..
• Lesions are frequently secondarily infected.
• 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 indicators.
Diagnosis.
• Clinical features • Histology Deep seated Suprabasal clefts with acantholytic keratinocytes.
• Immunofluorescence Direct-‐Non lesional tissue reticulate pattern Indirect: IgG4. • Immunoprecipitation, immunoblotting
Differential diagnoses.
• Pemphigus foliaceous • Pemphigoid group. • SSSSyndrome • 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. 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 remission.
Conclusion.
• Pemphigus is a common and serious immunobullous disease.
• Adverse outcomes are preventable by prompt holisic diagnosis and management.