Pustaka Ilmiah Universitas Padjadjaran

Abstrak

Combination Of Methylprednisolone Pulse Therapy And Azathioprine In Pemphigus Foliaceus Patient With Cushingoid Habitus Caused By Long Term Corticosteroid Used

Background Pemphigus foliaceus (PF)is an autoimmune blistering disease with cutaneous lesions, characterized by subcorneal cleft, deposit of IgG againts keratinocyte, anddesmoglein-1 as a major autoantigen. Systemic corticosteroid theraphy is the mainstay treatment. Methylprednisolone can be used as pulse theraphy in order to enhance therapeutic effect, decrease the need for long term use of steroid, and reduce side effects, because it is cummulatively less toxic than sustained steroid treatment at lower quantitative dosage. Immunosupressive agents, such as azathioprine can be combined as steroid-sparing therapy. Case A 22 year-old man was admitted toDermatovenereology Department of Hasan Sadikin General Hospital with itchy and painful erythematous macules, blisters, crusts, and scales on almost entire body. The clinical manifestation showed serous crusts and scales on erythematous base, a few flaccid blisters with positive Nikolsky sign spreads to almost entire body sparing both palms and soles, also striae rubra and skin atrophy on trunk and upper legs. Patient revealed slight hypertension, moon face, and muscle wasting. Plasma cortisol level within normal limit. Histopathological examination showed subcorneal cleft contained acantholytic cells. DIF found deposit of IgG. Therefore the patient was diagnosed as PF with cushingoid habitus Discussion Patient was treated with 1000 mg methylprednisolon pulse therapy for three consecutive days, continued with oral 50 mg azathioprine twice daily. Clinical improvement was seen on the 3rd day ofpulse therapy.