Efficacious treatment of pemphigus vulgaris by intravenous immunoglobulin during pregnancy and postpartum period

Abstract

Dear Editor, Pemphigus vulgaris (PV) is an autoimmune bullous disease which is characterized by fragile blister formation in cutaneous and mucosal areas. Due to increased susceptibility of PV patients to secondary cutaneous infections, septicemia, electrolyte imbalance, and dehydration, early diagnosis is significant especially in pregnancy (Salzberg, Gero, & Ragsdale, 2014). Herein, we would like to present a 32-yearold woman at 24 weeks gestation diagnosed with PV who was treated with oral corticosteroids and intravenous immunoglobulin (IVIG). While the patient was taking the fourth IVIG infusion after the birth, her breastfed baby developed widespread erythematous rash which was resolved without need for any intervention. A 32-year-old, gravida 4, para 4, abortion 0 woman at 24 weeks of gestation presented with mucocutaneous blister formation accompanied by crusting, erosion, and ulceration. The first bullous lesion appeared in the mouth when she was 1-month pregnant. The patient began to develop vesicle and bullae all over the trunk before she was appointed to our outpatient dermatology clinic. She had no other accompanying skin disorder and was treated with topical/intralesional corticosteroids and oral mouthwash with little help. In addition, she reported to have high fever and difficulty while swallowing leading to malnutrition. Dermatological examination showed diffuse white plaque formation on the tongue/buccal mucosa; hemorrhagic, oozing crust formation on the lips and scalp accompanied by fragile blister formation on an erythematous-eroded base involving the trunk (Figure 1a–c). She was then hospitalized and two skin biopsies were taken from the trunk for hematoxylin and eosin (H&E) and direct immunofluorescence (DIF) examination. Pathological examination revealed suprabasal acantholysis and eosinophil-rich superficial perivascular, interstitial inflammation in dermis. Intraepidermal IgG-positive immunofluorescence was detected by DIF. H&E and DIF results were compatible with PV. We preferred to start IVIG treatment along with moderate doses of oral corticosteroid to control disease activity rapidly. Since she had widespread cutaneous and severe oral lesions leading to malnutrition, combined therapy would be more efficacious. She was started on 30 mg/day oral prednisolone and 120 g IVIG treatment given on 5 consecutive days within a month. Remarkable