ACQUIRED HEMOPHILIA A IN A PATIENT WITH PEMPHIGUS VULGARIS -CASE REPORT

Sara Jasmine Al-shami; Adelina Popa; Raluca Gabriela Miulescu; Mihai Cristian Dumitrașcu; Claudia Mehedințu; Florica Sandru

doi:10.33695/rjcr.v4i2.83

Sara Jasmine Al-shami Department of Dermatology, ”Elias” University Emergency Hospital, Bucharest, Romania
Adelina Popa UMF ”Carol Davila”& Department of Dermatology, Elias University Emergency Hospital, Bucharest
Raluca Gabriela Miulescu ”Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania & Department of Pediatrics, ”Grigore Alexandrescu” Children´s Clinical Emergency Hospital, Bucharest, Romania & Department of Dermatology, Valenii de Munte Country Hospital, Romania
Mihai Cristian Dumitrașcu ”Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania & Department of Obstetrics&Gynecology, University Emergency Hospital, Bucharest, Romania
Claudia Mehedințu ”Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania & Department of Obstetrics&Gynecology, ”Nicolae Malaxa” Clinical Hospital, Bucharest, Romania
Florica Sandru ”Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania &Department of Dermatology, ”Elias” University Emergency Hospital, Bucharest, Romania

DOI: https://doi.org/10.33695/rjcr.v4i2.83

Keywords: Hemophilia A, Acquired hemophilia, pemphigus vulgaris

Abstract

Pemphigus vulgaris is a rare autoimmune disorder involving painful blisters and erosions on the skin and mucous membranes caused by antibodies directed against desmoglein 1 and 3. Acquired hemophilia A (AHA) is a relatively rare coagulation disorder caused by the spontaneous presence of anti-factor VIII immunoglobulin G antibodies (IgG1 and IgG4), known to cause spontaneous clinically significant hemorrhages into the skin and soft tissues in patients with no previous known diagnosis of bleeding disorders. This coagulopathy has been described in conjunction with autoimmune bullous skin diseases, malignancies, and drug reactions. Only a few cases of such interaction have been documented, we herein report a case of a 50-year-old woman with a history of pemphigus vulgaris who has developed extensive ecchymosis on her right arm and right calf. A diagnosis of AHA was made considering the modified coagulation tests. Initiation of treatment with factor VIII led to clinical improvement, however, the patient stopped showing up for further follow-ups.

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