Acquired Hemophilia in Association With Pemphigus Vulgaris: An Uncommon Coexistence: A Case Report

Acquired Hemophilia (AHA) is a relatively rare disease that occurs in patients with no previous family history of hemophilia. The spontaneous development of autoantibodies (IgG1 and IgG4) against factor VIII has been reported as the most probable cause of AHA. AHA has been reported in association with other conditions, including some autoimmune bullous skin diseases, such as bullous pemphigoid, pemphigus vulgaris, and pemphigus foliaceous. To the best of our knowledge, only 21 cases of AHA with skin autoimmune bullous diseases have been reported so far. Herein, we report a 63-year-old male with a previous history of pemphigus vulgaris who developed large ecchymotic areas on his lower abdomen and forearms after the second infusion of rituximab. Based on coagulation factors evaluation, he was diagnosed with AHA. Treatment with factor VII led to the improvement in his coagulation status, but unfortunately, he passed away because of inferior wall myocardial infarction four days later.

Childhood Occurrence of Pemphigus

ABSTRACT Pemphigus is a chronic mucocutaneous disease that initially manifests in the form of intraoral blisters which spread to other mucous membrane and skin. This study describes an unusual case of chronic generalized childhood pemphigus disease in an 11-year-old girl, who presented with multiple vesicles all over her body. Such a condition is seen more often in older people rather than children. It is crucial for dental professionals to be familiar with the diagnosis of bullous skin diseases in children and adolescents, especially in its initial stages in order to prevent the serious consequences and morbidity. The article highlights clinical presentation, histopathology, and successful management strategies useful for pediatric dental practice. How to cite this article Patil RU, Anegundi RT, Gujjar KR, Indushekar KR. Childhood Occurrence of Pemphigus. Int J Clin Pediatr Dent 2017;10(2):196-200.