Drug adherence in patients with autoimmune vesiculobullous disorders – a cross sectional study

Abstract

logical lesions and DI. Side effects, particularly neutropenia, were absent and, during 6 months of follow-up, there was no relapse. LCH more commonly affects children, in whom it is often fatal. In adults, LCH has a more benign course, and men are predominantly affected. Vulvar LCH is extremely rare, more common in middle-aged women, and often initially ignored or misdiagnosed as an inflammatory, herpetic, or neoplastic disease. It is very important to consider this entity in the differential diagnosis of vulvar ulcers. In our case, the first manifestation was DI and then vulvar LCH, which is extremely rare. The only case reports of vulvar LCH found in the literature were with multisystemic involvement, namely bone, lungs, and skin. Treatment for vulvar LCH is mainly based on case reports, with varying results obtained from radiotherapy, surgical excision, methotrexate, thalidomide, and topical and systemic glucocorticoids. Our patient was treated with a potent topical glucocorticoid with significant improvement of vulvar LCH before chemotherapy, which was instituted because of the pituitary involvement. Cladribine is a purine nucleoside analogue that is activated via phosphorylation by deoxycytidine kinase and has a cytotoxic effect exclusively on hematopoietic cells. Cladribine has been shown to be effective in achieving complete remission in a variety of patients with both single-organ and multisystemic disease and was very effective in this case. We highlight this clinical case due to its rarity, underlining vulvar ulcerations as a form of cutaneous presentation of LCH, although the initial manifestation in this case was DI. We also emphasize the importance of topical dermatological care as a key therapy for symptomatic relief for such patients.