Management of pemphigus vulgaris with rituximab: A case repo
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Pemphigus vulgaris is the most common autoimmune vesiculobullous disorder. It presents with flaccid bullae on the skin and erosions on the mucosa. Severe cases can be life-threatening and hence prompt treatment can prevent untoward consequences. Earlier, steroids were the mainstay of treatment but it causes further medical problems. Nevertheless, new studies have emerged about the pathogenesis of the diseases and treatment protocols have been updated .

A 55-year-old female patient reported with a complaint of ulcers in her mouth for the past 3 months. On further questioning, she revealed that she first developed bullae on her chest 5 months back which ruptured to form erosion and the lesion healed spontaneously leaving behind scab. Later she developed multiple ulcers in her mouth which affected her normal oral functions. Her medical history was significant for epilepsy and she was under phenytoin for the past 30 years.

Clinical examination revealed erythematous lesions with crustation of size 1 × 1.5 cm, irregular in shape which bleeds on touch on the lower lip as shown in . Erosions were present on the chest and trunk of size 1 × 1 cm, oval, erythematous base, and surrounded by erythematous halo as shown in . Intraoral examination revealed irregular ulcers in the lower labial mucosa below the vermilion border of size 1.5 × 2 cm, with erythematous base without surrounding erythematous halo, bleeds on touch. Ulcer involving the labial frenum of size 1 × 0.5 cm, covered with slough was present, Desquamation of upper and lower anterior gingiva was evident as shown in . Ulcers of irregular shape were seen on the floor of mouth with hyperkeratinized border, erythematous base as shown in. Mucosa over the lower lip and buccal mucosa peeled on lateral pressure with bleeding and showed a positive Nikolsky's sign.

ELISA test for anti-desmoglein 1 and 3 antibodies was done which revealed high levels of both anti-desmoglein antibodies 1 and 3, with predominant increase in anti-desmoglein antibody 3. Based on the examination and investigations, a final diagnosis of pemphigus was decided.

Management was based on an updated rheumatoid arthritis protocol with rituximab and immunosuppressants. Systemic corticosteroids 20 mg was started and oral prophylaxis was done. Routine oral hygiene instructions were given. Remission induction was done with rituximab, a monoclonal antibody given as 1 gm intravenously 2 infusions 15 days apart and systemic corticosteroids 20 mg once daily tapered by 5 mg every month, methotrexate 7.5 mg once a week along with vitamin B3, B6, B12 combination once daily throughout treatment. She was regularly under follow-up. When the patient was observed 6 months later, there was complete regression of the lesion.

In conclusion, pemphigus vulgaris is an immune-mediated disorder. Management has evolved from high dose corticosteroid therapy with increased adverse effects to targeted therapy against antibodies. Future treatment strategies include targeting specific pathways of autoimmunity

Source: http://www.jiaomr.in/article.asp?issn=0972-1363;year=2019;volume=31;issue=4;spage=386;epage=390;aulast=Francis
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