Journey of a Patient with Recalcitrant Anti-Laminin 332 Mucous Membrane Pemphigoid

CR30

Dr Sina Gilannejad

Dr Sina Gilannejad and Dr Melanie Simms

This case describes the journey of a patient referred to Cardiff Oral Medicine Department in 2021 for recalcitrant mucous membrane pemphigoid, subsequently diagnosed as laminin-332 subtype.

She presented with multi-site disease, initially affecting the oral cavity and vaginal mucosa. Despite treatment with prednisolone and mycophenolate, the disease continued to progress, giving ocular symblepharon, nasal involvement, and involvement of her larynx giving shortness of breath and difficulty speaking. During this time she also developed ophthalmic shingles, further complicating her management, and resulting in trigeminal post-herpetic neuralgia. Whilst awaiting ENT investigation she was admitted via A&E with difficulty in breathing which progressed to the need for tracheostomy.

The patient was subsequently treated with dapsone and rituximab, the latter of which seemed to give initial improvement, but this was not fully sustained, nor improved by further infusions. The disease has gradually burnt itself out and at recent review is inactive, with almost complete resolution of inflammation, however the patient has been left with long-term disabilities comprising extensive buccal mucosa scarring/ fibrosis and loss of buccal sulci, resulting in trismus, difficulty in eating and in having dental treatment carried out. The tracheostomy is permanent, impacting the ability to speak almost completely, meaning the patient now mainly communicates in writing.

This case will discuss this patient’s journey, including the diagnosis and treatment of laminin-332 pemphigoid, its association with solid tumours, and the scarring nature of this particular subtype of pemphigoid and highlight the consequences of this on patients’ quality of life.

Slide1