A suspected Mucous Membrane Pemphigoid MMP case 口腔黏膜类天疱疮

A 70-year-old retired female physician was presented with elevated bullae fluid-filled blisters on the roof of her mouth that had developed over the course of 12 months. Topical antiseptic gel was applied for the blisters and ulcers, which subsided slightly but reoccurring frequently, ruptured blisters leaving painful ulcers. Patient had discontinued wearing the dentures since the onset of the present complaint. Her dentist gave provisional diagnosis Mucous Membrane Pemphigoid MMP and suggested lesion biopsy for confirmation.

Treatment of MMP is similar to that for bullous pemphigoid. Topical or intralesional corticosteroids and a combination of oral doxycycline 100 mg orally twice a day and nicotinamide 500 mg orally 3 times a day may be useful for milder cases [1-4]. In severe MMP cases, high potent immuosuppressants (azathioprine, mycophenolate, cyclophosphamide, rituximab) required [1-4].

The patient is a non-smoker and has no history of systemic disease, her medical history was insignificant. Although clinical findings strongly suggest MMP, she has fear of painful oral lesion biopsy and a side effect of immunosuppressive therapy. If left untreated, MMP is usually a chronic, progressive disease that results in functionally limiting or, in some cases (such as those with airway involvement), life-threatening [5] conditions. She decided to seek alternative herbal medication first.

pemphigoid

On visit, extra-oral examination (skin, eyes) was unremarkable. An intra-oral examination revealed multiple irregular erosions and a blister on the upper mandible mucosal surface, covered with yellowish slough tissue. The herbal medicinal powder twice daily was prescribed, and she was reviewed every 2 weeks with photograph taking. The lesions improved considerably with herbal medicine within 4 weeks of starting the treatment. As there was complete resolution at week 10, herbal were not prescribed. We continued to follow-up the patient for six months with no recurrence of lesions observed.

–

Reference:

  1. Kirtschig G, Murrell D, Wojnarowska F, Khumalo N. Interventions for mucous membrane pemphigoid/cicatricial pemphigoid and epidermolysis bullosa acquisita: a systematic literature review. Arch Dermatol 2002; 138:380.
  2. Lazarova Z, Yancey KB. Cicatricial pemphigoid: immunopathogenesis and treatment. Dermatologic Therapy 2002; 15:382.
  3. Kirtschig G, Murrell D, Wojnarowska F, Khumalo N. Interventions for mucous membrane pemphigoid and epidermolysis bullosa acquisita. Cochrane Database Syst Rev 2003; :CD004056.
  4. Murrell DF, Marinovic B, Caux F, et al. Definitions and outcome measures for mucous membrane pemphigoid: recommendations of an international panel of experts. J Am Acad Dermatol 2015; 72:168.
  5. Fleming TE, Korman NJ. Cicatricial pemphigoid. J Am Acad Dermatol 2000; 43:571.