Aldehyde dehydrogenase implicated in mucous membrane pemphigoid
Preliminary studies suggest disulfiram could aid in the treatment of mucous membrane pemphigoid (MMP).
Used topically, the drug may inhibit aldehyde deydrogenase (ALDH), which plays a key role in fibrosis, wrote JK Dart, UCL Institute of Ophthalmology, London.
MMP with ocular involvement causes 60% of cicatrising conjunctivitis in the United Kingdom. In one national incidence survey of 50 MMP patients, the median age was 71 years, with a range from 20 to 90 years old.
Twenty-nine of these patients were male and 32 had extraocular MMP as well as ocular MMP. Of these, 8 had another autoimmune disease and 26 were taking immunosuppressive therapy at a 12-month follow up.
In the 1980s, the term benign mucous membrane pemphigoid (BMMP) changed to cicatricial pemphigoid (CP) to reflect the associated scarring.
The terms cicatricial pemphigoid and mucous membrane pemphigoid have since both been used interchangeably.
Studies describing the sites of involvement have attached the names of the sites, most often ocular CP (OCP) and ocular MMP (OMMP).
Cicatricial pemphigoid is now used to describe a rare disease having the same immunopathology as MMP, but which is limited to the head and neck. It is also called localised CP or Brunsting Perry cicatricial pemphigoid.
MMP involves all the orificial mucosal sites: oral, ocular, nasopharyngeal, genital, and anal. Less often it involves the trachea and oesophagus or skin.
The sites become severely inflamed. Scarring rarely occurs in the oral mucosa, but it is a mandatory part of the diagnosis in the conjunctiva. Oral MMP can have a benign course when restricted to the oral mucosa.
However, about 75% of patients with rapidly progressing ocular MMP undergo sight-threatening complications, making early diagnosis and treatment essential.
Ocular MMP presents with a red eye and persistent conjunctivitis that has not responded to topical therapy or with cicatricial entropion, along with trichisasis which may have failed surgical repair.
Some 30% of patients present with acute conjunctivitis and limbitis leading to rapidly progressive scarring and surface failure if uncontrolled.
Persistent epithelial defect, which occurs in about 20% of patients, has a poor prognosis. The remaining patients present with subacute or low grade chronic inflammation and slowly progressive scarring.