Table I.

Clinical spectrum in MMP patientsa

Ag/AbDiseaseClinical ProfilePrognosis
Integrin α6 subunitOPErosions limited to oral cavity onlySelf limiting, no long-term sequelae or hazards
No extra-oral diseaseIncidence of malignancy lower than normal (our unpublished data)
Integrin β4 subunitGeneralized MMPGeneralized erosions in all mucous membranes,Reasonable, if early intervention and if response to therapy is good
including oral cavity, esophagus, larynx, vagina, and the skinScarring can cause blindness, laryngeal, esophageal, vaginal, and anal stenosis
OCPErosions limited to conjunctiva, resulting invariably in scarringOcular involvement can lead to variable degree of scarring; 25% of patients can become blind
Incidence of malignancy lower than normal
Laminin 5/AntiepiligrinAECPSimilar to generalized MMPPoor prognosis, generally poor response to therapy, high incidence of malignancy (solid tumors) often resulting in death
  • a Clinical spectrum, prognosis, and association with malignancy observed in subsets of MMP patients.