This autoimmune skin disease is marked by subepidermal blistering respectively tense, large bullae. Normally it occurs after the age of 60 and rarely in children. The lesions of the bullous P. are spread over the whole skin of the body. Oral manifestations appear in only 10-30 percent of the cases. The cicatricial P. is rather rare and involves mostly mucous membranes especially the oral mucosa and the conjunctiva. The skin in in only 30 percent of the cases involved. It can lead to chronic conjunctivitis, scarring and blindness. Whereas the bullous P. is self-limiting within months to years, a spontaneous regression in the cicatricle P. is very rare. Therefore, immediate treatment with immunosuppressive agents is indicated. Surgical intervention could improve the quality of life or may be useful to restore the function but not to heal the disease.
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