PubMed1976
The so-called ocular pemphigus is an oculocutaneomucosal disease, the clinical manifestations of which have been recognized for over a century while its recognition as a nosological and histopathological unit is quite recent. The clinical picture in the eye consists essentially of progressive scar contraction which, preceded by a transient bullous phase, leads to symblepharon, entropion and trichiasis. Corneal complications, such as bullous eruption, erosions, proliferation of vasculoconnective tissue, supervene later. The final appearance is that of complete xerosis with total symblepharon producing a typical 'statute eye'. This local process may be accompanied by bullous lesions of the skin and mucous membranes but the general condition of the patient invariably remains good. In the advanced stages of the disease the appearance is described as 'pemphigoid facies'. The nosological place of ocular pemphigus has been a matter of debate. At various times it has been identified with pemphigus vulgaris, dermatitis herpetiformis of Duhring-Brocq, erythema multiforme of Hebra, Stevens-Johnson syndrome, syphilitic dermatitis bullosa and other bullous dermatoses. At present the condition is universally recognized as a clinical entity, referred to as chronic mucosynechial pemphigoid (CMSP). The anatomopathological picture is quite peculiar. The elementary lesion is a sub-epidermic bulla, hence easily distinguished from that of pemphigus vulgaris, which is situated in the Malpighian layer. This difference applies to the lesions of the skin as well as to those of the mucous membranes including the conjuctiva. In the conjunctiva, epithelial changes suggestive of its epidermization were demonstrated. The Malpighian layer shows a high glycogen and DNA content with overall reduction in enzyme activity. The dermis shows infiltration by lymphocytes, histiocytes and plasma cells, and a considerable increase in hyaluronic acid content. There are many areas of dermoepidermal separation with formation of bullae and the basal membrane is often missing. In some cases, in the centre of the bullae, we were able to demonstrate one or more lumps, consisting almost exclusively of hyaluronic acid, probably filtered in from the dermis across the altered basal membrane. Similar changes are met with also in other mucous membranes, that of buccal cavity in particular, even in ostensibly healthy areas. The histological and histochemical findings are sufficiently typical to justify early diagnostic biopsy in all suspected cases. The aetiology of the CMSP still remains debated. Two theories are under consideration at the moment, viral and autoimmune. The main argument in favour of the viral theory is that a cytopathogenic agent can be isolated from the blood of patients with different dermatoses of the pemphigus group, including the CMSP, whose cytotoxic activity can be passed through and potentiated by passage in cell cultures...