Сложности дифференциальной диагностики гранулематоза с полиангиитом и рубцующегося пемфигоида

The article presents a clinical case of a rare pathology. The patient for several years visited various specialists. In connection with a similar clinical picture, multi-organ damage, differential diagnosis was performed between systemic vasculitis (granulomatosis with polyangiitis) and scarring pemphigoid.The mucous membrane of the mouth and nose is involved in the pathological process with granulomatosis with polyangiitis in the form of ulcerative defects, which subsequently lead to deformation of the nose. The danger with this systemic vasculitis is renal damage with the development of nephritis, renal failure. With scarring pemphigoid, eye damage is typical. In granulomatosis with polyangiitis, damage to the organ of vision is also sometimes possible, but mainly in the form of an orbit pseudotumor. Despite the fact that treatment for both forms of pathology involves the use of glucocorticoids and cytostatics, with a scarring pemphigoid, the main care is provided by dermatologists and ophthalmologists, while the diagnosis and treatment of systemic vasculitis is the task of rheumatologists. One of the criteria for a scarring pemphigoid is loss of vision. However, in this case, it was possible to establish a diagnosis, obtain the first positive results of therapy before the patient shows signs of disability. So, there is a hope for the possibility of preserving vision and a favorable outcome. The rarity of the disease and its poor knowledge, difficulties in diagnosis and the absence of certain standards of therapy, this diagnosis requires more attention from the specialists.