Vegetative pemphigus is one of the clinical forms of pemphigus characterized by the formation of small growths (vegetations) at the bottom of erosion after opening the bubble. The features of the disease include the location of the process in the skin folds and in the area of the body’s natural openings. Vegetative pemphigus is an autoimmune disease in which, due to a violation in the immune system, antibodies are produced to the own cells of the epidermis.
The disease begins with the sudden appearance of bubbles with serous hemorrhagic fluid on the oral mucosa. Simultaneously with rashes in the oral cavity or some time after them, bubbles appear in the folds of the skin (between the buttocks, in the armpits, in the inguinal folds, under the mammary glands, behind the ears), around the navel and at natural openings in the places of transition of the mucous membrane into the skin.
Bubbles with vegetative pemphigus have a flabby shell and open up quite quickly with the formation of erosions. At the bottom of the erosions, the formation of bright red papillomatous growths is characteristic, appearing 4-7 days after the opening of the bladder. Erosion increases in size and can merge, forming a hearth of 10-15 cm in diameter. Their discharge has a fetid putrid smell. Erosions are accompanied by pain and a burning sensation, expressed to such an extent that the patient cannot actively move.
Further development of the process with vegetative pemphigus leads to severe cachexia. With the effectiveness of the treatment, vegetation flattening, drying and healing of erosions with the formation of pigment spots occur.
The diagnosis can be made on the basis of clinical symptoms and the characteristic localization of the process. Nikolsky’s symptom, specific for pemphigus (detachment of the epidermis with slight mechanical action on the skin) with vegetative pemphigus is observed only near the lesion. Only with the generalization of the disease in the terminal stage, it, as with vulgar pemphigus, is detected on healthy areas of the skin.
Microscopic examination of smears-prints taken from the surface of erosions reveals the presence of acantholytic cells. However, they are not strictly specific to pemphigus, since they are also detected in chickenpox, herpes and other diseases.
Histological examination of vegetative pemphigus reveals the presence of cavities and horizontal cracks inside the epidermis. Immunological studies of histological material allow us to confirm the autoimmune nature of the disease. This is a direct immunofluorescence reaction (RIF), which detects IgG accumulations in the spaces between the cells of the epidermis, and an indirect immunofluorescence reaction, which reveals a high titer of autoantibodies to intercellular compounds — desmosomes.
Treatment is carried out with corticosteroids: prednisone, metiprednisolone, dexamethasone, triamcinolone, etc. Therapy of vegetative pemphigus begins with high doses. When the effect of treatment occurs, the dose can be reduced, but complete rejection of glucocorticoids leads to the resumption of the disease. Therefore, therapy is carried out even against the background of complications arising from it.
If vegetative pemphigus has a severe course, then cytostatic drugs are used in its treatment: azathioprine, methotrexate or cyclophosphamide.
The prognosis, as with any form of pemphigus, is unfavorable. Patients die from cachexia during generalization of the process and from secondary infectious complications with the development of sepsis. Prolonged therapy with corticosteroids increases the likelihood of infection and leads to the occurrence of endocrine pathology, gastrointestinal diseases, vascular and neurological disorders.