3. Tasks for self-study during preparation for lesson.

3.1. Theoretical questions for the lesson:

1. Etiology, pathogenesis, classification of true pemphigus (pemphigus vulgaris, pemphigus vegetans, pemphigus foliaceus, pemphigus seborrhoicus).

2. Clinical picture of true pemphigus.

3. Histopathology of true pemphigus.

4. Diagnosis, differential diagnosis of true pemphigus.

5. Treatment (general measures and external treatment).of true pemphigus.

6. Etiology, pathogenesis, clinical picture, diagnosis, differential diagnosis and treatment of Duhring's disease.

7. Etiology, pathogenesis, clinical picture, diagnosis, differential diagnosis and treatment of erythema exudative multiforme.

8. Etiology, pathogenesis, clinical picture, diagnosis, differential diagnosis and treatment of the Stevens-Johnson syndrome.

The subject-matter:

BULLOUS DERMATOSES

True pemphigus (pemphigus vere)

The etiology and pathogenesis of pemphigus, like those of Duhring's disease, are not known despite a great number of theories that have been advanced (immunological, infectious, viral, metabolic, neurogenic, endocrine, enzymatic and toxic). Most researchers to date regard pemphigus as a manifestation of immunopathological auto-aggressive processes. Data on the detection of antibodies against the intercellular substance in the affected skin, fluid from the bullae and blood serum of patients are presented as evidence in confirmation of this conception. Immune fluorescent examination confirms the presence of fluorescence in the region of the intercellular space in the epidermal prickle-cell layer, which is characteristic only of pemphigus patients. Autoimmune mechanisms in pemphigus are evidently of pathogenic rather than of etiological significance.

Some authors recently consider all forms of pemphigus to be variants of the same disease, the causative agent of which is a filterable virus or a group of biologically related viruses. They bring forward numerous proofs of their theory; discovery of virus-like structures in the pemphigus cells by electron microscopy. All these data are not absolute proof of the virus nature of the disease, but they prove the expediency of further research into the role of viruses in this diseases.

The neurogenic conception of the origin of pemphigus is among the oldest. The theory was based on cases in which the dermatosis developed after neuropsychic traumas and strong emotional experiences.

The discovery of marked changes in water, mineral and protein metabolism in pemphigus patients provided grounds for creating the metabolic theory of the origin of pemphigus. It was eventually proved that chloride and sodium metabolism is regulated by the mineralocorticoid function of the adrenal cortex. It is very probable that the endocrine disorders, like the metabolic disorders, are of a secondary character occurring in response to the effect of some etiological factor. If the virus etiology of pemphigus and Duhring's disease is proved, the pathogenic role in their development will still be attributed to the immunological, endocrine, nervous and metabolic disorders. Occasional cases of inherited transmission of pemphigus are on record.

Classification. Four forms of pemphigus vere are distinguished:

1. Vulgaris,

2. Vegetans,

3. Foliaceus,

4. Seborrhoicus seu erythematosus (Senear-Usher syndrome)

Pemphigus vere predominates among females. The dermatosis usually begins between the ages of 40 and 60 (it is rarely encountered in children and boys under 17 years of age) and is characterized by a severe, sometimes malignant course with a fatal outcome.