Pemphigus erythematosus (seborrheic pemphigus, Senir-Asher syndrome) is a clinical form of pemphigus that combines the manifestations of true pemphigus, seborrheic dermatitis and erythematous variant of systemic lupus erythematosus. Typical symptoms are red, densely crusted areas of facial skin lesions in combination with seborrheic foci on the scalp. It is characterized by a long course with remissions. The diagnosis is based on the study of smear prints and biopsy material. Treatment is carried out with glucocorticosteroids, methods of extracorporeal hemocorrection, local administration of triamcinolone and the imposition of glucocorticoid ointments.
L10.4 Pemphigus Erythematosus
According to the majority of authors engaged in research in the field of clinical dermatology, pemphigus erythematosus is a clinical variant of true pemphigus, which is confirmed by cases of its transformation into leaf-shaped or vulgar pemphigus. However, other researchers consider the disease to be a kind of leaf-shaped pemphigus with a mild course. The localization of pemphigus erythematosus and the similarity of its manifestations on the scalp with the picture of seborrheic dermatitis served as the basis for the second name of the disease — seborrheic pemphigus.
Pemphigus erythematosus begins with the appearance of clearly delimited areas of redness of the skin — erythematous foci. They are localized mainly in the cheeks and the back of the nose. Erythematous spots are covered with loose and thin grayish-yellow crusts, which are formed due to the drying of the liquid coming out of the opened bubbles. The bubbles themselves that appear on the surface of the spots, thin and flabby, are opened so quickly that they often remain invisible to both the patient and the doctor.
Manifestations of pemphigus erythematosus localized on the face are combined with lesions of the scalp in the form of red foci covered with oily scales, resembling a picture of seborrheic dermatitis. Scar atrophy and hair loss occur in the affected areas, leading to alopecia.
For a long time (on average 5-7 years) manifestations of pemphigus erythematosus affect only the scalp. In the future, the generalization of the process may occur with the appearance of rashes on the skin of the interscapular area, chest and back. Erythematous spots located on the trunk are covered with dense crusts, the removal of which causes pain to the patient. When removing the crusts from the surface of the spot, open mouths of hair follicles are found under them, having the appearance of spikes.
In approximately 33% of patients with pemphigus erythematosus, skin manifestations of the disease are accompanied by damage to the mucous membranes. There is a positive symptom of Nikolsky. In some patients, typical eruptions of pemphigus erythematosus are combined with crusted papules or foci with fatty, well-removable scales characteristic of seborrhea.
Typical clinical manifestations and localization of lesions allow the dermatologist to assume the erythematous form of pemphigus. The diagnosis is confirmed by a positive symptom of Nikolsky, the detection of acantholytic cells typical of pemphigus in smears, the results of histological and immunological examination of the material taken by biopsy.
- Histological examination reveals the presence of blisters and cracks in the area of the horny and granular layers of the epidermis, follicular hyperkeratosis.
- During direct immunofluorescence, a “lupus band” running along the basement membrane and accumulations of intercellular antibodies in the epidermis are observed. Indirect immunofluorescence also detects antibodies, but does not give a “lupus band”.
- In the blood of patients with pemphigus erythematosus, antinuclear antibodies characteristic of systemic lupus erythematosus, antibodies to SS-A and ds DNA are often found.
Differential diagnosis of pemphigus erythematosus is carried out with seborrheic eczema, systemic lupus erythematosus, rosacea, actinic dermatitis, other forms of pemphigus, multiform exudative erythema.
The main therapy of pemphigus erythematosus consists in the systemic administration of glucocorticoids (prednisolone, triamcinolone, methylprednisolone). If the patient has a stomach ulcer or peptic ulcer of the 12p. intestine, drugs are administered intramuscularly. In the generalized form, methods of extracorporeal hemocorrection are shown to purify the patient’s blood from the immune complexes circulating in it:
- cascade plasma filtration;
Local treatment of pemphigus erythematosus consists in the application of occlusive dressings with glucocorticoid ointments and intraocular administration of triamcinolone.