For Dermatologists

Plasmapheresis in the treatment of atopic dermatitis in Zaporizhzhia.

The aim of plasmapheresis in atopic dermatitis is the removal of immunoglobulin E (IgE) from the blood. The procedure is carried out together with drug therapy (administration of antihistamines, sedatives and psychotropic drugs, systemic corticosteroids and cytostatics). The course of treatment is 8-12 sessions of intermittent PP with the removal of 700 – 1000 ml of plasma. The result of PP with replacement of the removed plasma by hemodilutant solutions is an improvement of microcirculation and transcapillary exchange in the tissues, as well as a reduction of the total peripheral resistance.

Criteria for assessing effectiveness:

• After the initial decrease of IgE within 6-21 hours due to mechanical removal, an increase of its concentration is observed after 48 hours. However, with repeated PP procedures the IgE level decreases significantly;
• The level of circulating immune complexes fluctuates in a similar way;
• After 3-4 PP sessions the pathological process stabilizes, itching stops, swelling and hyperemia decrease sharply.
• After completion of the PP course the acute manifestations of the disease undergo remission, the infiltration partially or completely resolves.

Plasmapheresis in the treatment of chronic urticaria in Zaporizhzhia

Indications: membrane plasmapheresis is used in the combined treatment of urticaria together with drug therapy. Immunoglobulins, especially Ig E, which cause increased sensitivity to light of a certain wavelength, are also involved in the pathogenesis of a particular type of this disease – solar urticaria.

Method: 1-3-5 procedures every 3-5 days (removal of 800-1000 ml each) both in combination with antihistamines and/or corticosteroids and as PP monotherapy. In patients with solar urticaria it is advisable to combine PP with PUVA therapy, which should be started immediately after completion of the PP course.

Criteria for assessing effectiveness:

- a significant reduction of the manifestations of urticaria;

- long-term remission (the exacerbations that occur in a number of cases are usually mild and do not require repeated courses of plasmapheresis).

Plasmapheresis in the treatment of psoriasis

Membrane plasmapheresis in psoriasis is indicated first of all for patients with universal skin involvement - erythroderma, and also with the exudative form of the disease. It is likewise used as an additional method when conventional psoriasis therapy is insufficiently effective, and also in a torpid course of the disease.

Therapy of psoriasis with the use of plasmapheresis can be divided into two stages. At the first stage a course of PP is carried out, and when the progression of the disease and the exudative phenomena stop and partial regression of the rash elements begins, they proceed to the second stage, using active methods of treatment: ultraviolet irradiation, photochemotherapy, selective phototherapy, the use of synthetic retinoid tigazones (tigazones).

Method: 7-10 procedures every 1-2 days with the removal of 800-1000 ml per procedure. PP is combined with the traditional methods of psoriasis treatment. The combination of PP with 1-2 sessions of hemosorption is effective. In this case the number of PP procedures can be reduced to 4-5.

Criteria of effectiveness:

• Reduction of the infiltration in the area of the plaques and subsequent resolution of the rash.
• An increase of the number of T-lymphocytes.
• Stimulation of the phagocytic activity of neutrophils
• Elimination of CIC from the blood
• After the first PP procedure the level of CIC in the blood rises (the rebound phenomenon), which indicates their release from the tissues.

In this connection it is necessary to warn patients about a possible exacerbation of the skin process after the first PP procedure. Repeated sessions lead to accelerated regression of the rash.

Plasmapheresis in the therapy of Lyell's syndrome

Indications: In the pathogenesis of the disease the leading role is played by an allergic reaction that is second in severity only to anaphylactic shock. Immune mechanisms play an important role, in particular the cytotoxic effect on the cells of the epidermis. There is in this case a toxic manifestation of idiosyncrasy of the epidermal cells to various agents.

The application of the eliminating effect of membrane plasmapheresis depends on the etiological variant of Lyell's syndrome - infectious, drug-induced, toxic-allergic or idiopathic.

Method: The drug of choice in the treatment of Lyell's disease is prednisolone, which is used in high doses up to pulse therapy: 1000 mg/day by drip for 3 consecutive days. Detoxification therapy is prescribed in addition and, in this connection, it is advisable to apply PP on the first day of the disease in order to interrupt endotoxicosis.

The volume of plasma removed should be not less than 2.5 litres, and it should be replaced with donor plasma and 4% albumin solution, since, owing to the extent of the area of the lesions, which are represented by large erosions and blisters, a large amount of protein is lost in epidermal necrolysis and it has to be replenished.

Criteria of effectiveness:

• Epithelialization of the lesions
• Relief of the symptoms of intoxication

Plasmapheresis in the therapy of Duhring's dermatitis herpetiformis

Indications: Since in Duhring's dermatitis herpetiformis autoantibodies belonging to various classes of immunoglobulins are detected in the blood of patients in 85-100% of cases, PP is justified in the combined therapy of this disease. Plasmapheresis (PP) is indicated first of all for patients in whom the therapeutic effect is achieved only at a high daily dose of dapsone (300 mg). A course of PP makes it possible to reduce the dose of dapsone from 300 to 100 mg/day, at which the regression of the rash is maintained.

PP is also indicated for patients who have contraindications to the administration of sulfone drugs, and also in a torpidly proceeding sulfone-resistant skin process.

Method: 6-10 procedures every other day with the removal of 800-1000 ml of plasma per session. It should be combined with sulfone, corticosteroid and antihistamine drugs. In case of intolerance to sulfone drugs or a torpid course of the pathological process it can be combined with injections of 1% emoxipine solution.

Criteria for assessing effectiveness:

• Elimination of Ig from the blood,
• Reduction of eosinophilia,
• Regression of itching and resolution of the rash.

Plasmapheresis in the therapy of bullous pemphigoid

Indications: The advisability of using Plasmapheresis (PP) in the combined therapy of bullous pemphigoid is connected with the presence in the blood of an increased level of circulating immune complexes and immunoglobulins. As in pemphigus, they are "washed out" of the blood and the tissues.

Main indications: severe forms of the course of the disease and resistance to the therapy being carried out.

Method: 6-10 procedures every other day with the removal of 800-1000 ml of plasma per session. The combination of PP with oral administration of prednisolone at a dose of 0.5 mg/kg/day is effective. In case of steroid resistance in bullous pemphigoid a course of PP should be added to the therapy after a single intravenous administration of 1000 mg of prednisolone. In such situations, against the background of a gradual reduction of the dose of systemic corticosteroids (over 2-3 months), it is necessary to perform PP procedures 1-2 times a week.

Criteria of effectiveness:

• Cessation of the appearance of new blisters,
• Epithelialization of the erosive surfaces,
• Complete regression of the rash.

Plasmapheresis in the therapy of Arndt-Gottron scleromyxedema

Indications: The etiology and pathogenesis of scleromyxedema (SM) remain insufficiently studied. At present SM is classified among the diseases in the development of which the main role is played by paraproteinemia. In this connection it is considered that the disease is of an immunopathological nature, possibly of autoimmune genesis. At the same time, in patients with SM the paraproteins stimulate the proliferation of fibroblasts and their synthesis of a large amount of glycosaminoglycans. Binding to protein, the latter form high-molecular carbohydrate-protein complexes – proteoglycans. Collagen, elastin, glycoproteins and proteoglycans form the "ground substance" of connective tissue, in which fibroblasts, macrophages and mast cells are located.

Method: The traditionally used therapy with glucocorticoids and cytostatics is not always successful, especially in a long course of the dermatosis. The drugs of these groups make it possible to halt the progression of the process and contribute to a reduction of induration and erythema, but do not provide complete clinical cure and stable remission. Confirmation of the pathogenetic significance of PP is the fact that the elimination of paraproteins from the blood of patients has no effect on the enhanced proliferation of fibroblasts. When only cytostatics are used, normalization of the functional activity of fibroblasts is noted, however a high level of paraproteins in the blood serum is preserved. The combination of immunosuppressive therapy and active "washing out" of paraprotein from the blood by means of PP provides a pronounced clinical effect in patients with scleromyxedema.

Membrane plasmapheresis is performed once every 2 days with a single removal of 600-800 ml of plasma with replacement by physiological saline. 6-10 procedures per course.

Criteria of effectiveness:

• Regression of the "fresh" rash,
• Reduction of the infiltration of the skin,
• Regression of the inflammatory phenomena,
• Long-term remission.

Plasmapheresis in the therapy of cryoglobulinemia

Indications: In this disease the cryoglobulins circulating in the blood are deposited on the walls of the vessels and cause their damage (type I). Cryoglobulins are also able to act on the vascular wall by forming immune complexes (types II and III). Clinically the said pathological process is manifested by the development of angiitis, in connection with which skin lesions are observed in 25% of patients, characterized by the presence of painful ulcerative defects in the distal parts of the lower limbs.

As a rule, the said lesions respond poorly to therapy and therefore PP is regarded as a pathogenetic therapy aimed at the elimination of cryoglobulins and their immune complexes from the blood.

Method: The gold standard of cryoglobulinemia therapy is the use of immunosuppressants, however, in case of their insufficient effectiveness it is recommended to resort to plasmapheresis as an additional method of treating the disease.

PP is performed once every 2 days with a single removal of 1000-1500 ml of plasma with replacement by saline solutions. 5-6 procedures per course.

Criteria of effectiveness:

• Regression of the "fresh" rash,
• Relief of the painful sensations,
• Scarring of the ulcerative defects.

Plasmapheresis in the therapy of erythema elevatum diutinum (EED)

Indications: Despite the insufficient clarity of the etiology of EED, this skin disease is not infrequently associated with paraproteinemia, and immunofluorescence examination of the skin reveals IgA deposits in the dermis. At present there is an opinion that this disease is a variant of leukocytoclastic angiitis. PP is indicated in a severe course of the disease accompanied by ulcerative lesions and general symptoms (fever, arthralgia, myalgia, malaise), and also in case of resistance to traditional therapy.

Method: Plasmapheresis (PP) should be combined with the drugs that are most effective in this disease – corticosteroids and dapsone. If the latter cannot be used, PP can be applied as monotherapy. In severe cases a combination with cytostatics (chlorambucil and cyclophosphamide) is possible. At the same time, the administration of cytostatics without accompanying PP procedures does not always give a significant effect.

PP is performed daily with the removal of 800-1000 ml of plasma with replacement by saline solutions. 8-10 procedures per course.

Criteria of effectiveness:

• Regression of the rash,
• Relief of the general symptoms.

Plasmapheresis in the therapy of pyoderma gangrenosum

Indications: The main role in the occurrence of pyoderma gangrenosum was earlier assigned to staphylococci and streptococci. At present there is an opinion that this disease is a severe form of angiitis that arises against the background of immune disorders. Plasmapheresis (PP) is indicated in case of resistance to traditional therapy.

Method: PP should be combined with antibacterial therapy and prednisolone (40-80 mg per day). PP is performed daily with the removal of 800 – 1000 ml of plasma with partial replacement by 300 – 600 ml of fresh frozen plasma and 400 – 600 ml of saline solutions. 7-10 procedures per course.

Criteria of effectiveness:

• Scarring of the ulcerative defect,
• Reduction of the level of pathological immunoglobulins.

Plasmapheresis in the therapy of scleroderma

Indications: In the pathogenesis of the disease a special role is played by autoimmune reactions. The success of the use of Plasmapheresis (PP) in this form of the disease, and also in the systemic process, is explained by the removal of antibodies as well as by the inhibition of their synthesis

Method: The best effect is observed when PP is combined with systemic corticosteroid therapy. Improvement of the clinical picture of the disease begins after 6-7 procedures. After 10-12 procedures the skin lesions largely undergo regression; in patients with an initial contracture the mobility of the joints improves.

Criteria of effectiveness:

• Cessation of the progression of the skin lesions,
• Improvement of the mobility of the joints.

Plasmapheresis in the therapy of pemphigus

Indications: The therapeutic effect of membrane plasmapheresis in pemphigus is explained by the elimination from the blood of immunoglobulins and circulating immune complexes that have the ability to adhere to the surface of the cells of the spinous layer of the epidermis. IgG cause acantholysis, which is clinically characterized by the appearance of blister elements on the skin. In addition, PP promotes the "washing out" of circulating immune complexes from the intercellular fluid of the tissues into the bloodstream with their subsequent removal together with the plasma. Besides purifying the blood of pathological immunoglobulins and immune complexes, PP inhibits the synthesis of antibodies by B-lymphocytes.

Plasmapheresis applied in the combined treatment of pemphigus serves as a method of pathogenetic therapy owing to the elimination of autoantibodies, leads to a more rapid remission and potentiates the effect of corticosteroid drugs.

PP is also indicated in case of the ineffectiveness of the therapy carried out previously and in steroid-resistant forms of pemphigus.

Method: 6-12 procedures every other day with the removal of 800-1000 ml of plasma per session. PP in the treatment of pemphigus is used as a supplement to the corticosteroid and cytostatic drugs applied. PP in the course of systemic corticosteroid therapy makes it possible to reduce the initial dose of prednisolone rapidly while maintaining the positive dynamics.

In the steroid-resistant form of pemphigus it is advisable to start PP with the simultaneous administration of prednisolone, which is prescribed according to the pulse therapy scheme.

The combination of PP with the administration of corticosteroid and cytostatic drugs makes it possible to prolong the remissions considerably.

After remission is achieved PP can be discontinued, and 1 procedure a week can be performed to maintain the therapeutic effect. A similar approach in the therapy of pemphigus can be successfully used for the purpose of preventing relapses of the disease.

Criteria for assessing effectiveness:

• Elimination from the blood of Ig and CIC that have the ability to adhere to the surface of the cells of the spinous layer of the epidermis (a decrease of the level of immunoglobulins correlates with a rapid onset of improvement in the clinical picture of the disease).
• Inhibition of the synthesis of antibodies by B-lymphocytes.
• Accelerated epithelialization of the erosions and rapid onset of remission.

Plasmapheresis in the therapy of rosacea

Indications: The etiology and pathogenesis of rosacea are multifaceted. In connection with this circumstance the range of methods of therapy of this disease is wide. Nevertheless, the available data on the influence of endogenous irritants such as bradykinin, kallikrein, circulating immune complexes and various toxins make it possible to a certain extent to regard PP as a pathogenetic method of treating rosacea.

Method: plasmapheresis is indicated in torpid forms of the disease that are resistant to traditional methods of treatment. PP is combined with external treatment and systemic administration of trichopol and tetracycline.

Membrane plasmapheresis is performed once every 2 days with a single removal of 600-700 ml of plasma with replacement by physiological saline. 7-10 procedures per course.

Criteria of effectiveness:

• Regression of the rash,
• Long-term remission.