Toxic epidermal necrolysis (TEN) is a critical life-threating condition developing as the total detachment of epidermidis and characterized by severe pathological reactions of all body systems. The current article describes two cases of TEN with similar clinical and laboratory signs. In one case the diagnosis of TEN was subsequently refused.The objective: analysis of methods of clinical and differential diagnostics of conditions accompanied with massive epidermidis detachment in ICU patients.Results. The immunomorphological evaluation of skin specimen obtained from the patient with a torpid form of TEN showed linear IgG fixation in the intercellular space of stratum basale, stratum spinosum and stratum granulosum and C3 fixation in the intercellular space of stratum basale.Conclusion. The complex of anamnesis data and pathomorphological evaluation of skin are crucial for the diagnosis and treatment of patients with atypical TEN.
Aim: To assess clinical efficacy of extra-corporeal photochemotherapy (EPCT) in the treatment of rare lymphomas – lymphomatoid papulosis and folliculotropic mycosis fungoides. Materials and methods: This is a presentation of two cases of lymphomatoid papulosis and folliculotropic mycosis fungoides treated with EPCT with duration of follow-up 9 and 12 years. Extracorporeal photochemotherapy involved administration of 8-methoxypsoralen 0.6 mg/kg 1.5–2 hours before the initiation of intermittent flow separation of mononuclear cells using Haemonetics MCS+ blood cells separator and corresponding protocol. Cell suspension was exposed to UVA-radiation (λ=320–400 nm) using blood irradiator Julia (10–15 ml/min) during 30 minutes then re-infused. In total, 4 procedures were conducted every other day. Results: Both patients demonstrated positive effect involving regression of the rashes after 3 EPCT cycles. Subsequently, the patients received maintenance EPCT 2–3 times a year. Conclusion: High clinical efficacy of EPCT was demonstrated in 2 patients with lymphomatoid papulosis and folliculotropic mycosis fungoides after 9 and 12 years of follow-up.
Aim: To assess clinical efficacy of extracorporeal photochemotherapy in patients with systemic lupus erythematosus (SLE). Materials and methods: 30 SLE patients were thoroughly examined. 16 of them received medical treatment and extracorporeal photochemotherapy (treatment group), 14 patients – routine therapy only (controls). Extracorporeal photochemotherapy involved administration of 8-methoxypsoralen 0.6 mg/kg 1.5–2 hours before the initiation of intermittent flow separation of mononuclear cells using Haemonetics MCS+ blood cells separator and corresponding protocol. Cell suspension was exposed to UV-radiation (λ=320– 400 nm) using blood irradiator Julia (10–15 ml/min) during 30 minutes then re-infused. In total, 4 procedures were conducted every other day. Results: After 3 courses of extracorporeal photochemotherapy, effect (reduced eruptions) was obtained in 14 patients (46.7%). Then, the patients underwent maintenance photochemotherapy 2–3 times a year. Conclusion: Extracorporeal photochemotherapy was effective in the treatment of SLE. Immunological studies have demonstrated pathogenetic effects of extracorporeal photochemotherapy.
Background: Psoriasis is the most prevalent chronic dermatosis of an autoimmune origin that is characterized by increasing incidence of both severe clinical forms and complications, the most threatening of which is psoriatic arthritis. Its treatment includes aromatic retinoids, immunosuppressant therapies (immunosuppressant agents, glucocorticosteroids), PUVA-therapy and other methods. However, these are insufficiently effective in clinical practice and are frequently associated with serious adverse reactions and complications. Aim: To increase the efficacy of treatment for psoriasis associated with psoriatic arthritis by means of incorporation of a new immunobiological method, the extracorporeal photochemotherapy (EPCT) into the standard treatment protocol. Materials and methods: We conducted a prospective cohort study with an active control. Seventy patients with various forms of psoriasis associated with psoriatic arthritis were randomized with stratifi-cation into two groups. The patients of the main group (n = 35) were treated with EPCT, whereas those from the control group (n = 35) received the standard treatment. The EPCT method included isolation of mononuclear cells preliminary sensitized with 8-methoxypsoralen with a cell separator Haemonetics MCS+. After the cell suspension was treated with UV А (λ = 320–400 nm), it was re-infused to the patient. The treatment course included 4 sessions performed every other day. Results: The analysis of clinical efficacy of EPCT in the combination treatment of psoriasis associated with psoriatic arthritis demonstrated that in the majority of cases a significant therapeutic effect was achieved. The mean PASI index decreased from 28.5 ± 1.63 to 6.6 ± 1.7 (p < 0.05), the activity of psoriatic arthritis (DAS score), from 3.7 ± 0.35 to 1.7 ± 0.36 (p < 0.05). This significant treatment effect was associated with a decreased correlation between expression of activation molecules HLADr by natural killer cells (r = 0.6, p < 0.05) and of integrin adhesion molecule CD11b (r = 0.7, p < 0.001). Restoration of apoptosis of autoaggressive cytolitic cells (CD8) determined an improvement in homeostatic imbalance between activation and tolerance. Conclusion: Incorporation of EPCT into the standard protocol of treatment of patients with psoriasis associated with psoriatic arthritis is considered to be highly effective and pathophysiologically based treatment method that allows for cessation of the pathological process within a short time, with further regression of clinical symptoms. The treatment effect is explained by correction of immune regulatory mechanisms that provide restoration and maintenance of immune homeostasis.
The article describes treatment experience with a tumor stage of mycosis fungoides patient treated with extracorporeal photochemotherapy that showed high clinical efficacy. The patient responded with significant regression of the skin elements after two courses of extracorporeal photochemotherapy. The follow-up period was 5 months and is ongoing, as is the treatment.
True pemphigus belongs to the group of autoimmune bullous dermatoses of the skin and mucosa, in which the key role is played by circulating autoantibodies (IgG) to the epidermal desmosomal system antigens. The disease is diagnosed by cytological and histological methods, but one of the main diagnostic methods is direct immunofluorescent study of the skin. Autoimmune pemphigus can be a separate disease or be associated with other skin diseases, such as psoriasis vulgaris, psoriasis guttata, lupus erythematosus, Layell ’s syndrome, dermatomyositis, pyogenic granuloma, vitiligo, folliculitis suffodiens. A case with pemphigus vulgaris combination with giant acantholytic seborrheic keratosis, involving the skin of the hairy part of the head, is described. Published data on atypical location ofvegetating pemphigus foci and detection of acantholysis in foci on the skin of the hairy part of the head necessitated differential diagnosis between vegetating pemphigus and pemphigus vulgaris combined with giant seborrheic keratosis. This case demonstrated the necessity of thorough collection of anamnesis and of all studies needed for correct diagnosis.
AIM:To evaluate the clinical efficiency of extracorporeal photochemotherapy (EPCI) in the treatment of psoriasis (Ps) and Ps associated with psoriatic arthritis (PsA).SUBJECTS AND METHODS:Ninety-three patients with different forms of psoriasis were examined. A study group (SG) comprised 52 patients who were treated with EPCT; a control group (CG) included 41 patients. All the patients received pharmacotherapy generally accepted for these diseases. The SG patients were given additionally EPCT (the patient took 8-methoxypsoralen in a dose of 0.6 mg/kg 1.5-2 hours before the procedure). Mononuclear cells were isolated from the patients' blood in the intermittent-line mode on a Haemonetics MCS+ cell separator. The cell suspension was irradiated with ultraviolet light A (lambda = 320-400 nm) on a JULIA irradiator at 10-15 mI/mm for 30 mm and reinfused in the patient. The course of therapy consisted of 4 sessions performed on alternate days.RESULTS:A varying positive effect was obtained in 49 (94%) SG patients; the mean PASI scores fell from 19.7 +/- 3.4 to 6.7 +/- 2.1 (p < 0.05). The DAS reflecting the activity of PsA reduced on average from 3.35 +/- 0.7 to 2.16 +/- 0.5 (p < 0.05), which corresponded to the change of PsA activity from moderate to weak. In CG, the positive effect was less pronounced in 27 (66%) patients, the PASI scores dropped on average from 19.2 +/- 3.7 to 12.2 +/- 3.1 (p < 0.05), DAS in patients with PsA decreased from 3.24 +/- 0.8 to 2.95 +/- 0.7 (p < 0.05).CONCLUSION:EPCT showed a high efficiency in patients with psoriasis and Ps associated with PsA; the immunological studies demonstrated the pathogenetic direction of the technique.