Coexistence of Parry-Romberg syndrome with homolateral segmental vitiligo

Parry-Romberg syndrome or progressive facial hemiatrophy was first described by Caleb Parry in 1825 and Moritz Romberg in 1846. This disorder is characterized by slowly progressing acquired unilateral hemifacial atrophy, which affects subcutaneous tissue together with the muscles and underlying bones. The pathogenesis and precise incidence of the syndrome remain unclear. Immune-mediated processes and disturbed central regulation, leading to the hyperactivity of the sympathetic nervous system, are primarily considered in the pathogenesis of this disorder. Parry-Romberg syndrome and localized scleroderma are considered to be interrelated as both of them have a similar clinicopathological appearance. We report the case of a 46-year-old man affected by both progressive atrophy of the left side of the face and homolateral, segmental vitiligo in the left side of the trunk and face

Palisaded neutrophilic and granulomatous dermatitis : cutaneous manifestation of Lyme disease or connected with CTD? Case report

Introduction. Palisaded neutrophilic and granulomatous dermatitis (PNGD) are terms which include such diseases as rheumatoid nodules, Churg-Strauss granuloma, and interstitial granulomatous dermatitis with arthritis. This heterogeneous group was first described in 1965. It is associated with immunological diseases. There are reported cases of coexistence with systemic lupus erythematosus, rheumatoid arthritis, Wegener‘s granuloma, inflammatory bowel disease, generalized vascular inflammation, and lymphoproliferative disorders. The etiology of the disease is unknown. It is probable that the deposition of immune complexes in blood vessels leads to cutaneus leukocytoclastic vasculitis, degeneration of collagen fibres and palisaded granulomatous inflammation. Ultimately, this leads to fibrosis of the skin. Objective. The aim of the study is to present a patient with skin lesions and histopathological features of palisaded neutrophilic and granulomatous dermatitis during the course of infection Borrelia burgdorferi. Several cases of this disease have been reported worldwide (30 entries in the PubMed database). To-date, there have been no reports of PNGD in Polish literature. Materials and method. The patient, aged 72, was admitted to hospital because of erythematous, indurated lesion of the skin on the side surface of the left thigh. Diagnosis of PNGD was made on the basis of typical histopathologic features due to clinical symptoms. Conclusions. Clinical diagnosis of PNGD is difficult, and is based mainly on the histopathological picture. Systemic therapy is incorporated mainly due to the systemic disease. The patient requires further observation in the direction of associated systemic disorders

The level of proinflammatory cytokines : interleukins 12, 23, 17 and tumor necrosis factor \alpha in patients with metabolic syndrome accompanying severe psoriasis and psoriatic arthritis

Introduction: The incidence of metabolic syndrome is estimated at 15-24% in the general population and at 30-50% in patients with psoriasis. A probable cause of the described correlation is a constant release in chronic dermatosis of proinflammatory cytokines and their influence on individual systems and organs. Aim: Assessment of the concentration of the proinflammatory cytokines (IL-12, IL-23, IL-17 and $TNF-\alpha$) in blood serum and their correlation with the intensity of skin lesions, the presence of psoriatic arthritis and the risk of development of obesity and metabolic syndrome. Material and methods: The concentrations of subunit p70 IL-12, IL-17 and IL-23, and $TNF-\alpha$ in subjects with psoriasis and metabolic syndrome were determined. Results: The level of the studied cytokines, IL-17, IL-23 and $TNF-\alpha$ was higher in patients diagnosed with psoriasis. Higher levels of IL-17, IL-23 and $TNF-\alpha$ were observed in patients with metabolic syndrome accompanying psoriasis. A higher level of IL-17 and IL-23 was found in sera of patients with psoriatic arthritis in comparison to normal psoriasis. Conclusions: In the study, a higher level of IL-17 and IL-23 was also shown in patients with psoriatic arthritis in comparison to patients with normal psoriasis. The effectiveness of anti-IL12/23 drugs in psoriatic arthritis is a confirmation of the obtained results of the studies. Additionally, the increased level of IL-17, both in patients with metabolic syndrome and with psoriasis, could indirectly indicate an increased cardiovascular risk in patients with affected joints in comparison to psoriasis affecting only the skin

Autoimmunogenicity during anti-TNF therapy in patients with psoriasis and psoriatic arthritis

Introduction: The tumor necrosis factor ($TNF-\alpha$) was initially described as lymphotoxin or cachectin. The discovery of therapies blocking the action of $TNF-\alpha$, in 1988, started a new era in the therapy. One of often reported adverse effects related to the use of $TNF-\alpha$ antagonists is induction of the formation of autologous antibodies and antibodies neutralizing anti-TNF drugs. The development of anti-TNF-induced lupus or classical drug-induced lupus is more rarely reported. Aim: To evaluate the presence and the level of anti-nuclear antibodies in patients with psoriasis and psoriatic arthritis and the influence of anti-TNF therapy used on the concentration of antinuclear antibody (ANA). Material and methods: A total of 28 subjects were included in the study. 71.4% of subjects were diagnosed with psoriatic arthritis and 28.6% with plaque psoriasis. Results: Among the patients with plaque psoriasis, the antinuclear antibodies were found in 25% of subjects and in 80% of patients with psoriatic arthritis. After the treatment an increase in the titer or appearance of antibodies was found in 66.7% in the infliximab group, 18.2% in the etanercept group and 54.7% in the adalimumab group. No subjects developed symptoms of drug-induced systemic lupus. Conclusions: Our findings have shown that all anti-TNF therapies induced ANA in psoriatic arthritis and psoriatic patients. Considering a mild course of lupus induced by anti-TNF treatment and, usually intrinsic, resolution of symptoms, the biological therapy still appears as a safe treatment for patients

Level of inflammatory cytokines tumour necrosis factor α\alpha, interleukins 12, 23 and 17 in patients with psoriasis in the context of metabolic syndrome

Introduction: Psoriasis is a chronic inflammatory skin disease with immunologic etiology. Aim: To investigate the levels of the proinflammatory cytokines tumor necrosis factor \alpha ($TNF-\alpha$), interleukin 23 (IL-23) and IL-17 in patients with psoriasis and psoriatic arthritis with concomitant metabolic syndrome. Material and methods: This study included 60 patients with severe psoriasis. Results: In patients with arterial hypertension concomitant with psoriasis, no statistically significant differences in cytokine levels were observed. On the other hand, in the group of patients diagnosed with diabetes, an increased level of IL-17 was observed. In patients with lipid disorders, the results were similar to the results of patients with diabetes. Conclusions: It is very important to study immunologic mechanisms responsible for the presence and severity of psoriasis, in order to personalize the therapy in the future and optimize the effect of action on the basic disease and on concomitant disorders

INF−γINF-\gamma serum level in patients with morphoea and atrophoderma Pasini-Pierini considering clinical activity of disease

The fundamental component in the pathogenesis of systemic scleroderma, as well as localized scleroderma, is immunologically determined fibrosis. $INF-\gamma$ is considered to be a strong, natural fibrosis inhibitor. Low level of this cytokine was observed in other skin diseases with the course of fibrosis. The aim of the study was to measure $INF-\gamma$ level in serum collected from patients with localized scleroderma. The group of localized scleroderma patients comprised 18 patients with en plaque type of localized scleroderma (M), 18 patients with atrophoderma Pasini-Pierini (APP) and 5 patients with both types of skin lesions (M + APP). Control group serum was taken from 18 healthy volunteers. $INF-\gamma$ level was assessed with the ELISA method. $INF-\gamma$ serum level was lower in all patients with localized scleroderma in comparison with the control group. $INF-\gamma$ serum level in patients with M + APP was also lower, whereas in patients with APP it was higher than in the control group. No statistical significance was observed. INF-\gamma serum level was significantly (p=0.04) lower in patients with the active stage of M compared with patients with the inactive one. Moreover, it was lower - but not significantly - in the active stage of M + APP and the whole group of patients with localized scleroderma than in inactive ones. $INF-\gamma$ serum level was higher only in the active stage of APP. $INF-\gamma$ serum level correlated with the number and area of skin lesions in all LS patients. The higher the number of skin lesions present, the lower was the $INF-\gamma$ serum level observed. The lowest $INF-\gamma$ serum level was observed in patients with greater than 30 cm2 area of skin affected by lesions (p=0.003). $INF-\gamma$ serum level was lower in all patients with localized scleroderma in comparison with the control group. Moreover, $INF-\gamma$ serum level was lower in the active stage of diseases in comparison with the inactive one. Serum level of $INF-\gamma$ might be a useful factor in predicting the intensity of skin fibrosis in localized scleroderm