Sociodemographic, clinical, laboratory, treatment and prognostic characteristics of 156 generalized pustular psoriasis patients in Turkey: a multicentre case series.

BACKGROUND: Generalized pustular psoriasis (GPP) is a rare and severe inflammatory disease characterized by widespread and superficial sterile pustules on an erythematous background. OBJECTIVES: This multicentre study aimed to determine the clinical profile and course in a large cohort of patients with GPP. METHODS: One hundred and fifty-six GPP patients (mean age, 44.2 ± 18.7 years) who met the diagnostic criteria of the European Consensus Report of GPP were included in the study. Sociodemographic characteristics, quality of life, triggering factors of the disease, clinical, laboratory, treatment and prognostic features were evaluated. RESULTS: 61.5% of the patients were female. The rate of working at or below the minimum wage (≤$332.5/month) was 44.9%. Drugs (36.5%) were the most common trigger. While hypocalcaemia (35.7%) was the most important cause of GPP during pregnancy, systemic steroid withdrawal (20%) was the most frequently reported trigger for infantile/juvenile and mixed-type GPP (15%) (P < 0.05). Acute GPP (53.8%) was the most common clinic. Nails were affected in 43.6% of patients, and subungual yellow spots (28.2%) were the most common change. In annular GPP, fever (P < 0.001) and relapse frequency (P = 0.006) were lower than other subtypes, and the number of hospitalizations (P = 0.002) was lower than acute GPP. GPP appeared at a later age in those with a history of psoriasis (P = 0.045). DLQI score (P = 0.049) and joint involvement (P = 0.016) were also higher in this group. Infantile/juvenile GPP was observed in 16.02% of all patients, and arthritis was lower in this group (24.4 vs. 16%). GPP of pregnancy had the worst prognosis due to abortion observed in three patients. CONCLUSIONS: Recent advances in treatment have improved mortality associated with GPP, but abortion remains a significant complication. Although TNF-α inhibitors have proven efficacy in GPP, they can also trigger the disease. Mixed-type GPP is more similar to acute GPP than annular GPP with systemic manifestations and course.

Clinical characteristics, quality of life and risk factors for severity in palmoplantar pustulosis: a cross-sectional, multicentre study of 263 patients.

BACKGROUND: Palmoplantar pustulosis (PPP) is a rare, chronic, inflammatory skin disease characterized by sterile pustules on palmar or plantar areas. Data on PPP are scarce. AIM: To investigate the clinical characteristics and risk factors for disease severity in a large cohort of Turkish patients with PPP. METHODS: We conducted a cross-sectional, multicentre study of patients with PPP recruited from 21 tertiary centres across Turkey. RESULTS: In total, 263 patients (165 women, 98 men) were evaluated. Most patients (75.6%) were former or current smokers. The mean Palmoplantar Pustulosis Area and Severity Index (PPPASI) was 8.70 ± 8.06 and the mean Dermatology Life Quality Index (DLQI) score was 6.87 ± 6.08, and these scores were significantly correlated (r = 0.52, P < 0.001). Regression analysis showed that current smoking was significantly associated with increased PPPASI (P = 0.03). Coexisting psoriasis vulgaris (PsV) was reported by 70 (26.6%) patients. Male sex prevalence, PPP onset incidence, disease duration, DLQI, and prevalence of nail involvement and psoriatic arthritis (PsA) were significantly increased among patients with PPP with PsV. Of the 263 patients, 18 (6.8%) had paradoxical PPP induced by biologic therapy, and these patients had significantly increased mean DLQI and prevalence of PsA (r = 0.03, P = 0.001). CONCLUSION: Our data suggest that smoking is a risk factor for both PPP development and disease severity. Patients with PPP with PsV present distinct clinical features and patients with biologic therapy-induced paradoxical PPP have reduced quality of life and are more likely to have PsA.

Clinical and demographic features of hidradenitis suppurativa: a multicentre study of 1221 patients with an analysis of risk factors associated with disease severity.

BACKGROUND: Hidradenitis suppurativa (HS) is a chronic, relapsing and debilitating inflammatory disease associated with profound morbidity. AIM: In this multicentre study, we investigated the demographic and clinical features of HS, and determined risk factors of disease severity. METHODS: In total, 1221 patients diagnosed with HS from 29 centres were enrolled, and the medical records of each patient were reviewed. RESULTS: The mean age of disease onset was 26.2 ± 10.4 years, and almost 70% (n = 849) of patients were current or former smokers. Mean disease duration was 8.9 ± 8.4 years with a delay in diagnosis of 5.8 ± 3.91 years. Just over a fifth (21%; n = 256) of patients had a family history of HS. The axillary, genital and neck regions were more frequently affected in men than in women, and the inframammary region was more frequently affected in women than in men (P < 0.05 for all). Acne (40.8%), pilonidal sinus (23.6%) and diabetes mellitus (12.6%) were the most prevalent associated diseases. Of the various therapies used, antibiotics (76.4%) were most common followed by retinoids (41.7%), surgical interventions (32.0%) and biologic agents (15.4%). Logistic regression analysis revealed that the most important determinants of disease severity were male sex (OR = 2.21) and involvement of the genitals (OR = 3.39) and inguinal region (OR = 2.25). More severe disease was associated with comorbidity, longer disease duration, longer diagnosis delay and a higher number of smoking pack-years. CONCLUSIONS: Our nationwide cohort study found demographic and clinical variation in HS, which may help broaden the understanding of HS and factors associated with disease severity.

Hidradenitis Suppurativa: a lesser-known cause of AA amyloidosis.

BACKGROUND: Hidradenitis suppurativa (HS) is a chronic, disabling skin disease. The estimated prevalence is 1-4 % worldwide. HS is a systemic inflammatory disease and can cause AA amyloidosis. The first report of HS-related amyloidosis dates back to 1966; since then, sporadic cases have been reported. Our work will be the first case series on HS and AA amyloidosis. CASE SERIES: We report eight HS cases complicated with amyloidosis. Six patients were male. The median age was 44 years, and the median disease duration before the amyloidosis diagnosis was 15.5 years. In a mean follow-up of 18 ± 6 months, we achieved favorable renal responses in four of the eight cases (50 %). All cases had a dermatologic response, with four complete and four partial remissions. CONCLUSION: HS is a systemic inflammatory disorder that may cause AA amyloidosis. Aggressive treatment of HS may halt the progression of amyloidosis. HIPPOKRATIA 2020, 24(1): 33-37.

Expression of human neutrophil proteins in acne vulgaris.

BACKGROUND: In acne vulgaris patients, the presence of a dysregulation of the production of innate and specific antimicrobial peptides has been postulated. OBJECTIVE: This study aims to determine whether human neutrophil proteins (HNP) 1-3 are expressed in acne patients. MATERIALS AND METHODS: HNP 1-3 expression was investigated in 35 acne patients treated with isotretinoin and in 25 healthy subjects. At the beginning of the study, two skin biopsies were taken from acne patients; one biopsy was taken from an established pustule and one from uninvolved skin, and the biopsies were repeated after treatment. Only one biopsy was obtained from controls. RESULTS: The statistical analysis showed that pustular lesions of acne patients had significantly higher levels of perivascular and interstitial HNP 1-3 expression when compared with the biopsy of uninvolved skin of these patients (P = 0.003, P = 0.001, respectively) and with that of healthy controls (P = 0.007, P = 0.014, respectively). Isotretinoin treatment achieved a decrease in the perivascular and interstitial HNP 1-3 expression of pustular lesions (P = 0.01, P = 0.001, respectively). CONCLUSION: Our current study demonstrates the novel observation that a recently identified antimicrobial peptide, HNP 1-3, is expressed in neutrophils of acne inflammation but not in uninvolved skin of these patients. These results suggest that HNP 1-3 may contribute to the development of inflammatory lesions of acne.

Therapeutic options for palmoplantar pustulosis.

Palmoplantar pustulosis (PPP) is a common chronic and recurrent pustular dermatosis characterized by multiple sterile pustules and erythematous plaques on the palms and soles. The exact cause and pathogenesis of the disease remain unknown, and there is still debate about whether PPP is a variant of psoriasis or a distinct condition. A review of the medical literature revealed that a wide range of treatments have been used in the treatment of PPP over the years. The literature in PPP is restricted to case reports or small case series, and there is a lack of well-documented clinical studies, which makes it difficult to select an ideal therapy for the condition. The purpose of this review is to discuss the current therapy options for PPP, based on results of randomized controlled trials.

Easy to diagnose, difficult to treat: keratosis lichenoides chronica.

Keratosis lichenoides chronica (KLC) is a rare disease, with approximately 70 cases reported in the literature. The problem in this long-lasting disease is generally the treatment, not the diagnosis. In the literature, many treatments failed to show any beneficial effect. We present a 20-year-old man with KLC that was successfully treated with a combination of phototherapy, acitretin and calcipotriol ointment. The patient's lesions showed a marked improvement with this combination. To our knowledge, this is the first report of this type of combination treatment being used successfully in KLC. This approach might help reduce doses of retinoids or psoralen ultraviolet A required when these are used separately, and limit the potential toxicity of these treatments.

A retrospective analysis of treatment responses of palmoplantar psoriasis in 114 patients.

BACKGROUND: Treatment options remain unsatisfactory for patients with palmoplantar psoriasis (PP) and palmoplantar pustular psoriasis (PPP). Aim To evaluate the therapeutic responses of PP and PPP patients that were treated in our psoriasis polyclinic between 2003 and 2007. METHODS: This retrospective study comprised PP (n = 62) and PPP (n = 52) patients. Treatments were individualized according to patient compliance and associating systemic diseases. The effect of systemic treatments was grouped as follows: 'no improvement': patients unresponsive for the present treatment; 'partial improvement': < 50% decrease in severity or affected area; 'moderate improvement': 50-75% decrease in severity or affected area, and 'marked improvement': > 75% decrease of the disease compared to baseline. RESULTS: In the PP group, 17 of 62 patients showed marked improvement to topical agents, while the remaining patients required systemic agents including oral retinoids (n = 24), local psoralen plus ultraviolet A (PUVA; n = 12), methotrexate (n = 9) and cyclosporine (n = 2). Marked improvement was achieved in 53%, 45%, 47% and 100%, respectively. In these patients, two (n = 10), three (n = 5), or four (n = 5) systemic agents were used alternately. In the PPP group, 18 of 52 patients achieved marked improvement by topical agents. Patients that required systemic agents were treated with colchicum (n = 19), local PUVA (n = 8), methotrexate (n = 4), oral retinoids (n = 3) and cyclosporine (n = 2). These treatments achieved a marked improvement in 60%, 33%, 57%, 83%, and 50% of the patients, respectively. In the course of the disease, 18 patients required two and 3 patients required three systemic agents alternately. CONCLUSIONS: Although the success rates appeared to be high, the high number of patients who required multiple systemic agents emphasized the fact that localized forms of psoriasis were resistant to therapy.

The role of human herpesvirus 6, human herpesvirus 7, Epstein-Barr virus and cytomegalovirus in the aetiology of pityriasis rosea.

AIM: To identify the role of human herpesvirus 6 (HHV-6), HHV-7, Epstein-Barr virus (EBV) and cytomegalovirus (CMV) in the pathogenesis of pityriasis rosea (PR). MATERIAL: Polymerase chain reaction with specific primers for HHV-6 and HHV-7 DNA sequences was performed on the blood and tissue samples of 25 patients with PR and on the blood samples of age- and sex-matched healthy controls. HHV-6, EBV, CMV immunoglobulin M (IgM) and IgG were analysed by enzyme-linked immunosorbent assay, HHV-7 IgM and IgG were analysed by indirect immunofluorescence on the serum samples of the study population. In the patient group, the values were studied 2 weeks later again (second control). RESULTS: There were no differences between the first and second controls of the patients and healthy subjects regarding HHV-6 IgM, HHV-7 IgM, CMV IgM, EBV IgM results. There were significant differences between the first [HHV-6 DNA (2 of 25), HHV-7 DNA (6 of 25)] and second control [HHV-6 DNA (1 of 25), HHV-7 DNA (11 of 25)] of the patients for the blood samples in favour of HHV-7. PR patients showed higher amounts of HHV-6 and HHV-7 DNA positivity when compared with that of healthy subjects. HHV-7 seemed to be more important regarding tissue samples [HHV-6 DNA (7 of 25), HHV-7 DNA (12 of 25) first control, HHV-6 DNA (6 of 25), HHV-7 DNA (12 of 25) second control] as well as blood samples. CONCLUSION: Though our results failed to support a causal relationship among EBV, CMV and PR, they indicated a possible role for HHV-6 and especially HHV-7 in a group of Turkish patients but other aetiological factors may exist.

When there is no single best biological agent: psoriasis and psoriatic arthritis in the same patient responding to two different biological agents.

Guidelines and treatment strategies for the new biological agents have been developed, but dermatologists continue to face difficulties in adopting these guidelines into their daily practices. We report a patient with psoriasis and psoriatic arthritis whose skin lesions responded only to efalizumab, and the arthritis to etanercept. This case shows that different biological agents may achieve different success rates even in the same patient. Each biological agent offers different advantages and disadvantages, which sometimes make it difficult to choose the single best agent for a patient. Psoriasis often becomes one of the most difficult diseases to treat and does not respond to any single antipsoriatic agent. Perhaps in the future, rotational or combination treatment with different biological treatments will be used.

The effects of calcipotriol and methylprednisolone aseponate on bcl-2, p53 and ki-67 expression in psoriasis.

OBJECTIVE: The decrease of physiological apoptosis in the psoriatic lesions is thought to be involved in the pathogenesis of psoriasis, and induction of apoptosis was shown to contribute to the regression of psoriatic hyperplasia. In the present study, we compared the effects of calcipotriol and methylprednisolone aseponate (MPA) treatments on bcl-2, p53 and ki-67 expressions in psoriatic patients in order to define a relationship between regulation of apoptosis and healing process in psoriasis. METHODS: Thirty psoriatic patients with stable and moderate chronic plaque psoriasis applied either calcipotriol or MPA ointment for 6 weeks twice daily. Evaluation of bcl-2, p53 and ki-67 positivity was performed at baseline and was repeated at sixth week for each therapy. RESULTS: The mean percentage of positive keratinocytes was 8.63 +/- 7.15% for p53, 20.66 +/- 14.45% for ki-67, and 3.74 +/- 2.83% for bcl-2 in psoriatic skin at baseline. Normal skin values were 3.27 +/- 3.21% for p53, 4.93 +/- 4.77% for ki-67, and 1.80 +/- 0.41% for bcl-2. The psoriatic skin showed higher ki-67 (P < 0.05) and bcl-2 (P < 0.05) expression rates when compared to normal skin. The p53 positivity observed in psoriatic skin and normal skin was not significantly different (P > 0.05). Following calcipotriol and MPA treatments, there was a significant reduction in p53 and ki-67 positivity accompanied by an increase in bcl-2 positivity (P < 0.05 each). No significant differences were found at sixth week between calcipotriol and MPA groups with respect to p53, ki-67 and bcl-2 positivity (P > 0.05). The post-treatment psoriatic skin showed lower expression of p53, higher expressions of ki-67 and bcl-2 when compared to normal skin (P < 0.05 each). CONCLUSION: The results of this study provide evidence that both calcipotriol and MPA decrease the p53 and ki-67 expression and increase bcl-2 expression. However, it should further be elucidated if these changes were the common behaviour of psoriatic keratinocytes to any antipsoriatic medication.