Should children with psoriasis be consulted to a rheumatologist? Result from pediatric rheumatology-dermatology collaboration.

OBJECTIVE: The objectives of this study were to determine the musculoskeletal (MSK) conditions associated with pediatric psoriasis (Pso) and to evaluate the thickness of Achilles tendon of children with Pso and healthy controls (HCs). METHODS: Pso patients who were followed-up in dermatology outpatient clinic were referred to a pediatric rheumatology center. All patients and healthy peers were evaluated with standardized forms. Both patients and controls underwent ultrasonographic evaluation for Achilles tendon thickness. RESULTS: A total of 55 pediatric Pso and 46 healthy children were included in the study. Of patients with Pso 56.4% had arthralgia, 25.5% had lower back pain, 18.2% had heel pain, 12.7% had hip pain, and 10.9% described morning stiffness. Arthritis was detected in 7.3%, sacroiliac tenderness in 12.7%, and enthesitis in 9.1% of the patients. Arthralgia, lower back pain, and heel pain were significantly frequent in Pso group than healthy children median left and right Achilles tendon thicknesses of Pso patients who were significantly greater than that of HCs prevalence of psoriatic arthritis (PsA) among Pso patients was 7.3%. CONCLUSION: Evaluation of a child with Pso regularly for the MSK complaints is critical for the early recognition of PsA. Ultrasonography is a useful technique for screening Pso patients for early detection of enthesopaty.

Clinical course of psoriasis patients that discontinued biologics during the COVID-19 pandemic.

BACKGROUND: Since psoriasis is a chronic disease, it is not recommended to discontinue the treatment agents used. However, in real life, the treatment of psoriasis patients may be interrupted for various reasons. During the pandemic period, the treatment of many patients was also interrupted. OBJECTIVES: To evaluate relapse and clinical worsening in psoriasis patients whose biological therapy was interrupted during the pandemic and reveal associated factors. METHODS: The study included patients aged ≥18 years, who were followed up with moderate and severe chronic psoriasis controlled by the last biological agent [Psoriasis Area Severity Index (PASI) 75 response achieved] but had to discontinue their treatment during the pandemic. The patients' demographic and clinical characteristics, clinical course after the discontinuation of these agents, presence of clinical worsening, and relapse were evaluated. Risk factors were analyzed with the logistic regression analysis. RESULTS: The study included 169 patients, with a mean age of 47.3 ± 14.5 (18-87) years. The mean biologics-free time was 18.2 ± 12.3 (2-56) weeks. Clinical worsening was detected in 41.4% and relapse in 48.5% of the patients. The significant risk factors for clinical worsening and relapse in both univariate and multivariate analyses were alcohol use during the biologics-free period, total time off biologics, and the presence of an additional triggering factor. The use of secukinumab and ustekinumab was found to be a protective factor against clinical worsening in multivariate analyses. CONCLUSION: As the biologics-free period is prolonged, the likelihood of clinical worsening and relapse increases, therefore, we do not recommend discontinuing biological agents.

[Analysis of IFN-γR1 (CD119) and IL-12Rß1 (CD212) Deficiency by Flow Cytometry]. / IFN-γR1 (CD119) ve IL-12Rß1 (CD212) Eksikliginin Akan Hücre Ölçer ile Analizi.

Mendelian susceptibility to mycobacterial diseases (MSMD) is a rare primary immune deficiency (PID). IL-12Rß1 deficiency is the most frequently observed of more than 16 genetic defects that have been identified for MSMD. Genetic and immunological tests are remarkable in the diagnosis of PID. In this study, it was aimed to determine the expression of IFN-γR1 and IL-12Rß1 in patients with MSMD, their relatives, and healthy individuals and to evaluate the importance of flow cytometry as a fast and reliable method in the diagnosis of MSMD. IFN-γR1 and IL-12Rß1 expression levels were analyzed in 32 volunteers including six patients, six relatives, and 20 healthy individuals. The normal range of IFN-γR1 and IL-12Rß1 levels among healthy individuals were determined. IL-12Rß1 expression level in lymphocytes was found to be low in one patient's relative, and less than 1% in three patients and in one patient's relative. It was observed that the IL-12Rß1 expression levels of the patient with STAT1 deficiency were increased compared to the healthy individuals. No difference was found in the expression levels of IFN-γR1 and IL-12Rß1 in one patient, but IFN-γR1 expression was decreased in one patient compared to healthy individuals. Our results show that the determination of IL-12Rß1 and IFN-γR1 deficiencies by flow cytometry can be used as a rapid and reliable method for the diagnosis of MSMD. The use of this method as a screening test will enable early diagnosis especially in patients whose genetic diagnosis has not been confirmed and clinically compatible with MSMD. In addition, it is thought that IL-12Rß1 and IFN-γR1 range data obtained from healthy individuals will be considered as a reference source in routine and research studies to be conducted with MSMD.

New dermatological findings of MIS-C: Can mucocutaneous involvement be associated with Severe Disease Course?

BACKGROUND: Little is known about mucocutaneous involvement in critically ill patients with the multisystem inflammatory syndrome in children (MIS-C). The aim of our study was to describe the localisation and variety of rash and to investigate whether presenting with rash at admission alters the clinical course of MIS-C. METHODS: This prospective, observational cohort study was conducted amongst children under 18 years of age who were admitted to our paediatric intensive care unit (PICU) between May 2020 and May 2021 with a possible diagnosis of MIS-C. RESULTS: A total of 33 children with MIS-C, 21 boys (64%), with a median age of 9.4 years (3.4-11.5) were enrolled. Twenty-four children presented with mucocutaneous symptoms (72%). Age, male gender, PICU length of stay, presenting symptoms, inotrope requirement, the existence of myocarditis or respiratory failure were higher but not significantly different in patients with rash compared to those without rash (P > 0.05). The median duration of symptoms before admission and presence of cervical lymphadenopathy were significantly higher in patients than those without rash (P < 0.05). Children with a rash had a significantly higher neutrophil count, CRP, procalcitonin, troponin levels and lower lymphocyte counts and albumin levels than those without rash (P < 0.05). Twelve children with rash (50%) had symmetrical intertriginous distribution. Two children had erythematous lesions on the areola and the surroundings. In conclusion, intertriginous involvement, periareolar erythema and other mucocutaneous manifestations might be the first alarming symptoms of moderate to severe MIS-C. Therefore, close monitoring with a multidisciplinary approach should be considered for these patients to assess potential disease progression.

Towards a combined pediatric rheumatology-dermatology clinic: One-year experience.

OBJECTIVE: Dermatological findings may be the sole complaints of diseases in pediatric rheumatology practice. Evaluating patients with a multi-disciplinary approach may facilitate access to an accurate diagnosis. Herein, we reported our one-year experience in collaborative pediatric rheumatology-dermatology. METHODS: Patients were initially evaluated separately in pediatric rheumatology-dermatology outpatient clinics. Subsequently, once a week, the final diagnoses of patients with suspected skin rash were collaboratively discussed by two pediatric rheumatologists and a dermatologist. RESULTS: A hundred and one patients were included in this study. Of these 101 patients, 65 attended to dermatology outpatient clinic initially, while the remaining 36 applied to the pediatric rheumatology outpatient clinic. The most common mucocutaneous finding was squamous lesions in 30 patients, followed by erythematous lesions in 28 and mucosal ulcers in 14. Finally, 69 patients were diagnosed with a rheumatic disease while 32 had differential diagnoses apart from rheumatic diseases. CONCLUSION: Patients with rheumatologic diseases frequently present with only mucocutaneous findings. Thus, a detailed examination of the mucosa, skin and its attachments is of paramount importance in rheumatology practice. We suggest that a close interaction between pediatric rheumatology-dermatology and the formation of consensus clinics are going to assist clinicians in making easier and accurate diagnoses.

Adalimumab-induced scalp psoriasis with severe alopecia.

In recent years, with the increase usage of tumor necrosis factor (TNF) inhibitors, more side effects have revealed. The incidence of paradoxical psoriasis (psoriasis vulgaris, palmoplantar pustulosis, scalp psoriasis, or their combinations) ranges from 1 to 5%; however alopecia due to anti-TNF-α-induced scalp psoriasis, rarely reported in the literature. We report a 37-year-old woman who developed palmoplantar pustulosis and scalp psoriasis with severe alopecia after 2 months of treatment with adalimumab for chronic plaque psoriasis. Biopsies from the palmar and scalp lesions showed psoriasiform changes. Adalimumab treatment was discontinued, and methotrexate was started (15 mg/weekly, subcutaneously) with topical adjuvant agents. A dramatic improvement was seen in both the skin and scalp with complete hair regrowth in 1 month. We conclude that, in anti-TNF-α-induced scalp psoriasis, suspension of anti-TNF-α agent and systemic and topical treatments should be considered to avoid scarring alopecia.

Gardner-Diamond Syndrome in an Adolescent With Suicidal Ideation: A Case Report.

Gardner-Diamond syndrome (GDS) is a rare disease often seen in young women involving painful localized inflammation and ecchymosis. Ecchymosis usually develops spontaneously after emotional stress. The pathophysiology of the disease is not fully understood, and little is known about management modalities for this syndrome. The primary approach of health professionals in the evaluation of this rare condition should involve identification of cases and investigation of potential accompanying psychiatric pathologies. The case presented here highlights the importance of assessing for GDS and reviews descriptions of GDS in the context of the existing literature.

Pediatric patients with psoriasis and psychiatric disorders: premorbidity and comorbidity in a case-control study.

BACKGROUND: Psychiatric disorders are thought to play an important role in the onset, exacerbation and course of several chronic dermatological diseases. We aimed to investigate psychiatric diagnoses in children with psoriasis before and during the disease and to examine potentially related factors. METHODS: A total of 108 children aged 8-16 years, 54 with a diagnosis of psoriasis and 54 healthy individuals, were included in the study. Participants were evaluated using The Kiddie Schedule for Affective Disorders and Schizophrenia, Present and Lifetime Version (K-SADS PL), Children's Dermatology Life Quality Index (CDLQI), Screen for Child Anxiety Related Emotional Disorders (SCARED) and Children's Depression Inventory (CDI), and the results were compared using statistical techniques. RESULTS: At least one psychiatric diagnosis was present in 70.3% of children with psoriasis and in 27.7% of the control group, the difference being significant (p = .0001). It was seen that 73.6% of children with a psychiatric diagnosis were psychiatric diagnoses in the premorbid period. Children with psoriasis were determined to have 9.21-fold greater risk of anxiety (p = .0001) and a 6.65-fold greater risk of depression (p = .0019) compared with the control group. CONCLUSIONS: A statistically significant increase in psychiatric disorders occurs in disease periods in cases of pediatric psoriasis. Moreover, a high prevalence of psychiatric disorders was detected in the premorbid process. We think that it is important for these to be considered in the management of the disease and in controlling exacerbation, and for the mechanisms involved to be elucidated.

Interactions between Posttraumatic Stress Disorder and Alopecia Areata in Child with Trauma Exposure: Two Case Reports.

Alopecia areata (AA) is a dermatologic disease that can be seen in all age groups with nonscarring hair loss. While the causes of AA are suggested to be the role of genetic, psychological stresses, cellular and humoral immunity, and endocrine and neural factors, the underlying cause is not fully known. Psychiatric diseases are frequently reported in many studies in patients with AA. In this report, children with AA and psychiatric evaluation of them and the prominence of psychiatric evaluation in AA were discussed; AA and posttraumatic stress disorder were reviewed in the light of the relevant literature.

Assessment of quality of life in Turkish children with psoriasis and their caregivers.

BACKGROUND/OBJECTIVES: The effect of pediatric psoriasis on quality of life has been demonstrated, but data regarding its influence on caregiver quality of life are scarce. The objective was to investigate how psoriasis affects quality of life of children and their caregivers. METHODS: This multicenter study included 129 children with psoriasis and their caregivers, who were family members accompanying patients to the clinic. Patient quality of life was measured using the Child Dermatology Life Quality Index. Caregiver quality of life was assessed using Dermatological Family Impact Scale, a 15-item questionnaire validated for use in the Turkish language. RESULTS: Mean Child Dermatology Life Quality Index score was 7.6, indicating a moderate effect on patient quality of life. Symptoms and feelings were the most severely impaired domains of patient quality of life, and emotions was the most severely impaired domain of caregiver quality of life. Dermatological Family Impact Scale score was significantly correlated with Child Dermatology Life Quality Index (correlation coefficient [r] = .554, P < .001) and Psoriasis Area and Severity Index (r = .350, P < .001). Caregivers of patients receiving systemic agents or phototherapy had relative impairment of multiple domains of quality of life compared to caregivers of patients receiving topical treatment only. CONCLUSION: Psychosocial effect of pediatric psoriasis was shown to extend beyond the individual, highlighting the importance of addressing patient and caregiver quality of life concerns in an integrated approach.

Vulvar syringomas as a part of nonfamilial generalized eruptive form: Unusual lesions leading to pseudohyper-trophy of the labia majora.

We present a case of eruptive generalized syringoma with vulvar involvement. This case is unique in terms of being a nonfamilial, linear variant, leading to pseudohypertrophy of the labia majora.

Is there a relation between clinical disease activity and acute phase response in Behcet's disease?

The aim of this study was to evaluate a possible relation between clinical disease activity determined by a scale validated and most widely used acute phase response parameters in patients with Behcet's Disease (BD). One hundred patients with BD were included in this study. The demographic properties including age, sex, and disease durations were recorded. BD Current Activity Form (BDCAF), which scores the history of clinical features that have been present during the four weeks prior to the day of assessment, was used for the evaluation of disease activity. Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels were evaluated as acute phase response. The independent samples t test and Pearson's correlation test were performed for the statistical analysis. There were 60 male and 40 female patients with an age range of 19-51 years. The disease durations of the patients were between 12 and 240 months. In the comparison of the laboratory parameters of the patients with or without the components of BDCAF, we found significantly higher levels of ESR and CRP in patients with erythema and arthralgia-arthritis versus without these involvements. There were significant positive correlations between ESR-CRP levels and patient's perception of disease activity (P = 0.012 and P < 0.05, respectively), clinician's overall perception of disease activity (P < 0.005), and total BDCAF score (P = 0.008 and P < 0.000, respectively). In conclusion, the presence of a newly-developed erythema nodosum, superficial thrombophlebitis, or joint involvement may be associated with higher levels of ESR and CRP. Also, correlations between these indices and general disease activity scores may contribute the overall disease activity perception in BD.