Cutaneous Rosai-Dorfman disease: a systematic review and reappraisal of its treatment and prognosis.

Cutaneous Rosai Dorfman disease (CRDD) is a rare histiocytic disorder that shows distinctive clinical presentation and prognosis. Sufficient data is currently lacking regarding evidence-based management of CRDD. This systematic review aims to provide a comprehensive overview of CRDD, focusing on treatment approaches and outcomes. PubMed and Scopus databases were searched for studies on CRDD from June 1st, 2013 to May 31st, 2023. Articles describing cases of CRDD confirmed with histological examination were eligible for inclusion. All interventions for CRDD were analyzed. The primary outcome measure was the response of cutaneous lesions to treatment including complete response (CR), partial response (PR), and no response. The secondary outcome measures were mortality rate, relapse rate, and the occurrence of adverse events related to CRDD treatment. Eighty-seven articles describing 118 CRDD cases were included. The mean age was 48.2±16.8 years. The sex ratio (F/M) was 1.53. Nodular (46.6%) erythematous (45.3%) lesions, located on the face (38.1%) were the most prevalent presentations. Associated hematological malignancies were noted in 8 (6.8%) cases. Surgical excision was the most prevalent intervention (51 cases) with CR in 48 cases. Systemic corticosteroids were used in 32 cases with 20 CR/PR, retinoids in 10 cases with 4 CR/PR, thalidomide in 9 cases with 5 CR/PR, methotrexate in 8 cases with 7 CR/PR while observation was decided in 10 cases with 6 CR/PR. Factors independently associated with the absence of response to treatment were facial involvement (OR = 0.76, p = 0.014), and cutaneous lesion size (OR = 1.016, p = 0.03). This systematic review shows distinctive clinical characteristics of CRDD and provides insights into the appropriate management of the disease. It allowed a proposal of a treatment algorithm that should be interpreted in the context of current evidence and would help practitioners in treating this rare disease.

Direct medical cost related to the management of pemphigus: A pilot Tunisian study.

INTRODUCTION: Pemphigus is a therapeutically challenging disease with high morbidity and economic burden. First-line prescription of rituximab remains limited in Tunisia due to its high cost. Systemic steroids remain the standard of care but are associated with a major risk of morbidities and higher treatment costs. AIM: To assess the direct medical costs of pemphigus in Tunisia. METHODS: Retrospective estimation of direct medical costs during the 18 months following the diagnosis using the "bottom-up approach" in the Dermatology Department of Hedi Chaker Hospital, Sfax, Tunisia. RESULTS: Total medical costs were estimated at 38745.7 €, with an average cost of 1 210 € per patient and per year: paraclinical investigations (46%), medical treatment (30%), hospitalization (21%) and outpatient visits (3%). The average cost was the highest in the age group of 15-24 years (1553 €). Treatment costs related to corticosteroid-induced morbidity were estimated at 1208 €. CONCLUSIONS: The management of pemphigus in Tunisia needs to be adapted to take into account the health economic analysis in order to reduce overall disease costs and the burden of steroid-induced morbidities.

Becker Nevus Syndrome: A Rare Entity but Important to Recognize.

Becker's nevus syndrome is defined by the association of a Becker nevus with homolateral breast hypoplasia or more rarely skeletal cutaneous or muscle deformities. Early diagnosis is important, especially in female patients to prevent and treat breast hypoplasia. We report two cases of Becker nevus syndrome with serious functional impairment and discuss possible treatment options.

Systemic lupus erythematosus presenting as lupus erythematosus tumidus and lupus nephritis: a case report.

BACKGROUND: Lupus nephritis and lupus erythematosus tumidus (LET) are uncommon manifestations of systemic lupus erythematosus (SLE), and their coexistence as the initial presentation of SLE is exceedingly rare. Here, we report such a case, emphasizing the diagnostic challenges and therapeutic implications of this unusual association. CASE REPORT: A 38-year-old North African woman presented in Nephrology department with a history of lower extremity edema, fatigue, and weight loss of 3 kg in 4 weeks. Physical examination revealed LET lesions on the chest and the Neck. Laboratory investigations showed lymphopenia, low C3 and C4 complement levels, positive antinuclear antibodies, anti-dsDNA antibodies, and anti-SSA/Ro antibodies. Renal function tests showed normal serum creatinine and nephrotic proteinuria. Renal biopsy revealed Class V lupus nephritis. Skin biopsy confirmed the diagnosis of LET, with the presence of lymphohistiocytic infiltrates and dermal mucin. The patient was diagnosed with SLE based on the 2019 EULAR/ACR criteria and treated with prednisone (1 mg/kg/day) and hydroxychloroquine. She showed significant improvement in her cutaneous and renal symptoms at 6 and 12 months follow-up. CONCLUSION: The rarity of the coexistence of LET and lupus nephritis as the initial manifestation of SLE, especially in the North African population, underscores the need for further research to elucidate the immunopathogenic mechanisms and prognostic factors associated with this association.

Dermoscopy of Onychomycosis: A Systematic Review.

INTRODUCTION: Onychomycosis represents a global burden accounting for about 50% of nail consultations. Several studies have tried to assess the dermoscopic features of onychomycosis. With the multiplication of papers, several "new" dermoscopic signs keep being added leading to some inconsistency in onychoscopic terminology. OBJECTIVE: This study aimed to summarize the existing literature on the dermoscopic features of onychomycosis and propose a unified onychoscopic terminology. METHODS: The literature search was performed using PubMed and Scopus databases up to October 30, 2021 to identify eligible contributions. In total, 33 records (2111 patients) were included. RESULTS: The main dermoscopic signs of onychomycosis are "ruin appearance", "longitudinal striae" and "spikes" on the proximal margin of onycholytic areas, with a specificity of 99.38%, 83.78%, and 85.64% respectively. The "aurora borealis" sign had the highest sensitivity and specificity. CONCLUSIONS: The current review provides a framework for issues related to the onychoscopic terminology of onychomycosis and is intended to serve as an aid for students, teachers, and researchers. We proposed a unifying terminology to describe dermoscopic signs of onychomycosis. Dermoscopic signs of onychomycosis show good specificity and are useful in distinguishing nail psoriasis, trauma, and onychomycosis. It helps differentiate fungal melanonychia from nail melanoma, nevi, and melanocytic activation.

Patients with vitiligo have a distinct affective temperament profile: A cross-sectional study using Temperament Evaluation of Memphis, Paris, and San Diego Auto-Questionnaire.

Background: Vitiligo is a skin disease associated with significant psychiatric comorbidities. Affective temperaments represent the inherited foundation of personality and represent the biologically stable part of emotional reactivity. Studies assessing the temperament profile of patients with vitiligo are still scarce. Method: This was a cross-sectional study enrolling 34 patients with vitiligo and 34 age and sex-matched healthy controls. Affective temperament profile was investigated using the Temperament Evaluation of Memphis, Paris, and San Diego Auto-questionnaire. Dermatology life quality index was used to evaluate disease impact on patients' quality of life. Results: We found significant differences with vitiligo patients scoring higher in anxious (11.5 ± 4.76 vs. 9.06 ± 6.22; p = 0.036) and lower in hyperthymic (5.79 ± 3.82 vs. 7.5 ± 3.64; p = 0.027) temperaments. Vitiligo worsening reported by patients was associated with recent psychological stressors in 18 cases (52.9%) and Koebner phenomenon in 20 cases (58.8%). Koebner phenomenon was more frequently associated with the hyperthymic temperament (p = 0.035). Cyclothymic temperament was positively correlated with Dermatology life quality index (rho = 0.417, p = 0.014). Conclusions: This study demonstrated that patients with vitiligo have a distinct premorbid temperament profile. Having low hyperthymic and high anxious temperament traits seem to predispose patients to be less resilient to psychological stressors. A better understanding of the affective traits of vitiligo patients would be vital in personalising and adapting the management of this chronic skin disease.

Matrix Metalloproteinases; A Biomarker of Disease Activity and Prognosis in Spondyloarthritis: A Narrative Review.

BACKGROUND: Matrix metalloproteinases, as components of the proteolytic system, are deemed to be implicated in the pathogenesis and progression of several rheumatic diseases. Their role in spondyloarthritis has been investigated by several studies. OBJECTIVE: This article aims to review and summarize the current knowledge related to metalloproteinases in patients with spondyloarthritis. METHODS: To examine the association between matrix metalloproteinases and spondyloarthritis, we conducted a narrative review using a literature search in SCOPUS for English-language sources. The search included studies published from the database inception to December 2020. RESULTS: A total number of 74 articles were included. It was found that levels of matrix metalloproteinases 3 were higher in radiographic axial spondyloarthritis patients and seemed to play a role in the progression of joint damage. The levels of matrix metalloproteinases 1, 2, and 9 were upregulated in psoriatic arthritis patients compared to psoriasis and could identify psoriasis patients who would develop rheumatic manifestations. The levels of matrix metalloproteinases correlated significantly with disease activity in ankylosing spondylitis and decreased upon treatment with Tumor Necrosis Factor inhibitors (TNFi). CONCLUSION: Excessive matrix metalloproteinases activity is associated with articular destruction. Their levels can reflect disease activity, structural damage, and response to TNFi in patients with spondyloarthritis. Nevertheless, further studies are needed to confirm these results.

The Use of Dermoscopy in the Delineation of Basal Cell Carcinoma for Mohs Micrographic Surgery: a Systematic Review With Meta-Analysis.

Introduction: Several studies investigated the use of dermoscopy in the delineation of basal cell carcinoma (BCC) for Mohs micrographic surgery (MMS) with conflicting results. Objectives: The purpose of this systematic review with meta-analysis was to evaluate the effectiveness of the use of dermoscopy-guided MMS in the treatment of BCC. Methods: We included all comparative studies. Cases of BCC treated using dermoscopy-guided MMS (or slow MMS) were compared to those treated with curettage-guided MMS or "standard" MMS. Results: A total of 6 studies including 508 BCCs were reviewed. There was no statistically significant difference in the proportion of total margin clearance on the first MMS stage between BCCs removed using dermoscopy-guided MMS and those that had curettage or visual inspection. However, lateral margin involvement was significantly lower in BCCs that had dermoscopy-guided MMS. Conclusions: Dermoscopy allows visualization of structures up to 1mm into the dermis. Therefore, it is rational to use it for lateral margin evaluation. Currently, there are two comparative studies showing the efficacy of dermoscopy for lateral margin evaluation during MMS. Future studies are required to develop an evidence-based recommendation regarding the utility of dermoscopy in MMS.

Cutaneous and rheumatological manifestations of reactive arthritis: A case report.

Reactive arthritis is a rare form of spondyloarthropathies occurring after genital or enteric infection. It is most often self-limited but can progress to chronic spondylarthritis. We report the case of a 30-year-old man who presented with acute arthritis occurring 2 months after an episode of urethral discharge. Physical examination revealed polyarthritis, dactylitis, sacroiliac joint involvement, and plantar papulosquamous plaques. The human leukocyte antigen B27 was positive. Detection of Chlamydia trachomatis and Gonococcus in the first catch urine specimen was negative. Hepatitis B and C, Chlamydia trachomatis, human immunodeficiency virus, and syphilis serologic test results were negative. Pelvic magnetic resonance imaging revealed left sacroiliitis. The patient was treated with antibiotics, diclofenac, and sulfasalazine. After 6 months of follow-up, a significant clinical improvement was obtained without remission, suggesting an evolution to chronic spondylarthritis. Diagnosis of Reactive arthritis is difficult since microbiologic examinations are commonly negative. This disease should be considered in patients with rheumatologic manifestations occurring after a urogenital or enteric infection, mainly when associated with skin manifestations and human leukocyte antigen B27.

Multiple keratoacanthomas of Ferguson-Smith type.

We report a case of a 41-year-old male patient with no family history, presented with extensive multiple keratoacanthomas with disfiguring scars. The diagnosis of a sporadic form of Ferguson-Smith syndrome was made. Treatment with acitretin showed a marked response. Recognizing this syndrome is crucial. Early treatment helps avoid scar formation.

Omphalolith: An underdiagnosed entity.

Omphalolith is a rare and underdiagnosed entity due to the accumulation of sebum and keratin in the umbilicus. It usually occurs in the elderly with deep and narrow umbilicus. Early recognition of omphalolith is important to prevent complications, unnecessary investigations, and anxiety. We report herein two new cases of omphalolith.

Assessing the validity and interpretability of the Simplified Psoriasis Index in Tunisian patients.

INTRODUCTION: Multiple scores have been developed to assess the severity of psoriasis, but these scores have many limitations. The Simplified Psoriasis Index (SPI) is a summary score with separate components for current severity (SPI-s), psychosocial impact (SPI-p), and past history and interventions (SPI-i). It is available in two similar versions: proSPI and saSPI. AIM: To assess the validity of the SPI by studying its correlation to the benchmark scores in Tunisian patients. METHODS: It was a prospective bicentric study including 80 patients with plaque psoriasis. RESULTS: The median PASI was 7.6 and the median DLQI was 9. The median proSPI-s was 6 and the median saSPI-s was 8. The median SPI-p was 7. The median SPI-i was 2. There was a strong correlation between the proSPI-s and PASI (r=0.87) and between the proSPI-s and saSPI-s (r=0.82). There was a medium correlation between saSPI-s and PASI (r=0.70) and between SPI-p and DLQI (r=0.67). The threshold value for proSPI-s and saSPI-s was 7.25. The threshold value for SPI-p was 6.5. CONCLUSION: The SPI aims to provide a concise but global measure of the severity and impact of psoriasis on quality of life. The use of SPI has several advantages: the simplicity of use, the additional weight given to critical locations of psoriasis, the possibility for the patient to self-assess his own disease, and the possibility of evaluating all the dimensions of psoriasis at the same time.

Melanotic lupus erythematosus: A review of a newly described clinical form of chronic cutaneous lupus erythematosus.

Melanotic lupus erythematosus (LE) is a rare and newly described form of chronic cutaneous LE. In this review, we have synthesized existing data on the epidemiologic, clinical, histologic, and immunologic features of melanotic LE. We performed a systematic review using PubMed to identify eligible publications. Eight contributions fulfilled the eligibility criteria and were included in the qualitative synthesis. Twenty-eight patients with a mean age of 57.7 years were included. All patients had medium to dark skin phototypes. The lesions presented either as solitary and localized, poorly limited, round, or oval patches (50%) or as a more diffuse or generalized, sometimes reticulated, hyperpigmentation (50%). Patients diagnosed with systemic LE accounted for 14% of included cases. Antinuclear antibodies were positive in 10.7% of cases. Hydroxychloroquine and topical steroids were the most commonly used treatments. Complete resolution of the lesions was noted in 27.27% of cases. No patient experienced atrophy, destruction, or deformity of the skin. Melanotic LE appears to affect mainly patients with dark phototypes. It is characterized by late age at the onset of disease and an overall good prognosis.

Side effects of gel nail polish: A systematic review.

Gel nail polish (GNP) has recently gained worldwide popularity. We have conducted a comprehensive summary of the complications of GNP through a literature search using the PubMed, Scopus, and Google Scholar databases to identify eligible contributions. Complications were divided into mechanical and traumatic nail disorders, allergic contact dermatitis (ACD), and ultraviolet (UV)-induced lesions. A total of 12 contributions were included, identifying 88 patients, all of whom were women. Six of the reports described ACD (62 cases, 70.5%), 3 concerned mechanical nail damage (23 cases, 26.1%), and 3 reported UV-induced skin lesions (3 cases, 3.4%). ACD developed an average of 30 months after GNP initiation. The most frequent culprit allergens were 2-hydroxypropyl methacrylate and 2-hydroxyethyl methacrylate. Pterygium inversum unguis was the most frequent mechanical lesion (n = 17). Squamous cell carcinoma was reported in 3 cases. The delay between UV exposure and the diagnosis of squamous cell carcinoma ranged from 11 to 15 years. Scant literature and a lack of education among consumers and beauticians have led to the uncontrolled use of GNP. The principle of managing nail cosmetic problems is prevention through education. There is a need for understanding the processes involved and the associated complications to facilitate appropriate treatment and safe use.

Cutis verticis gyrata: Three cases illustrating three different etiologies.

Cutis Vertcis gyrata is an uncommon neurocutaneous syndrome characterized by excessive growth of the skin of the scalp or the face, forming folds of similar aspect to cerebral cortex gyri. Three categories have been individualized: the primary form, essential or non-essential, and the secondary form.