Pharmacological Management of Synovitis, Acne, Pustulosis, Hyperostosis, and Osteitis Syndrome Syndrome: A Proposal of a Treatment Algorithm.

ABSTRACT: Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome is a rare chronic disease with marked clinical and radiological heterogeneity. It is characterized by a combination of dermatological and osteoarticular manifestations. The treatment of SAPHO syndrome is not yet codified. It includes several therapeutic options such as anti-inflammatory drugs, bisphosphonates, antibiotics, conventional disease-modifying antirheumatic drugs, and biological treatment.This article aims to provide an updated review of the different pharmacological options for SAPHO syndrome. We also propose a therapeutic algorithm for the management of this disease.

Effectiveness of dermoscopy in the demarcation of surgical margins in slow Mohs surgery.

There is a need for adjuvant imaging techniques that would allow reducing the number of slow Mohs stages. This study aimed to evaluate the use of dermoscopy in the demarcation of basal cell carcinoma (BCC) surgical margins for slow Mohs surgery. This was a retrospective study over 3 years (2016-2019), including patients with BCC excised using slow Mohs surgery. On the basis of the treatment received, the patients were divided into 2 groups: group 1 (28 BCC) and group 2 (26 BCC). In group 2, BCC margins were demarcated using dermoscopy. A total of 54 patients were enrolled in the study. The number of positive lateral margins was significantly lower in the group where BCC margins were demarcated using dermoscopy (19% vs 53%, P = .012). In this group, the number of Mohs stages needed to achieve complete clearance was significantly lower. However, the mean interval between the first Mohs excision and Mohs clearance was not significantly different between the 2 groups (9 ± 4 vs 12 ± 7 days). In conclusion, preoperative dermoscopy is useful for reducing the number of positive lateral margins and the number of slow Mohs stages in treating BCC especially pigmented tumors.

Rickets in association with skin diseases and conditions: A review with emphasis on screening and prevention.

BACKGROUND: Rickets is a common disease worldwide. In the developed world, its prevalence dramatically decreased but still diagnosed in at-risk populations. The skin plays a critical role in vitamin D synthesis. Therefore, several skin diseases, especially keratinization disorders, could lead to impaired vitamin D metabolism and vitamin D deficient rickets. OBJECTIVE: The article aimed to summarize the current knowledge of skin diseases and conditions associated with rickets. METHODS: To examine the association between rickets and skin diseases, we performed a systematic review of the literature using PubMed database. The search included studies published from the database inception to August 2019. RESULTS: A total number of 75 articles were included. Identified conditions associated with rickets were ichthyosis being a more common skin diseases, alopecia, epidermal and melanocytic nevi, xeroderma pigmentosum, mastocytosis, psoriasis, and atopic dermatitis. Three types of rickets were identified: vitamin D-dependent rickets, hypocalcemic vitamin D-dependent rickets type 2, and hypophosphatemic rickets. Cutaneous skeletal hypophosphatemia syndrome is a newly described and under-recognized condition. It is defined by the association of epidermal or melanocytic nevi, hypophosphatemic rickets, and elevated levels of fibroblast growth factor 23. Rickets in patients with ichthyosis was mainly due to impaired ability of ichthyotic skin to synthesize vitamin D, poor UV penetration of the skin caused by keratinocyte proliferation, and dark phototype. The latter may be considered a risk factor for rickets in patients with ichthyosis. CONCLUSION: Despite its rarity, these associations should be properly recognized by dermatologists. Early diagnosis of rickets is important to prevent growth retardation and skeletal deformities.

A Challenging Cutaneous T-Cell Lymphoma.

Cutaneous peripheral T-cell lymphomas not otherwise specified (CPTL-NOS) are rare neoplasms accounting for just 2% of cutaneous peripheral T-cell lymphomas (CPTL). Only very few case series have been reported. They represent a phenotypically and prognostically heterogenous group of CPTL that do not fit into any of CPTL well-defined subtypes. The authors report a case of a 64-year-old man with simultaneous plaque-like lesions and disseminated nodules growing rapidly on the face, trunk, and extremities over a 6-month period. There was no a history of preceding patches, erythematous plaques, rash, or pruritic lesions. These lesions were extending over 80% of the skin surface. Histopathologic analysis revealed dense diffuse infiltrates composed of mostly medium-sized to large lymphoid cells throughout the entire dermis without epidermotropism. Neoplastic cells were atypical with markedly pleomorphic nuclei. Immunohistochemistry showed that the tumor cells were positive for CD3, CD4, and CD5 with a loss of CD7. They were negative for CD20, CD8, CD56, CXCL13, PD1, TIA-1, granzyme-B, perforin, CD25, and CD30. The proliferative fraction was low, with MIB-1 labeling less than 10% of cells. The authors diagnosed the patient with primary CPTL-NOS. Despite the rarity of these tumors, clinicians as well as dermatopathologists and pathologists should be familiar with these rare CPTL especially because most of these lymphomas have an aggressive behavior and exhibit an unfavorable prognosis.

Significant alleviation of Darier's disease with fractional CO2 laser.

Darier's disease (DD) is a dominantly inherited genodermatosis with highly variable expression. It is characterized by symmetrical hyperkeratotic papules affecting seborrheic areas and extremities. The existence of unsightly lesions could lead to discomfort and social handicap. Conventional treatment consists of topical and systemic steroids and/or retinoids alleviating DD. Ablative lasers also have been used to treat these conditions with variable results and side effects. To the best of our knowledge, fractional CO2 laser has never been used to treat DD. We present a case of a 36-year-old woman with verrucous and hyperkeratotic plaques of the forehead significantly improved after two sessions of fractional CO2 laser treatment. Neither scars nor pigmentary disorders were noted.

Nail Alterations From Musical Instruments: Insights for Dermatologists Treating Musicians.

Long-term practice and performance with a musical instrument predispose musicians to several skin conditions and nail disorders. We conducted a comprehensive literature search of the PubMed, Scopus, and Google Scholar databases for articles on nail alterations in musicians. Complications were divided into modifications of the nail surface and nail plate, soft-tissue abnormalities, and periungual tissue and distal pulp disorders. Health care professionals should be aware of these various modifications related to the use of musical instruments and provide preventive measures.

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.

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.

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.

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.