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.

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.

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.

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.

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.

Nail changes in systemic amyloidosis.

Nail examination must be an essential part of physical examination in our daily clinical practice, as nail changes may be the revealing sign of systemic diseases in the absence of other alarming signs.

Chronic macrocheilia: a clinico-etiological series of 47 cases.

BACKGROUND: Macrocheilia is an inflammatory disfiguring condition responsible for the swelling of the lips. This multi-etiological entity represents a diagnostic and therapeutic challenge. Published data on macrocheilia is scarce, often limited to granulomatous cheilitis. METHODS: We conducted a retrospective study, including all patients presenting with chronic macrocheilia (CM) for nineteen years. CM was defined as a persistent enlargement of one or both lips for at least eight weeks. Both descriptive and analytical analyses were performed. RESULTS: Of the 47 patients identified, 20 (43%) had cutaneous leishmaniasis, 10 (21%) had Miescher's cheilitis, five (11%) had Melkersson-Rosenthal syndrome, five (11%) had sarcoidosis, one (2%) had lepromatous leprosy, one (2%) had systemic amyloidosis, and one (2%) had Crohn's disease. In four cases, the CM was unlabeled. Ulcerations were significantly associated with leishmaniasis (P < 0.05). Histological study showed a granulomatous infiltrate in 72% of cases. Medical treatment was adapted to the etiology of CM. Surgery was performed in two cases. Improvement of CM secondary to leishmaniasis was seen in all cases. In patients with idiopathic orofacial granulomatosis, partial improvement was noted in four cases and a total improvement in one case. Recurrences were noted in three cases after complete regression. CONCLUSIONS: Macrocheilia is a rare and disfiguring condition that requires an etiological investigation, considering that it can reveal a serious underlying systemic disease. We identified several factors that could help recognize the cause of CM, including age, history of intermittent swelling, the extent of lip enlargement, the existence of ulceration, and systemic symptoms.

Intradermal tranexamic acid microinjections: a novel treatment option for erythematotelangiectatic rosacea.

BACKGROUND: Treatment options for erythematotelangiectatic rosacea (ETR) are still scarce. Tranexamic acid (TXA) is an antifibrinolytic drug that was recently used for the treatment of ETR. AIMS: To evaluate the efficacy and safety of intradermal microinjections of TXA for ETR. PATIENTS/METHODS: This was a retrospective study enrolling patients, treated with TXA intradermal microinjections for ETR, from January 2019 to February 2020. Response to treatment was assessed based on subjective symptoms, clinical photographs, and the Investigator Global Assessment of Rosacea Severity Score (IGA-RSS). RESULTS: Six patients were included. The mean number of monthly intradermal TXA microinjections was 5.1 ± 1.3. The mean decrease of IGA-RSS was 2.4 ± 0.5. Local side effects, mainly transient erythema and swelling, were noticed in three cases. No systemic effects were noted. Clinical improvement, in respondent patients, lasted after 3 months of follow-up. CONCLUSION: Intradermal TXA microinjections are a safe and effective treatment option for ETR. The optimal number of monthly sessions has yet to be determined.

Maintenance Phototherapy for the Treatment of Early-stage Mycosis Fungoides.

Mycosis fungoides (MF) is the most common type of cutaneous T cell lymphoma. Phototherapy is a first-line treatment option of early stages MF. The present study aimed at assessing the efficacy of phototherapy in Tunisian patients with MF treated with phototherapy and evaluate the efficacy of maintenance phase.

Multiple endocrine neoplasia type 1 revealed by a hip pathologic fracture.

Multiple endocrine neoplasia type 1 is a rare autosomal inherited syndrome that affects a variety of endocrine tissues such as the parathyroid, endocrine pancreas, and anterior pituitary. Osseous complications are often misdiagnosed. We presented a case of a 46-year-old woman with pathological fractures of the lower limb. She had a history of type 1 diabetes and galactorrhea. Laboratory examinations showed hypercalcemia and an increased level of parathyroid hormone related to hyperparathyroidism. Serum chromogranin A level was increased at 9369 ng/mL (N < 102). A somatostatin receptor scintigraphy (octreoscan) revealed pathological uptake in the gastric wall, later cave adenopathy, and liver. The diagnosis of multiple endocrine neoplasia type 1 was made based on radiological and histological findings. The patient underwent a subtotal parathyroidectomy associated with somatostatin analog treatment leading to significant improvement. A literature review was conducted by searching PubMed using these following terms: multiple endocrine neoplasia type 1, hyperparathyroidism, fracture, menin, osteoporosis. We emphasized bone involvement related to multiple endocrine neoplasia type 1 syndrome. This diagnosis should be considered when pathological fractures occur in young patients with a history of endocrine disorder. We highlighted the importance of imaging features in making the diagnosis of multiple endocrine neoplasia type 1. Early management of this disease is necessary. Treatment including parathyroidectomy and somatostatin analogs leads to bone preservation and functional improvement.

Cutaneous leiomyosarcoma mimicking arteriovenous malformation.

There is a clinical overlap between hypervascularized sarcomas and arteriovenous malformations (AVM). MRI imaging should be interpreted with caution keeping in mind that some cancers could mimic AVM. A biopsy is mandatory in doubtful cases.

Acquired epidermodysplasia verruciformis in renal-transplant recipients.

Acquired epidermodysplasia verruciformis in renal-transplant recipients is associated with a high risk for developing squamous cell carcinoma. An accurate diagnosis and a regular monitoring in this high-risk population must be stressed.