Acta dermatovenerologica Croatica : ADC最新文献

Juvenile dermatomyositis with emphasized vasculopathy is rare, but the most severe form of the disease, with a poor prognosis with relapsing and chronic course or, in some cases, lethal outcome. We present a case of a 19-year-old Caucasian female, who developed severe acute juvenile dermatomyositis with emphasized multisystem vasculopathy, including retinal vasculopathy and maculopathy (cotton-wool spots, retinal hemorrhages, macular edema) at the age of 8. Due to no response to standard treatment protocols and rapid worsening of clinical symptoms and laboratory findings, a TNF inhibitor (infliximab) was introduced after the third week of treatment resulting in complete normalisation of muscle enzyme levels and complete resolution of eye changes within the next 2 weeks with a gradual general recovery. To the best of our knowledge, this is the first long-term follow-up of an early TNF inhibitor introduction in a patient with acute, severe form of juvenile dermatomyositis and retinal vasculopathy. After 12 years of infliximab therapy, the outcome was excellent, with no side effects throughout the whole treatment.

Introduction: Aging is a continuous and irreversible process which affects the skin. During the aging process, intrinsic progressive degenerative changes in the skin impair its structure, which makes it prone to different dermatoses, resulting in impaired quality of life in the elderly.

Objective: Carboxytherapy is considered as a safe, minimally invasive modality applied for skin rejuvenation, restoration, and recondition. Herein, we have assessed the efficacy of carboxytherapy in the treatment of skin aging through clinical and sonographic studies.

Materials and methods: Our study was a prospective 2-split clinical trial in which the efficacy of carboxytherapy was assessed in the treatment of abdominal skin aging through clinical and sonographic evaluations.

Results: Twenty-eight patients with skin-intrinsic aging manifestations in the abdomen completed the study. Their mean age was 44.13 years. The mean weight, BMI, and waist circumference of the subjects significantly decreased after the treatment. The clinical subjective and objective evaluations revealed statistically significant improvement of skin wrinkles, laxity, and skin pigmentation and overall satisfaction by carboxytherapy. Upon sonographic investigation, a significant increase in epidermis and dermis thickness was observed. No significant side-effect was reported by the subjects.

We report the case of an 18-month-old boy who developed a phototoxic skin reaction to terbinafine on his scalp, ears, and face in the form of disseminated erythematous plaques, which resembled subacute lupus erythematosus (SCLE) in their clinical presentation. Skin changes appeared a short time after the boy was exposed to sunlight during the period of time when he was treated with oral terbinafine due to Microsporum canis fungal scalp infection. Tinea capitis is a common dermatophyte infection primarily affecting prepubertal children (1). Microsporum canis remains the predominant causative organism in many countries of the Mediterranean basin, the most important dermatophyte carriers being stray cats and dogs. Systemic therapy is required for treatment because topical antifungal agents do not penetrate down to the deepest part of the hair follicle (2). Terbinafine is commonly used in the treatment of microsporosis, as its fungicidal action permits short periods of treatment (3,4). The first skin changes occurred in the parietal scalp region in the form of round scaly alopecia, with the presence of unevenly broken hairs and enlarged regional lymph nodes (Figure 1). Diagnosis of fungal infection included clinical assessment and Wood's light examination, which revealed green-yellow fluorescence on the lesional scalp region. Fungal culture identification was performed according to conventional methods, revealing fungal culture positive for dermatophytes from the genus Microsporum canis. The boy had a history of contact with a cat. Systemic therapy with the oral antifungal drug terbinafine was administered at a dose of 62.5 mg per day (5 mg/kg), with topical application of antifungal cream (miconazole), 10% Ichthyol cream in the evening, and antifungal shampoo (ketoconazole) twice a week. After two weeks of therapy, we observed initial regression of scalp lesions. Oral terbinafine was well-tolerated, and the patient did not experience any side-effects. Laboratory findings included liver function tests and were within normal ranges. At this point, the oral dose of terbinafine was increased to 125 mg per day (10 mg/kg) at a revised schedule according to body weight: 10-25 kg, 125 mg/day (5). Approximately five weeks after starting the treatment with oral terbinafine, after the boy was exposed to the sun, acute disseminated erythematosus lesions appeared on the face and scalp. Clinical presentation of the lesions and acute onset during exposure to sunlight raised the suspicion of a phototoxic reaction to terbinafine (Figure 2). The patient was not taking any other medication at that time, had no history of drug or food allergies, and had not previously experienced photosensitive skin reactions. Due to the inflamed skin changes resembling subacute lupus and photosensitivity, an immunological assay tests were also performed. Due to the young age of the patient, no skin biopsy or photo-patch test was performed. Despite the recent skin changes and sus

Schnitzler syndrome (SS) is an extremely rare acquired systemic disease that shares many similarities with various hereditary autoinflammatory syndromes. It presents as chronic non-pruritic urticarial rash, monoclonal gammopathy, and systemic symptoms, such as recurrent fever, arthralgia, myalgia, bone pain, bone lesions, and enlargement of the spleen and liver. The specific feature associated with SS is its spectacular response to treatment using anti-interleukin-1 (anti-IL-1) agents, such as anakinra or canakinumab. If it remains untreated, the disease can have a devastating effect on the patient's quality of life as well as increased mortality due to systemic complications. Herein, we will summarize the most recent findings in the pathogenesis, diagnosis, and management of SS.

Autosomal recessive congenital ichthyosis (ARCI) comprises a group of rare, clinically heterogeneous disorders of keratinization, characterized by hyperkeratosis, abnormal skin scaling, and a variable degree of erythroderma. Affected infants are most often born encased in a collodion membrane, which is usually shed within 2-4 weeks, revealing the underlying skin condition. To date, at least 14 genes have been identified as causative for ARCI, and phenotypes associated with mutation of different genes may overlap. Herein we report the case of an infant with ARCI due to heterozygous pathogenic mutations in the 12(R)-lipoxygenase (ALOX12B) gene.

Eccrine angiomatous hamartoma (EAH) is a rare benign skin neoplasm characterized by an increased size and number of eccrine glands or ducts, along with proliferation of vascular structures in the dermis. This case is unique in its presentation of bilateral symmetrical nodules on both hands and the development of new nodules during puberty. It highlights the need for further research and understanding of this rare condition and its potential progression over time.

Background: An extensive body of literature has been published regarding alopecia areata (AA) in the past 50 years. The current paper used a bibliometric analysis (BA) to identify high-quality research articles using criteria such as annual citations (ACs) and journal impact factor.

Objectives: To identify and analyze the top 100 most cited articles in AA scientific literature over the past 50 years using BA methods.

Methods: Web of Science (webOS) citation indexing database was used, on April 4th, 2023, to identify the most cited articles on AA. Articles were ranked by their ACs. Data sets were then subdivided into corresponding and senior authors, year of publication, journal and impact factor, total citations according to webOS database, ACs, affiliation, country of origin, manuscript type, design, focus, and usage count since 2013.

Results: The extracted articles were published between 1975-2019. Mean total citations ranged between 67 and 578. The most cited paper was: "Tofacitinib for the treatment of severe alopecia areata and variants: A study of 90 patients" by Liu et al. with an AC of 26.5. Most publications were published between 1990 and 1999 (n=28). The Journal of the American Academy of Dermatology was the most published journal (25 articles). The research focus of original papers was treatment (36%), epidemiology (22%), pathogenesis (20%), basic science (16%), and diagnosis (6%).

Conclusion: This analysis is the first to provide detailed bibliometric characteristics, highlighting the worldwide burden and research trends in.

Plasma cell mucositis (PCM) is an unusual disorder most evident in the accessible mucosa and usually reported in the upper aerodigestive tract, although it is named according to its specific anatomical site of involvement such as plasma cell cheilitis, plasma cell gingivitis, plasma cell vulvitis, and Zoon's balanitis. PCM reflects a dense polyclonal rather than a monoclonal plasma cell proliferation of unclear and unknown etiology. This perplexing disorder tends to be treated by avoiding possible triggers and intralesional and/or systemic steroids. In this work, we provide a review and update on PCM, which often represents a clinical conundrum.