Acta Dermatovenerologica Alpina, Panonica et Adriatica

Primary cutaneous CD4-positive small or medium T-cell lymphoproliferative disorder (PCSM-LPD) is characterized by a slow-growing and asymptomatic solitary plaque or tumor, usually involving the head, neck, or upper extremities. The diagnosis is established based on clinical presentation, histopathological features including pleomorphic morphology and CD4-positive immunophenotype of neoplastic T lymphocytes, and molecular analysis showing clonally rearranged T-cell receptor (TCR) genes. Plaques typical of mycosis fungoides are essentially absent. Treatment options include surgical excision, radiotherapy, and topical or intralesional steroids. Because the disease is indolent, aggressive diagnostic tests and systemic treatments are not recommended. We present a case of PCSM-LPD in a previously healthy young man that spontaneously regressed after a biopsy.

Katarina Trčko

Nuša Lukinovič

Daja Šekoranja

Acta Dermatovenerologica Alpina Pannonica et Adriatica

Primary cutaneous CD4-positive small or medium T-cell lymphoproliferative disorder: a case report and literature review

原发性皮肤CD4阳性小或中型T细胞淋巴增殖性疾病的病例报告及文献综述

Angiosarcoma (AS) is a rare and aggressive soft tissue sarcoma originating from endothelial cells, with cutaneous manifestations often seen in the head and neck region. Despite its rarity, AS poses significant diagnostic challenges due to its variable presentation and ability to mimic other dermatological conditions. We report the case of an 87-year-old female that presented with a 4-month history of an asymptomatic nodule on her neck, which rapidly progressed into an indurated plaque spreading to her face, chest, and scalp. Initially misdiagnosed as cellulitis and dermatitis, the lesion was unresponsive to antibiotics and steroids. Imaging showed extensive infiltration in the neck, precluding surgical resection. This case underscores the diagnostic difficulty of AS, which can be mistaken for benign skin conditions. Despite a multidisciplinary approach, the prognosis for AS remains poor, with a 5-year survival rate of approximately 35%. Treatment options include surgery, radiation, chemotherapy, and immunotherapy tailored to the patient's condition and tumor characteristics.

Njoud AlNodali

Nouf Alqahtani

Aisha Sharafuddin

Rana Atef

Hanadi AlNodali

Mohammad Dhulaimi

Bander Alshomrani

Yazeed Alwagdani

Cutaneous angiosarcoma masquerading as photodermatitis: a case report

皮肤血管肉瘤伪装成光化性皮炎：一例报告

Co-occurrence of blisters in patients with lichen sclerosus (LS) can raise the question of whether they represent a bullous variant of LS or a concomitant autoimmune disorder. We report a rare case of bullous pemphigoid (BP) occurring on previous LS lesions. To the best of our knowledge, this is also the first BP180-negative case reported in literature. Here, we propose alternative mechanisms, independent of BP autoantibodies, that may lead to development of BP on skin affected by LS. In addition, we provide a literature review that explores the underlying pathophysiology and offers practical treatment insights, equipping clinicians with valuable guidance for similar complex cases.

Tijana Orlić

Igor Kapetanović

Vesna Reljić

Snežana Minić

Dubravka Živanović

A rare case and literature review of bullous pemphigoid appearing in the setting of lichen sclerosus: a dermatopathological conundrum and what to expect

一例罕见病例及文献综述：硬化性苔藓背景下出现的类天疱疮——皮肤病理学难题及预期结果

Dermoscopy is a highly practical noninvasive diagnostic tool. Several dermoscopic algorithms have been proposed in the evaluation of skin diseases, which allow clinicians not only to identify and make differential diagnosis, but also to determine the treatment choices in challenging clinical circumstances. Over the years, we have witnessed a rapid increase in the utilization of dermoscopy in the assessment of nail disorders. However, to assess the diagnostic utility of dermoscopy in inflammatory nail diseases, current evidence is insufficient. Nail psoriasis is a significant challenge because of the difficulties in its diagnosis. Detection of nail involvement is of utmost importance in psoriasis because it is highly associated with arthritis, which is an indication for systemic treatment. Dermoscopy holds promise as a potential tool in the diagnosis of nail psoriasis, capable of providing characteristic clinical findings without any delay and discomfort. This review summarizes current evidence regarding the unique dermoscopic features of nail psoriasis. It addresses whether dermoscopy may serve as the gold-standard diagnostic tool, excluding the necessity of histopathological examination for the ultimate diagnosis of nail psoriasis.

Ahu Yorulmaz

Dermoscopy: the ultimate tool for diagnosis of nail psoriasis? A review of the diagnostic utility of dermoscopy in nail psoriasis

皮肤镜：诊断甲银屑病的终极工具？甲银屑病皮肤镜诊断效用的综述

Darier disease is a rare autosomal dominant genodermatosis that initially first presents in adolescence with scaly reddish brown keratotic papules and plaques with a seborrheic and intertriginous distribution. The absence of specific targeted medications complicates the treatment process, and managing resistant cases can prove challenging due to recurrent exacerbations that may result in serious complications such as secondary bacterial and viral infections. Treatments of choice include antiseptics, topical corticosteroids, and systemic retinoids, mainly acitretin and isotretinoin. We report the case of a female patient with Darier disease that was unsuccessfully treated with acitretin and isotretinoin but showed significant improvement with alitretinoin. Previous reports on the efficacy of alitretinoin in Darier disease are reviewed.

Andreja Pagon

Mateja Dolenc Voljč

Efficacy of alitretinoin in the treatment of Darier disease: a case report

阿利维A酸治疗达里埃病的疗效：一例报告

Chronic spontaneous urticaria (CSU) is characterized by the occurrence of itchy wheals, angioedema, or both, without identifiable triggers, persisting for at least 6 weeks (1).It usually occurs in the form of relapses and remissions.The more severe the disease, the lower the likelihood of its remission (2-4).CSU may significantly impair the quality of life (5).Disease activity is measured with the urticaria activity score (UAS).Daily UAS values can range from 0 to 6 points, depending on the number of wheals (0 to 3) and the intensity of pruritus (0 to 3) (5, 6).Treatment is aimed at complete control of the signs and symptoms (1).The primary medications are second-generation H1-antihistamines (sgAH), which are taken regularly in a dose of one to four tablets per day.Omalizumab is commonly used to treat patients with sgAH-resistant CSU.It is an anti-IgE humanized monoclonal antibody that lowers the concentration of free IgE and downregulates high-affinity receptors for IgE (FcεRI) on mast cells and basophils, preventing their degranulation (7).Omalizumab treatment is symptomatic and has no effect on the natural course of CSU (8).We aimed to observe the probability of disease remission in patients with sgAH-resistant CSU and its duration after discontinuation of omalizumab.

Klara Cvenkel

Mojca Bizjak

Julij Šelb

Mitja Košnik

Remission of chronic urticaria in patients treated with omalizumab

奥马珠单抗治疗患者慢性荨麻疹的缓解情况

IntroductionEpidermolysis bullosa (EB) is a condition that occurs due to the absence of various components of the basal membrane secondary to inherited autosomal dominant or autosomal recessive genetic mutations.It is manifested by skin fragility and chronic wound formation.EB is divided into four main types: simplex EB, junctional EB, dystrophic EB, and Kindler EB, depending on the genetic mutations detected (1).This case report refers in particular to the dystrophic form of EB, which has been associated with mutations in the COL7A1 (3p21.31)gene, which encodes collagen type VII (2).Depending on the nature of these mutations, there can be reduced secretion or complete absence of collagen VII.The condition manifests through the formation of skin and mucosal blisters, which subsequently heal but leave behind dystrophic scars.These lesions primarily affect the limbs and may eventually lead to the development of pseudosyndactyly.Dystrophic epidermolysis bullosa causes high morbidity and mortality among patients.The lack of specific treatment and complications that occur secondary to repeated injuries coupled with impaired healing can lead to a decrease in the quality of life of patients.Even with the observance of rigorous hygiene and prevention rules, patients may experience specific complications of the disease as well as loss of mobility of the fingers and toes, anemia, recurrent infections, and malnutrition, and they have an increased risk of AbstractRecessive dystrophic epidermolysis bullosa (RDEB) is a rare genetic skin disease caused by mutations in the type VII collagen gene (COL7A1; 3p21.31).Mutations in this gene lead to an alteration in function or reduced amounts of collagen VII.This alteration of collagen VII leads to skin fragility and lesions at minor injuries with difficult healing.Cutaneous squamous cell carcinoma (cSCC) is more frequent in patients with RDEB than in the general population because of chronic wound formation; it constitutes a major cause of morbidity and is often cited as a cause of death for these patients.There is little experience with the treatment of cSCC in patients with RDEB.We report the case of a 19-year-old female patient with RDBE and inoperable locally advanced cSCC of the left arm.Because of the lack of therapy options, therapy with cemiplimab was started at a dose of 350 mg administered intravenously every 3 weeks.A confirmed clinical response was observed after the second cycle of treatment with no toxicity.During follow-up, the patient had a notable clinical response with no auto-immune adverse reactions.This shows that cemiplimab has a good safety profile for cSCC in patients with RDEB and is a valuable therapy option.

Daniel Ciurescu

Simina Condruz

Marius Irimie

Good clinical response to cemiplimab in a young patient with locally advanced cutaneous squamous cell carcinoma on preexisting recessive dystrophic epidermolysis bullosa

年轻局部晚期皮肤鳞状细胞癌伴先前隐性营养不良性大疱性表皮松解症患者对西米普利单抗的良好临床反应

短名	Acta. Dermatovenerol. Alp. Pannonica. Adriat.
Journal Impact	0.73
国际分区	DERMATOLOGY(Q4)

ISSN	1318-4458, 1581-2979
h-index	32

涉及主题	医学生物内科学病理皮肤病科遗传学免疫学外科化学生物化学疾病心理学放射科解剖
出版信息	出版商: Slovenian Medical Society，出版周期: ，期刊类型: journal，开源期刊: 否
基本数据	创刊年份: 1994，原创研究文献占比: 84.00%，自引率:0.00%， Gold OA占比: 0.00%

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Primary cutaneous CD4-positive small or medium T-cell lymphoproliferative disorder: a case report and literature review

Cutaneous angiosarcoma masquerading as photodermatitis: a case report

A rare case and literature review of bullous pemphigoid appearing in the setting of lichen sclerosus: a dermatopathological conundrum and what to expect

Dermoscopy: the ultimate tool for diagnosis of nail psoriasis? A review of the diagnostic utility of dermoscopy in nail psoriasis

Efficacy of alitretinoin in the treatment of Darier disease: a case report

Primary cutaneous CD4-positive small or medium T-cell lymphoproliferative disorder: a case report and literature review

Cutaneous angiosarcoma masquerading as photodermatitis: a case report

A rare case and literature review of bullous pemphigoid appearing in the setting of lichen sclerosus: a dermatopathological conundrum and what to expect

Remission of chronic urticaria in patients treated with omalizumab

Good clinical response to cemiplimab in a young patient with locally advanced cutaneous squamous cell carcinoma on preexisting recessive dystrophic epidermolysis bullosa