Acta Dermatovenerologica Alpina Pannonica et Adriatica最新文献

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.

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.

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.

Psoriasis is a common chronic inflammatory skin disorder that primarily affects the skin, nails, and joints. Beyond its cutaneous manifestations, psoriasis is associated with several systemic comorbidities. Various factors can trigger or exacerbate psoriasis, including stress, infections, medications, and vaccinations. This article reports what is, to the best of the author's knowledge, the first known case of acute exacerbation of plaque-type psoriasis, presenting as guttate psoriasis (GP), following herpes zoster vaccination. A 52-year-old male with a history of longstanding plaque-type psoriasis developed a sudden flare of GP lesions 2 weeks after receiving the recombinant herpes zoster vaccine. Physicians should be vigilant for potential triggers of psoriasis exacerbation, with the recombinant herpes zoster vaccine being among them.

Introduction: Idiopathic guttate hypomelanosis (IGH) is a common leukodermic dermatosis that primarily affects middle-aged and elderly adults. This study evaluates the dermoscopic features of IGH and their correlation with histopathological findings.

Methods: In the present study, 100 patients with IGH were evaluated. Each patient underwent a comprehensive clinical history assessment along with a dermatological and physical examination. Dermoscopic examination was performed, followed by a histopathological examination to confirm the diagnosis.

Results: The mean age of the participants was 64.67 ± 9.59 years, with 53% being male. The most prevalent dermoscopic pattern observed was nebuloid (33.3%), followed by petaloid (26.7%), and both amoeboid and feathery patterns were seen in equal proportions (20% each). The abdomen (33%) and legs (27%) were the most common sites for IGH lesions. Histopathological examination revealed features such as basket weave hyperkeratosis, atrophic epidermis in some lesions, reduced melanin globules or melanocytes, skip lesions, and flattening of rete ridges across all dermoscopic patterns.

Conclusion: IGH is characterized by distinct dermoscopic patterns, including amoeboid, feathery, nebuloid, and petaloid types. When these patterns are interpreted within the clinical context and corroborated with histopathological findings, they aid in the accurate diagnosis of IGH and its differentiation from other hypopigmented and depigmented dermatoses. Dermoscopy can be considered an adjunctive tool to confirm the diagnosis of IGH.

Actinic lichen planus (ALP) is a rare photosensitive subtype of lichen planus (LP) with four major forms recognized: annular, pigmented (melasma-like), dyschromic, and classic lichenoid. The prevalence is highest among dark-skinned younger females residing in tropical and subtropical regions. There are very few reports of ALP across Europe, with most of the cases among individuals living in warm countries or in people of Middle Eastern and Indian ancestry. We report a case of a 68-year-old white man that presented with a 9-year history of a mildly pruritic solitary hyperpigmented patch on the tip of his nose. Histopathological examination demonstrated signs of classic LP with epidermal atrophy, pigmentary incontinence, and signs of solar elastosis. Based on these findings, a diagnosis of pigmented ALP was established. Topical pimecrolimus and tretinoin along with rigorous photoprotection proved effective, with mild residual hyperpigmentation after 6 months of treatment. Many differential diagnostic possibilities should be considered for such a lesion. Nevertheless, a biopsy and correlation of histopathological and clinical findings can shorten the time from onset to a proper diagnosis. Treating both the hyperpigmented and inflammatory component of this dermatosis is necessary, as well as strict long-term photoprotection to prevent recurrences.

Recessive 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.

Introduction: Cellulite, also known as edematous fibrosclerotic panniculopathy (EFP), affects up to 90% of women and has a significant aesthetic impact. EFP is a multifactorial condition characterized by local circulatory changes, increased adipose tissue thickness, and a fibrotic response involving thick collagen bundles and septa, driven by local hypoxia. Although numerous treatments exist, their effects are typically temporary. This study evaluates the outcomes of four patients with EFP treated using a combined recombinant enzymatic therapy consisting of a lyase, lipase, and collagenase.

Methods: A standardized protocol involving injections of a combined enzyme solution (pbserum Medium™) was administered to the lower limbs in three separate sessions. Pre- and post-treatment photographs were collected for comparative analysis.

Results: All four patients showed improvements in skin appearance and fibrosis, with no systemic or local adverse events reported.

Conclusions: We propose that a treatment strategy targeting the edematous, adipose, and fibrotic components of EFP may offer an economical and pathogenic-based approach for managing this condition in affected women.

Introduction: Vitiligo is a prevalent skin disorder characterized by the destruction of melanocytes, leading to depigmented patches across various areas of the body. Interleukin (IL)-31 has been implicated in the development of pruritus and skin inflammation, potentially contributing to cutaneous symptoms. This study measures IL-31 levels in vitiligo patients with and without pruritus, comparing them to healthy controls, and explores the relationship between IL-31 levels, disease activity, and other clinical factors to assess its potential role in the early diagnosis of vitiligo.

Methods: Ninety individuals were enrolled in the study and equally divided into three groups: vitiligo, vitiligo with pruritus, and healthy controls. The serum level of IL-31 was measured using the enzyme-linked immunosorbent assay (ELISA).

Results: Significant differences in IL-31 levels were observed across all groups. IL-31 levels were highest in vitiligo patients with pruritus, followed by those without pruritus, and lowest in healthy controls, with mean values and standard deviations of 196 ± 67.28, 152.10 ± 74.39, and 80.03 ± 32.30 pg/ml, respectively. In addition, IL-31 levels in serum showed significant differences in relation to disease activity in both vitiligo groups. Positive correlations were found between IL-31 levels and the Vitiligo Area Scoring Index (VASI) and Vitiligo Disease Activity (VIDA) in both patient groups, as well as between IL-31 levels and lesion extent in vitiligo patients without pruritus. In patients with pruritus, IL-31 levels also positively correlated with age and the 5-dimension itch scale score.

Conclusion: IL-31 may serve as a crucial marker and play a significant role in the early diagnosis of vitiligo in patients both with and without pruritus.

Beta defensins (β-defensins) are peptides primarily produced by epithelial cells in mammals to safeguard the skin, other organs, and mucosa from microbial colonization. These peptides are generated by epithelial cells, keratinocytes, and macrophages, mainly in response to interactions with microorganisms (bacteria, viruses, and fungi) or the influence of various pro-inflammatory cytokines. Human β-defensin (HBD) 2 plays an indirect role in allergic reactions by promoting mast cell activation and degranulation. In dermatological and allergic conditions, the role of HBD2 has been well documented. Although HBD2 is predominantly produced in keratinocytes, along with HBD3 it has also been detected in serum. Elevated serum levels of HBD2 have been observed in patients with skin diseases such as atopic dermatitis and psoriasis. In addition, HBD2 is significant in chronic spontaneous urticaria (CSU), in which urticarial skin lesions can be triggered by infections. Notably, CSU is often accompanied by angioedema, which may be related to HBD2 because patients with CSU and associated angioedema have higher serum HBD2 levels compared to those without angioedema. Current evidence suggests that HBD2 could serve as a marker of inflammation and may have potential therapeutic applications. However, due to limited data on HBD2 levels and its expression in the skin of patients with allergic skin diseases, further research is needed to elucidate the underlying causes and mechanisms of elevated HBD2 levels in these conditions.