Acta Dermatovenerologica Alpina Pannonica et Adriatica最新文献

Idiopathic acquired true leukonychia is a rare benign acquired disorder that typically affects adults. Diagnosis is made clinically, with investigations required to exclude any underlying cause. The cause of this condition is largely unknown, yet it understandably causes significant anxiety to patients due to concerns about underlying systemic disease. We report a case of idiopathic acquired true leukonychia totalis in a healthy 20-year-old man. Intermittent complete whitening of some of the fingernails was reported over a 7-year period. No underlying medical cause or laboratory abnormality was identified. This case highlights the importance of considering idiopathic acquired true leukonychia as a differential diagnosis of nail whitening. In addition, increased awareness of this unique entity is needed for patient reassurance, as well as avoidance of unnecessary investigations and prolonged systemic treatment.

Introduction: Skin cancer, a significant global health issue, requires prompt detection and management, particularly among the elderly. Primary care physicians play a critical role in early diagnosis because they are often the first to evaluate patients with skin lesions. Accurate diagnosis by family physicians is essential for effective treatment and improved patient outcomes.

Methods: Over a 12-year period, this study analyzed 125 samples from 89 patients that presented with visible skin changes or suspicious lesions identified during routine examinations. Initial working diagnoses made by family physicians were compared to final pathohistological diagnoses (PHD).

Results: The study found a significant difference between working diagnoses and final PHDs for malignant versus benign cases, with an accuracy of 83.3% and statistical significance (p = 0.04). A difference of 12.9% was observed, indicating areas for improvement. The diagnostic effectiveness for premalignant versus benign cases is high (accuracy of 88.2%), but without statistical significance (p = 0.50), and it suggests that these differences are not clinically relevant.

Conclusions: Primary care physicians demonstrate high accuracy in identifying malignant skin conditions, which is crucial for effective skin cancer management. However, improving diagnostic accuracy, particularly to reduce the misclassification of benign conditions as malignant, remains necessary. Continued training and development are essential to further enhance diagnostic precision for all skin conditions.

Actinic granuloma (AG) is a rare dermatological condition with only a few dozen cases reported worldwide. Initially classified as a variant of granuloma annulare, it is now recognized as a distinct entity characterized by asymptomatic annular plaques in sun-exposed areas of the skin. The exact pathogenesis remains unclear, but it is believed to be an inflammatory response to sun damage, possibly involving injured elastic fibers. Numerous local and systemic therapeutic options exist, but no specific treatment guidelines have been established. We present a case of AG treated with intralesional application of triamcinolone acetonide in a 64-year-old male patient. We also discuss the most important clinical and histological characteristics and various treatment options.

Hailey‒Hailey disease is a rare chronic autosomal-dominant blistering disease characterized by erosions, fissures, and vegetations occurring in intertriginous regions. To date, there is no specific treatment and there are no therapeutic guidelines, which makes management of the disease challenging. We present the case of a 43-year-old man unsuccessfully treated for Hailey‒Hailey disease with topical and systemic corticosteroids, antibiotics, and surgical debridement. At presentation he had erosions, vegetations, and infection in the axillae and groin. We introduced oral methotrexate, 10 mg weekly, and complete remission was achieved in 3 weeks. After 8 weeks, we decided to discontinue methotrexate due to lesion absence. Over 3 years of follow-up, mild flares were effectively managed with topical miconazole or mild steroid creams. We conclude that oral methotrexate is safe and effective for achieving long-term remission in Hailey‒Hailey disease.

Multiple autoimmune syndrome is a manifestation of polyautoimmunity with the co-occurrence of three or more autoimmune diseases in a single patient. We report a unique case of a 55-year-old female patient that presented with four autoimmune diseases: autoimmune thyroid disease, vitiligo, morphea, and lichen sclerosus. She was evaluated for progression of morphea and lichen sclerosus, and we confirmed histopathological overlapping of these two diseases in the same lesion. We discuss the increasing prevalence of autoimmune diseases and similar case reports on dermatological polyautoimmunity.

Introduction: Recent research has shown that blood coagulation and the extrinsic coagulation cascade are involved in the pathogenesis of chronic spontaneous urticaria (CSU), but little is known about the coagulation factors in angioedema.

Methods: This study included 58 participants: 29 patients with chronic angioedema (14 with isolated angioedema and 15 with angioedema with wheals) and 29 healthy controls (HCs). We compared the values of coagulation factors in patients with isolated angioedema to those with wheals. Plasma levels of D-dimer, fibrinogen, and factor VII were measured by enzyme-linked immunosorbent assay (ELISA) for all participants.

Results: Significantly higher D-dimer (p = 0.016; ε² = 0.381) and fibrinogen (p = 0.044; ε² = 0.331) levels were recorded in patients with angioedema (both groups) than in the HCs, with higher levels for angioedema with wheals. Factor VII and fibrinogen levels did not differ significantly between the groups with angioedema, but coagulation factors were more often elevated in both angioedema groups than in HCs.

Conclusions: One characteristic of angioedema is an elevated blood coagulation potential, which may help produce fibrin and may be important in controlling angioedema attacks.

Introduction: This study explores the immunogenetic associations of human leukocyte antigens (HLA) and the calcium release-activated calcium modulator 1 (ORAI1) and stromal interaction molecule 1 (STIM1) genes in HIV-1‒positive patients with HIV-related skin disorders.

Methods: This study assessed the distribution of variants of HLA class II alleles and expression levels of ORAI1 and STIM1 genes in the blood between HIV-1‒positive patients with HIV-related skin disorders and the control group with no HIV within the Latvian population.

Results: The research group comprised 115 HIV-1‒positive patients with HIV-related skin disorders, and the control group included 80 healthy individuals. Risk alleles (HLA- DQB1*02:01-0301 and HLA-DQA1*01:01-0501) and protective alleles (HLA-DRB1*07-13, DRB1*01-13, DRB1*04-11, and HLA-DQA1*05:01-0501) showed statistical significance in the groups. In 38 out of 115 patients, higher expression levels of ORAI1 and STIM1 genes were detected in the blood at the beginning of treatment. A significantly higher level of the microribonucleic acid (mRNA) ORAI1 gene was also found in the control group.

Conclusions: The results demonstrate that HLA class II alleles are associated with a trend toward risk/protection concerning HIV-related skin disorders in HIV-1‒positive patients. It was also shown that a low level of ORAI1 mRNA and the risk allele HLA-DQB1*0201-0301 were simultaneously present in the research group.

Introduction: This study examined the remission probability and duration in chronic spontaneous urticaria (CSU) patients resistant to second-generation H1-antihistamines (sgAHs) undergoing omalizumab treatment.

Methods: This is a retrospective observational study of 176 adult CSU patients exhibiting a significant pruritus component (≥ 8) of the weekly urticaria activity score (UAS7) despite four daily sgAH tablets and starting omalizumab treatment with 300 mg every 4 weeks. After excluding 13 nonresponders, we analyzed 163 omalizumab responders (mean age 51.8 years, 74.4% female). The intervals between applications were increased. Discontinuation was considered for patients that remained asymptomatic on a gradually reduced dosage (to 150 mg every 12 weeks) without sgAHs.

Results: Omalizumab discontinuation was possible in 25.8% (42/163). The duration of omalizumab treatment before remission ranged from 7 to 63 months. Twenty-one patients (50.0%) maintained complete remission until the end of the observation period (September 2021) for 8 to 68 months. Of the relapsed patients, 71.4% (15/21) effectively controlled CSU with sgAHs. Six patients (28.6%; 6/21) required omalizumab reintroduction after 6 to 40 months of remission, responding favorably.

Conclusions: The study shows that a quarter of severe CSU patients achieve long-term remission. In addition, sgAHs effectively manage symptoms in a majority of relapsed cases, and those requiring omalizumab reintroduction respond favorably.

Introduction: Dermatomyositis (DM) is a group of autoimmune idiopathic inflammatory myopathies characterized by typical cutaneous signs and symptoms of muscle involvement. The diseases can be associated with cancer in the paraneoplastic syndrome, calcinosis, interstitial lung disease, other autoimmune connective tissue diseases (in overlap syndrome), and Raynaud's phenomenon.

Methods: Clinical and capillaroscopic data were gathered from 43 patients with DM. The diagnosis was based on the Bohan‒Peter and European League against Rheumatism / American College of Rheumatology (EULAR/ACR) classification criteria. In addition, nailfold capillaroscopy was performed in all patients.

Results: In our cohort, eight patients had overlap syndrome, six had paraneoplastic syndrome, eight presented with interstitial lung disease, and nine had calcinosis, two of whom also had a cancerous pathology. Raynaud's phenomenon was reported in 74% of patients. Upon nailfold capillaroscopy, 84% of patients presented giant capillaries, 81% ramified capillaries, and 70% both. The latter, notably giant ramified capillaries, could be considered specific for DM. The detection of prominent subpapillary venous plexuses was associated with pulmonary involvement. In contrast, alterations of the pericapillary spaces were associated with the severity and prognosis of DM.

Conclusions: Our results underline the usefulness of nailfold capillaroscopy in the diagnosis and prognosis of DM. Based on the results and literature data, specific nailfold capillaroscopy features should be included in DM diagnostic criteria.