Acta dermatovenerologica Croatica : ADC最新文献

Cutaneous leishmaniasis (CL) is common in the pediatric population, but there are only a limited number of studies focused on the clinical and epidemiological characteristics of patients in this age group. In this study, our objective was to investigate the epidemiological and clinical characteristics of pediatric subjects diagnosed with CL. A total of 8047 patients who had been diagnosed with CL between 2010 and 2021 in an endemic region were included in this retrospective study. The clinical and demographic characteristics such as age, gender, number, size, duration, location, and type of lesions and the administered CL treatments were recorded. In order to better understand the epidemiological and clinical characteristics of patients with pediatric CL (PCL), the study patients were divided into three groups according to their age (0-6, 7-12, and 13-18 years) and the clinical and epidemiological characteristics of these groups were compared. When patients with PCL were compared according to age groups, it was found that the highest number of patients were in the 13-18 age group. It was determined that the patients in the 6-12 age group had fewer lesions, that and the size of the lesions was smaller than the other groups. The disease duration was the longest in the 0-5 age group. The highest rate of nodular, ulcerated, and recurrent lesions was in the 13-18 age group, and the highest rate of papular lesions was in the 6-12 age group. Systemic pentavalent antimony therapy (IM or IV) was administered to 438 patients with PCL (5.44%), while intralesional pentavalent antimony therapy (IL) was administered to 7447 patients (92.54%). Patients receiving systemic therapy had larger lesions compared with patients receiving IL therapy and no treatment. The lesion duration was longer in patients who received systemic treatment, and the number of lesions was higher than those who received IL treatment. The highest rate of systemic treatment was in the 13-18 age group (43.8%). In conclusion, our study found that the intragroup comparison of the age group with the highest CL rate displayed similar clinico-epidemiological characteristics reported in previous studies conducted in the same region.

Vulvar lichen sclerosus (VLS) is an inflammatory disease with numerous detrimental characteristics, namely its distressing symptoms, chronic course, sexual dysfunction, disfiguring anatomical changes, only partial response to treatment, and risk of evolution towards cancer. Interest in the burden of VLS on patient quality of life is fairly recent and only relatively few studies have addressed it so far. In order to focus on the impact of VLS in affected women, an electronic search was performed using the National Library of Medicine PubMed database. All the studies assessing VLS-related suffering and quality of life impairment published in the English literature were analyzed, including controlled studies, case series, and guidelines. The available literature shows that VLS can negatively affect patients' daily lives and significantly impair their physical and social activities, mental health, self-esteem, sexual functioning, and satisfaction. Health-related quality of life impairment among women with VLS is defined as moderate to severe, comparable to that of patients affected with other high-impact chronic skin disorders such as atopic dermatitis, psoriasis, and hidradenitis suppurativa. The symptoms are the main causes of the detrimental effect of VLS. By ameliorating symptoms, treatments are expected to highly improve patient quality of life, especially in case of complete clearance. Treating VLS has a beneficial impact on sexual dysfunction as well, even though dyspareunia appears less responsive than the other disease-related symptoms. In conclusion, the emotional and sexual dimensions are strongly impaired by VLS. Both in clinical practice and in clinical trials, quality of life and suffering should be taken into account and considered as strong conditioning factors in patient well-being. They should also become a measure of therapeutic response in treated patients.

Neutrophilic dermatosis of the hands (NDDH) is a localized variant of Sweet's syndrome which has been recently introduced. Strutton et al.in 1996 and then in Galaria et al. in 2000 reported cases with violaceous papulonodules on the dorsal surfaces of the hands with histopathological findings of a neutrophilic dermatosis in association with leukocytoclasia, but clinically and histologically without true vasculitis findings. Eventually, they proposed the term NDDH for these lesions (1,2). A 46-year-old man was referred to our outpatient dermatology clinic with a painful ulcerative lesion on the dorsal side of the left hand that had been present for one year. Initially, the lesion had appeared as a small purulent papule, which gradually extended to a large ulcer. The patient underwent frequent referrals to several physicians and had taken a variety of topical and systemic antibiotics, meglumine antimoniate (Glucantime), and amphotericin with the clinical diagnosis of cutaneous bacterial or fungal infections, or leishmaniasis. All of these therapeutic regimes were ineffective in eradicating the lesion. Given the history, he denied any trauma to the site of lesion; he also did not report any similar lesions in his family. The physical examination revealed an extensive tender ulcer of 4×7 cm2 in size, with a shallow violaceous border superimposed on an edematous region on the dorsal side of the left hand. Atrophic scars resulting from old similar lesions were visible on the dorsal aspects of the 3rd and 4th proximal and middle metacarpal joints (Figure 1). The examination of the other parts of the body was unremarkable. Laboratory tests showed an impaired white blood cell count and their differentiation, including leukocytosis (white blood cell count of 16.12/mm3) with neutrophilia (neutrophil percent at 65.9%). Additionally, altered liver function tests were remarkable for high serum levels of AST (SGOT) (105 IU/L) and ALT(SGPT) (355 IU/L), while the total bilirubin and alkaline phosphatase were within normal limits. Hemoglobin levels (13.90 g/dL) and platelet count (272/mm3) were within normal range. The other laboratory tests, including serological tests for fasting blood sugar, hemoglobin A1c, creatinine, BUN, and an immunoassay for ruling out vasculitis lesions (anti-MPO (P-ANCA) and anti-PR3 (C-ANCA)) revealed no remarkable results. An erythrocyte sedimentation rate of 16 mm/h was reported. A biopsy was performed. Histologic features demonstrated a dense, diffuse dermal infiltrate comprised almost entirely of neutrophils. The epidermis was slightly acanthotic and showed small foci of spongiosis, but the inflammatory infiltrate remained largely in the dermis. Sheets of neutrophils were present, admixed with karyorrhectic debris. The infiltrate did not appear to be peri-vascular, and most vessels that could be observed clearly appeared to be undamaged. However, some vessels appeared to show some neutrophils infiltrating vessel walls (Figures

Lupus erythematosus tumidus (LET) is a rare photosensitive skin disease classified as a separate subtype of cutaneous lupus erythematosus. Clinically, it is characterized by erythematous plaques on sun-exposed areas. Typical histopathological findings are perivascular and periadnexal lymphohistiocytic infiltrates and prominent mucin deposition in the dermis. Treatment is based on photoprotection, topical corticosteroids, and antimalarial drugs. The exact pathogenesis of the disease is unknown. Drugs are considered a minor risk factor for the development of LET. We present a case of a 56-year-old woman who developed LET after starting treatment with atorvastatin. We describe her clinical course and review the literature concerning the cutaneous adverse reactions induced by statin drugs. To our knowledge, this is the first case of statin-induced LET. We conclude that statins can induce LET and that it is important for clinicians to be aware of this potential adverse effect associated with statins.

Background: Multifocal infantile hemangiomas (IHs) are focal cutaneous lesions affecting more than 1 anatomic site. The multifocal distribution pattern is the rarest form of IH manifestation, accounting for only 3-4% of all affected infants. This type of cutaneous IHs may be a marker for extracutaneous disease, with the liver being the most frequently affected organ.

Methods: We investigated the clinical and epidemiological characteristics of a small case series of infants with multifocal IHs presenting with different clinical patterns, all diagnosed and treated in a regional tertiary-care pediatric clinic.

Results: Four infants with multifocal IHs were included in the analysis. There were 3 girls and 1 boy. Three out of 4 infants were prematurely born (2 of them very preterm), while only 1 was full-term. Clinical patterns in all cases were quite different, but more than 20 cutaneous IHs were present in each of the patients. Two infants had multifocal liver hemangioma, but without complications. In 3 out of 4 patients, systemic propranolol therapy was introduced, with excellent response in two cases (both with liver involvement).

Conclusion: With the increase in the number of cutaneous IHs, the probability of internal organ involvement, most often the liver, also increases. Evaluation for extracutaneous lesions is indicated in infants with 5 or more cutaneous IHs. Treatment of infants with multifocal IHs should be individualized and consider all relevant risk factors, including prematurity.

While basal cell carcinoma is the most common type of skin cancer in humans, its subepidermal presentation is extremely rare. The risk factors for basal cell carcinoma development are well-known, but it remains unclear in which setting the tumor restricts itself to the dermal compartment. We present the fifth known case of subepidermal basal cell carcinoma. However, this particular presentation is unique due to arising beneath a capillary malformation. The patient had previously undergone multiple laser treatments which yielded no success. Initially, the vascular malformation was removed and sent for histopathological diagnosis. After the discovery of basal cell carcinoma, wide surgical resection was performed. The patient had no recurrence up to the last follow-up at 18 months postoperatively. This case demonstrates a new presentation of a very rare condition, but also highlights the importance of histopathological examination and the need for future research on any possible association between laser therapy and carcinogenesis.

Merkel cell carcinoma (MCC) is a rare and highly aggressive primary cutaneous neuroendocrine carcinoma most often occurring in the elderly. Risk factors include chronic sun exposure and immunosuppression (1). MCC is associated with frequent recurrences and a high metastatic potential and mortality rate (1). It is the second most common cause of skin-cancer-related death after melanoma. At primary diagnosis with an apparent cutaneous tumor, loco-regional metastases are present in up to 30% of patients, and 6-12% have distant metastatic disease (2-3). Up to 5% of cases present with unknown primary origin (4). Five-year overall survival for patients with advanced or metastatic disease is 13-18% (4). We report two cases of MCC presenting without primary cutaneous involvement; first at an unusual location in the adipose tissue of the right breast, and the second one with only a clinically positive left inguinal lymph node. In October 2018, a 78-year-old woman presented with a 15-week history of a painless solitary mass in the upper outer quadrant (UOQ) of the right breast with no visible cutaneous involvement. Her medical history included hypertension, dyslipidemia, and plaque psoriasis. She underwent ultrasound guided biopsy, and histopathology confirmed the diagnosis of metastatic MCC (mMCC). Positron emission tomography/computed tomography (PET/CT) scans showed increased standardized uptake values in the mass in the UOQ and an additional mass in the lower inner quadrant (Figure 1A). The patient underwent mastectomy and lymph node dissection of the right axilla. Histopathology confirmed mMCC and negative axillary lymph nodes. Regular follow-up (clinical examination, PET/CT scan, ultrasound, mammography) every 6 months revealed no disease recurrence during this 4-year period (Figure 1B). In September 2021, a 66-year-old man was referred to our Clinic with clinically detectable painful left inguinal lymphadenopathy. Excisional biopsy was performed, and histopathology confirmed the diagnosis of mMCC (Figure 2). After an extensive clinical and imaging evaluation (PET/CT scan), which confirmed disseminated disease (Figure 3A), initial treatment with the programmed cell death ligand 1 inhibitor (anti PD-L1) avelumab was proposed. The first cycle consisting of seven intravenous applications, and was applied in October 2021. After one year and completion of the third cycle of therapy, imaging assessment (PET-CT scan) detected a solitary lesion in the pancreas. Fine needle aspiration biopsy confirmed a distant metastasis of MCC that was later treated with stereotactic radiosurgery. The fourth cycle of immunotherapy was completed in March 2023. No treatment-related adverse events were noted during these 18 months of follow-up. Recent PET/CT scans demonstrated scaring tissue in the pancreas with no signs of locoregional or distant metastatic disease (Figure 3B). Management of MCC should be individualized based on the specific pattern of disease presentat

Muir-Torre syndrome (MST) is a rare autosomal dominant subtype of hereditary non-polyposis colorectal carcinoma. The diagnosis is established based on the coexistence of sebaceous gland tumors and visceral organ malignancies. Mutations in the mismatch repair genes are responsible for Muir-Torre syndrome. Internal malignancies seen in MTS are most commonly colorectal, gastrointestinal system, endometrial, genitourinary system, breast, lung, brain, and hepatobiliary system malignancies. Detection of sebaceous neoplasia is essential in investigating Muir-Torre syndrome, allowing early detection of internal malignancies. Herein, we present the case of a patient with sebaceous adenomas, internal malignancies, and a new mutation detected during the genetic examination.

The mortality risk factors for Corona Virus Disease-19 (COVID-19) infection (caused by the severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2)) include advanced age, male sex, certain comorbidities, and immunosuppression (1). Pemphigus vulgaris is a rare mucocutaneous autoimmune disease with autoantibodies against desmosomal desmoglein-1 and desmoglein-3, resulting in acantholysis and blister formation. This epithelial barrier defect increases susceptibility to infections, which may lead to relapses (2). Additionally, therapy-associated immunosuppression can lead to severe infections. Corticosteroids are the mainstay therapy. For moderate and severe pemphigus, rituximab is recommended in first-line treatment along with other immunosuppressants, and it may also be added in refractory cases. It is a monoclonal antibody against CD20 with long-lasting B-cell depletion potency. Recovery of B-cell function may last from one to seven years. Consequently, patients receiving rituximab cannot produce enough COVID-19 specific plasma cells, leading to a severe course of COVID-19 (2). Shashidi-Dadras et al. reported five mild COVID-19 cases among 167 patients with pemphigus who had received rituximab one to five years earlier. The authors presumed rituximab use within five years increases COVID-19 susceptibility regardless the number of courses received (3). Among 48 patients with pemphigus treated with rituximab within five years, Uzuncakmak et al. reported one mild case of COVID-19 (in a patient who had received a single course seven months earlier) (4). In another study, high titers of SARS-CoV-2 antibodies and high counts of antibody-secreting cells were associated with severe COVID-19 (5), which may be the consequence of antibody-dependent enhancement (6). Mahmoudi et al. concluded that B-cells may not be necessary for recovery in COVID-19, but they may protect from reinfection (7). Considering these data, rituximab should be postponed during the pandemic (8). In exceptional cases, it may be applied with careful consideration of the risk-benefit ratio (2,4). Patients should be monitored for signs of COVID-19 before and during treatment. A 63-year-old woman with pemphigus vulgaris presented at our department with widespread skin lesions. Comorbidities included hypertension, hypothyroidism, and glaucoma. Diagnosis was established based on histology and direct and indirect immunofluorescent microscopy results. Both desmoglein-1 and desmoglein-3 autoantibodies were detectable by ELISA. The patient was initially treated with low-dose systemic methylprednisolone (8 mg/day), because glaucoma contraindicated a higher dose. Azathioprine was subsequently started (gradually increased from 0.6 to 2.5 mg/kg/day). Continuous mucocutaneous progression 4 weeks later led to the decision to add rituximab therapy. The patient was confirmed as SARS-CoV-2 negative and received 1000 mg 12 weeks after starting glucocorticoid treatment. Two weeks later, she devel

Spitz tumors are a subset of melanocytic neoplasms characterized by epithelioid or spindled melanocytes(1). The benign nature of the "Spitz nevus" has since been clarified, but the debate regarding Spitzoidtumors (STs) is still ongoing. Spitzoid tumors encompass a wide spectrum of cutaneous lesions ranging from benign Spitz nevus (SN) to Spitzoid melanoma (SM), the latter displaying capacity for widespread metastasis and a potentially lethal outcome (2). The term atypical Spitz tumors (ASTs) refers to melanocytic tumors exhibiting the morphological features of SN, as well as some features associated with malignancy, but not sufficient to classify them as SMs. Currently, histopathology is the gold standard for the diagnosis of STs and cutaneous MM. However, the differential diagnosis between benign and malignant melanocytic lesions with spitzoid features remains challenging (3-6). In order to facilitate the work of clinicians and pathologists, we attempted a comparative clinical and demographic study comparing ASTs and MMs of patients referred to two Italian institutes. Patient data were obtained from two different Italian dermatological centers (Melanoma Registry of the Instituto Dermopaticodell'Immacolata IDI-IRCCS Rome, Lazio and the Skin Cancer Unit of Dermatology, Hospital Sant'Orsola-Malpighi, University of Bologna), from January 2007 to December 2017. Histological reports presenting pre-operative queries of both "atypical Spitz nevi" or "malignant melanoma" and a final diagnosis confirming one of the queries were included in the study. The chi-square test or Mann-Whitney U-test were applied to analyze differences between the groups for categorical variables such as sex, diagnosis, and continuous variables (age). The "anatomic site" variable was classified into three categories as follows: the limbs, trunk, and head/neck. A multivariate binary logistic model was used to investigate if the anatomic site was an independent predictor of MM. Age and sex were considered confounding factors. A total of 504 patients (51.8% men; 48.2% women) met the inclusion study criteria (mean age 52 years, SD = 22.8) (Table 1). 373 were cases of MM and 131 were cases of AST. Mean age of MM cases and AST were 61.2 years old (SD = 17.6) and 25.8 years old (SD = 13.8), respectively. Subjects with MM were predominantly men (58.2% versus 33.6%) (P<0.0001) and older (median age 62 years versus 25 years) (P=0.0001) than subjects with AST. The most frequent anatomic site for MM was the trunk (39.7 %), while the lower limb was the most frequent anatomic site for AST (48.1 %) (P<0.0001). Table 2 shows the multivariable analysis used to assess if anatomic site was an independent predictor of cutaneous melanoma. Multivariate analysis confirmed an increased risk for MM in comparison with AST for both localization on the trunk (OR:2.78; 95 %CI: 1.74-4.45) (P<0.0001) and head/neck (OR:3.20; 95% CI: 1.60-6.38) (P=0.0001). After introducing age (model 1, OR: 2.11; 95% CI: