Acta Dermatovenerologica Croatica最新文献

We present a case of a 10-year-old boy with a longstanding history of seborrheic dermatitis (SD) referred to the Allergy and Immunology Department for recurrent Kaposi varicelliform eruption (KVE) secondary to herpes simplex 1 (HSV-1) infection and possible primary immunodeficiency. The patient was the second child of non-consanguineous parents, with an older, healthy brother. Family history was negative for primary immunodeficiency and skin disorders. The patient's skin problems began in infancy when he was diagnosed and treated by a dermatologist for SD. From preschool age, he was under the care of a pediatric neurologist and a defectologist for a sensory processing disorder. For the last two years, the patient had been receiving chlorpromazine therapy for aggressive behavior. The first episode of KVE was diagnosed at the age of six, following potent topical corticosteroid therapy for SD and sun exposure, another known risk factor for HSV infection. After the third KVE episode, prophylaxis with oral acyclovir was initiated. The skin changes were treated with topical steroids and oral antibiotics during disease flares, with poor clinical response. On presentation, the patient was in good general health, adipose, and of unremarkable somatic status, except for numerous symmetrical yellowish-brown keratotic papules and plaques on the forehead, cheeks, and the lateral side of the neck (Figure 1). The nail plate had multiple red and white longitudinal streaks and V-shaped notches on the distal free end of the nail plate (Figure 2). The allergy tests revealed increased total immunoglobulin E (IgE) and sensitization to ragweed. Immunological workup showed normal immunoglobulins and good specific immunity (good vaccine response and normal humoral response to HSV-1) but a decreased number of T- cells (CD3+ 1020/µL (1320-3300), CD3+CD8+ 281/µL (390-1100) with normal T-cell response after antigen stimulation. The diagnosis of Darier disease (DD) was confirmed based on medical history, clinical findings and histological finding of focal suprabasal acantholysis and dyskeratosis (Figure 3). Low-dose oral retinoid therapy was initiated with modest clinical response after 6 months of therapy. In the light of recent publication (1), we initiated intravenous immunoglobulin (IVIG) substitution (400 mg/kg every month) with excellent clinical response. After 4 months, the patient's skin improved in terms of reduced inflammation, scab healing, and reduced itching. Acyclovir prophylaxis was continued. The patient had no new episodes of KVE during follow-up. Kaposi's varicelliform eruption (KVE) or eczema herpeticum occurs in a chronic inflammatory skin disease such as atopic dermatitis (AD), SD, Hailey-Hailey disease, allergic contact dermatitis, psoriasis, and DD (2). It is considered a dermatologic emergency due to its high mortality rate if misdiagnosed or left untreated (3). DD is a rare autosomal dominant genodermatosis of variable expressivity caused by mu

We describe a rare case of an eccrine syringofibroadenoma with a foci of squamous cell carcinoma in situ, which has to best of our knowledge been reported only twice in the English literature. An excisional biopsy of an elevated, lobular tumor of the lower leg in an 86-year-old male patient was performed. Histologic examination revealed a tumor consisting of anastomosing strands of epithelial cells originating from the epidermis, occasionally showing ductal eccrine differentiation. Foci of squamous cell carcinoma in situ were observed within the described lesion. The diagnosis of eccrine syringofibroadenoma with squamous cell carcinoma in situ was established. Eccrine syringofibroadenoma is a rare lesion, mostly considered to be a reactive process arising secondarily in association with other cutaneous diseases such as dermatoses or neoplasms, although some researchers do not exclude the possibility that it is a primary neoplasm with a potential for malignant transformation.

Erythrodermic psoriasis (EP) is a very rare but extremely severe subtype of chronic plaque psoriasis. Its pathogenesis still remains unknown, and current therapeutic strategies frequently end in failure. Erythrodermic psoriasis often requires hospitalization in order to control any kind of possible serious complications. Treatment of EP is a challenge for clinicians because international guidelines are lacking. Nevertheless, Th17 has been shown to be the second-most predominant T-cell type after Th2 in EP lesions. There is a growing body of evidence supporting the safety and efficacy of biologics in rapidly achieving near-total clearance of EP, particularly within the IL-17 class. Herein we report a series of 5 cases of EP successfully treated with anti-interleukines 17: Ixekizumab and Secukinumab.

Background: Hidradenitis suppurativa (HS) is an under-diagnosed chronic inflammatory skin disease of the pilosebaceous unit of apocrine gland-rich parts of the body. The mammary area is the fourth most HS-affected area and, as typical lesions include non-fluctuating nodules, abscesses, and tunnels/sinus tracts, mammary HS is often mistaken for other mammary "boils", such as sub-areolar and granulomatous non-lactating breast abscesses. Our objective was to present a spectrum of mammary HS lesions, explore a possible classification, and expose mammary HS as a possible differential diagnosis to non-lactational breast abscesses.

Methods: A cross-sectional study on current and newly-referred patients treated for HS affecting the mammary area. Anamnestic information, subjective outcome measures, and lesion counts including anatomical location were collected. Patients with similar morphologies were grouped, and characteristics for the groups were investigated.

Limitations: We were not aware of the number of morphologies we would find, and as a result the study did not have sufficient power to show significant differences after correction for multiple testing.

Results: We found three morphologically different subtypes of mammary HS; the Sternal, the Frictional, and the Nodule types. These groups differed in anatomical lesion characteristics and other patient characteristics. Furthermore, we found a fourth Mixed type - a combination of the other three.

Conclusion: Differential diagnosis between mammary HS and sub-areolar or granulomatous non-fluctuating non-lactating breast abscess is most easily performed by assessing the precise anatomical location of the lesion and determining if the mammary lesion is the only lesion present or if similar lesions exist in other HS-specific areas.

Background: Melanoma can early metastasize to regional lymph nodes. The sentinel lymph node (SLN) is the first lymph node draining directly from the site of primary melanoma, and the pathohistological status of the SLN is the most significant prognostic factor for overall survival prevalence and prognosis in patients with melanoma. Ultrasound is a very useful for the imaging of regional lymph node metastases, combined with Doppler and cytopuncture.

Objective: The aim of this study was to investigate the role of ultrasound assessment of regional lymph nodes in melanoma staging.

Patients and methods: The study included all patients with primary melanoma detected in the period between 2003 and 2012, in whom diagnostic processing has not proven distant metastases or physical examination did not find enlarged lymph nodes. In total, 202 surgically treated patients were included in the study, of which 101 patients underwent ultrasound examination of regional lymph nodes using a linear probe of at least 12 MHz, while ultrasound of regional lymph nodes was not performed for 101 patients.

Results: The results of this study emphasize the importance of ultrasound in the diagnostics and treatment of patients with melanoma. Based on the observation of the occasional positive ultrasound and fine needle aspiration cytology (FNAC) in regional lymph nodes, our results indicate that a proportion of patients can avoid sentinel lymph node biopsy (SLNB). In case of a positive ultrasound findings (complemented with FNAC of suspicious nodes), direct dissection of regional lymph nodes is recommended. However, negative ultrasound findings do not exclude the presence of micrometastases due to poor sensitivity of this method and is not a contraindication for SLNB.

Conclusion: Therefore, there is a need for further studies on metastatic melanoma, especially those in the sentinel lymph nodes and in its early stage.

Dear Editor, Primary cutaneous diffuse large B-cell lymphoma, leg-type (PCDLBCL-LT) is a rare and aggressive neoplasm. A timely diagnosis may prevent fatal outcomes; physicians should take this entity into consideration when assessing non-specific lesions on the lower limbs. We present a 69-year-old woman with a 1-month history of a firm plaque on her left leg. Physical examination revealed an asymptomatic, indurated, smooth, and erythematous plaque on the pretibial region of her left extremity (Figure 1, a). The rest of the physical examination was normal. Histological examination revealed cohesive sheets of a dense cell infiltrate in the dermis, composed of large round immunoblast-type cells with prominent nucleoli, and the presence of mitoses. Immunohistochemical stains were positive for CD20, Bcl2, and MUM1 (Figure 1, b-d). Additionally, c-MYC and Ki67 exhibited a 20% positivity; CD3 and CD10 were negative. The diagnosis of PCDLBCL-LT was established. Imaging and blood workup ruled out systemic involvement. Treatment with R-CHOP chemotherapy was initiated, with complete tumor regression by the third cycle. The patient completed 6 cycles and has remained disease-free after 18 months. Primary cutaneous B-cell lymphomas (CBCL) are lymphoproliferative disorders that appear on the skin without evidence of extracutaneous manifestations at the time of diagnosis (1). They represent 25 to 35% of all primary cutaneous lymphomas (2). In 2018, an updated version of the 2008 WHO-EORTC classification divided CBCLs into 5 subtypes: PCDLBCL-LT, primary cutaneous marginal zone B-cell lymphoma (PCMZL), primary cutaneous follicle center lymphoma (PCFCL), Epstein-Barr virus-positive mucocutaneous ulcer (EBVMCU), and intravascular large B-cell lymphoma (3). PCDLBCL-LT is the least common subtype, representing approximately 10% of all CBCLs and only 4% of all cutaneous lymphomas (2,3). Although the pathogenesis for most CBCLs is still unknown, positive serology for Lyme disease in a significant number of patients has been recognized as a probable etiologic association (4). PCDLBCL-LT is more frequent in women, and the mean age of presentation is 76 years. It usually presents as erythematous or bluish nodules, and up to 75% of the cases appears on one or both legs (1). Although infrequent, other locations have been reported, including the head, neck, trunk, and upper extremities (5). Workup should include a complete physical exam, skin biopsy, blood tests, and imaging (2,3). Histopathology shows a diffuse infiltrate in the dermis composed of large B-cells (centroblasts and/or immunoblasts) with extension to subcutaneous cellular tissue. These cells have round nuclei that are more than twice the size of normal lymphocytes, with prominent nucleoli. The immunophenotype of PCDLBCL-LT is CD20+, CD79a+, CD10-, and Bcl-6+/-, and strongly expresses Bcl-2, MUM1/IRF4, and FOX-P1 (1-3). Unlike the other indolent subtypes, PCDLBCL-LT is generally more aggressive with a p

Dear Editor, The ongoing pandemic of coronavirus disease 2019 (COVID-19) was declared by the World Health Organization on March 11, 2020, and remains a global challenge. COVID-19 is caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), transmitted primarily through respiratory droplets and aerosols. Even though the COVID-19 vaccine has become available since December 2020, the main preventive measures still include social distancing, hand washing, and the use of protective face masks. By May 22, 2021, 3,437,545 deaths caused by SARS-CoV-2 have been registered by WHO, confirming the burden of this disease (1). Consequently, the pandemic has become a challenge for health care systems, as they had to be focused on the care of patients with COVID-19. During the first lockdown from March to May 2020, it was advised to postpone clinical visits whenever this could be done without risk. This recommendation was mainly aimed at older patients and those with chronic diseases, as it has been shown that they are at greater risk for complications from COVID-19. Renal transplant recipients (RTRs) are at a greater risk for infections and different cancers due to their permanent immunosuppressive therapy. The most common malignancies in RTRs are skin cancers, particularly non-melanoma skin cancers. It has been estimated that RTRs have a 65-250 times higher risk for cutaneous squamous cell carcinoma (SCC), 10 times higher risk for basal cell carcinoma, and 2-5 times higher risk for melanoma when compared with the general population (2-4). RTRs are at a higher risk for complications from COVID-19, not only because of their immunosuppressive therapy but also because of different comorbidities, such as hypertension, cardiovascular disease, and diabetes mellitus (5). Therefore, RTRs tend to limit their medical visits and postpone clinical examinations for skin cancer screenings. Moreover, during clinical visits the patients are commonly asked to keep their protective masks on, increasing the risk of overlooking their facial skin changes. Herein we present two RTRs who developed skin cancers during the COVID-19 pandemic, and the tumors were diagnosed with a significant delay. Patient 1 A 67-year-old woman with unknown primary kidney disease received a renal allograft from a deceased donor in 2014. The immunosuppressive protocol included antithymocyte globulin induction with tacrolimus, mycophenolate mofetil, and steroid maintenance. In January 2020, she had noticed a reddish squamous lesion on her right cheek, which enlarged slowly. Since there were no other symptoms, she postponed the dermatologic examination. Additionally, she further postponed the visit to her physician during the pandemic as she wanted to avoid social contact as much as possible. One year later, at the nephrologist's examination, she was asked to take off her face mask for a skin check, and two skin tumors on her right cheek were noticed (Figure 1). One lesion was located at

Background: The role of the T-regulatory cells (Tregs) marker forkhead box Protein 3 (FOXP3) in mycoses fungoides (MF) pathogenesis is unclear and the results of previous studies are inconclusive.

Objective: We aimed at ascertaining the possibility that FOXP3 expression may serve to predict MF stage and response to therapy.

Patients and methods: Immunohistochemistry staining for FOXP3 was performed on 30 skin biopsies from patients with MF, and FOXP3 expression level was quantitatively graded. Disease stage, progression, and response to treatment were determined based on clinical and imaging evidence, and association with FOXP3 expression was assessed.

Results: FOXP3 expression in the dermis correlated with poor response to treatment (P=0.047). A negative non-significant relationship between epidermal FOXP3 expression and clinical stage severity was observed (P=0.17).

Conclusions: Dermal FOXP3 expression in MF lesions could be used to predict response to treatment in patients with MF.

This retrospective study included 63 patients with obesity (Body Mass Index; BMI ≥ 30) and psoriasis. Our aim was to verify the effectiveness of different systemic therapies administered to the above cohort of subjects over a period of 1 year. Improvements of 75%, 90%. and 100% compared with the baseline Psoriasis Area Severity Index (PASI 75, PASI 90, and PASI 100, respectively) were used as clinical outcome measures. In a median time of 16 weeks, 85.7%, 68.2%, and 38.0% of patients achieved PASI 75, PASI 90, and PASI 100, respectively. In parallel, the Dermatology Life Quality Index (DLQI) and the visual analog score for measuring itch intensity (VAS itch) decreased significantly (P<0.0001 for both parameters). At the achievement of PASI 75, BMI increased as compared to baseline (P=0.02) and did not significantly vary at the attainment of PASI 90 and PASI 100 (P= 0.07 for both outcomes). Logistic multivariate regression analysis showed that treatment with biologic drugs was a positive predictor for achieving PASI 75, PASI 90, and PASI 100. BMI >31.7 and the presence of psoriatic arthritis were negative predictors for the achievement of PASI 90, while having a DLQI >6 was a positive predictor.

Hidradenitis suppurativa (HS) is a chronic inflammatory skin disease primarily affecting apocrine gland-rich areas of the body and presenting with painful nodules, abscesses, sinus tracts, and scarring (1). HS is a defect of the follicular epithelium; some have therefore called for the naming the disease acne inversa instead of hidradenitis suppurativa. The term acne inversa links the pathogenesis to acne and reflects the fact that it is an expression of follicular occlusion in localizations inverse to acne vulgaris (2). HS typically occurs after puberty. Studies have shown that the average onset is in the second or third decades of life (3). One of the most frequently cited risk factors for HS is cigarette smoking. Another significant risk factor for HS is obesity. About one-third of patients with HS have reported a family history of the disease (4). A clinically relevant staging and disease severity assessment is essential for the development of evidence-based treatments. There are several scoring systems for the assessment of disease severity of HS, including Hurley staging, HS Physician's Global Assessment (PGA), the modified Sartorius score (MSS), and the HS Severity Index (HSSI). Each of these assessments has both advantages and limitations in daily practice; there is currently no gold standard (5-8). The Hurley staging system is the simplest and most widely used instrument for HS classification in routine clinical practice. It classifies HS into three stages. HS-PGA is relatively easy to apply and is frequently used to measure clinical improvement in clinical trials of medical treatments (5). The system describes six disease stages, increasing in severity on a scale from 1 to 6 (9). MSS is a more detailed and dynamic classification system based on the counting of individual nodules and fistulas within seven anatomical regions. The system, which was developed by Sartorius et al. and later modified, is the first disease-specific instrument for dynamically measuring clinical severity of HS (10). The treatment of HS includes topical clindamycin, triamcinolone acetonide, clobetasol, topical resorcinol, oral antibiotics, hormonal therapy, oral retinoids, and biologic therapies (11). Biologic therapies are increasingly used in patients who fail to sufficiently respond to antibiotic and hormonal treatments. Adalimumab, infliximab, and etanercept have all been tested in the treatment of HS but vary in effectiveness and in how well they have been studied. Subcutaneous weekly adalimumab (160 mg at week 0, 80 mg at week 2, and 40 mg each week thereafter) is the only biologic agent approved by the US Food and Drug Administration (FDA) and the European Medicine Agency (EMA) for the treatment of HS, and it is recommended as first-line therapy for patients with moderate-to-severe disease who are intolerant or unresponsive to oral antibiotics (12). The first male patient aged 59 years was referred to our Department with very long history of HS. The f