Acta dermatovenerologica Croatica : ADC最新文献

Dear Editor, Pemphigus vegetans (PV) of Hallopeau is a rare and indolent variant of pemphigus clinically characterized by vegetating lesions preceded by pustules mainly in flexural areas (1,2). This helps us to differentiate it from PV of Neumann, which is a more extensive and refractory disease, more alike to a pemphigus vulgaris outbreak with blisters which turn into vegetating plaques (3). We report the clinical presentation, course, and therapeutic response in a patient diagnosed with PV of Hallopeau from its early stage during a 3-year follow up. A 62-year-old man, non-smoker, presented at our clinic in July 2018 with hemorrhagic-serous crusts and fissures on the vermilion of the lower lip (Figure 1, a) and two merged circinate, sharply demarcated plaques on the right side of the groin (Figure 1, b). Plaque margins were elevated, with hypertrophic granulation tissue studded with pustules. Mucosal and cutaneous lesions persisted 6 and 4 weeks, respectively. The rest of the mucosa and skin were unaffected; the general state was good. The patient's family history for skin diseases was negative. The medical history included hypertension, atherosclerosis and hypercholesterolemia, hiatus hernia, and recent surgery (3 months prior) of an aortic abdominal aneurysm with reconstruction and synthetic graft placement. He was taking antihypertensives (fixed combination of 3 drugs, among them the ACE-inhibitor perindopril) with well-regulated blood pressure, statins, a pump-proton inhibitor, and acetylsalicylic acid. Differential blood count revealed eosinophilia. Histopathology finding showed acanthosis, suprabasal clefting with a suprabasilar bulla and acantholysis, prominent eosinophilic intraepidermal spongiosis, and heavy dermal infiltration of eosinophils and lymphocytes (Figure 2, a and b). The diagnosis of pemphigus was confirmed by direct immunofluorescence (DIF), which detected C3 deposits on the surface of keratinocytes throughout the epidermis of perilesional skin. Circulating pemphigus antibodies were detected by indirect IF. Only Dsg 3 antibodies were detected using an ELISA assay (233.23 RU/mL). After establishing the diagnosis of PV of Hallopeau, treatment with prednisolone 0.75 mg/kg/day orally in combination with adjuvant immunosuppression (azathioprine 100 mg daily) was started. Appropriate topical therapy with local steroids and antiseptic was applied. The steroid dose was titrated and gradually tapered down to the minimum required to control the disease - 10 mg. One-year remission was achieved. Azathioprine was withdrawn in October 2019 and since then the patient experienced a flare-up twice. The control of pemphigus flare-ups was achieved by a low dose of steroids (30 mg prednisolone orally). It remains debatable whether surgical trauma and radiology procedures such as angiographies (4) well as ACE-inhibitor drugs (5) triggered or aggravated the pemphigus. Early recognition and correct diagnosis of this rare type of pemphigus a

Dear Editor, The Leser-Trélat sign is a rare paraneoplastic cutaneous marker of internal malignancy characterized by sudden eruption of multiple seborrheic keratoses (SK). It is mostly associated with gastrointestinal adenocarcinomas (gastric, colon, rectal), and less frequently with breast cancer and lymphoproliferative disorders/lymphoma (1). It can be also associated with lung, kidney, liver, and pancreas malignancy (1). Pruritus occurs in half of the patients. Lesions rarely require any treatment, as they mostly tend to resolve once management of the underlying malignancy has started (2). A 32-year-old female patient with family history of colorectal cancer presented with an acute eruption of SK. She reported that the first symptoms were the loss of appetite and intense pruritus. The brown papules appeared over a period of 2-3 months, first on her back, then on the abdomen, thorax, neck, and lasty on the extremities (Figures 1a and b.). Physical examination showed numerous brown hyperkeratotic papules and plaques on the trunk, neck, and extremities. The patient complained of night sweating, epigastric pain, and heartburn. Over the last three months, she had lost over 15 kg. The patient had experienced an episode of acute gastritis 10 years ago and had been treated for Helicobacter pylori infection 4 years ago. Laboratory results showed elevated sedimentation rate and decreased levels of hemoglobin, erythrocytes, and hematocrit. CA-19-9 and CEA levels were elevated. Gastroscopy with multiple biopsies confirmed gastric adenocarcinoma. An abdominal CT scan revealed enlarged retroperitoneal lymph nodes. SK withdrew after total gastrectomy and commencement of chemotherapy. The Leser-Thrélat sign was named after two surgeons, Edmund Leser and Ulysse Trélat, who described the eruption of cutaneous lesions in patients with cancer (3). However, the correlation between multiple SK and internal malignancy was described by Hollander in 1900 (4). Acute eruption of SK has also been reported in some other cases, such as benign tumors, pregnancy, human immunodeficiency virus infections, use of adalimumab, and others, which indicates that the Leser-Trélat sign is not highly specific (5). It is also somewhat controversial whether a sudden appearance of SK can be considered a marker for internal malignancy, since both SK and malignancies occur more frequently in the elderly population, thus allowing for a higher likelihood of coincidence (6). However, the patient in this case was young and therefore less likely to suddenly develop such a large number of SK, which are more commonly seen after the age of 50 (7). Although the pathogenesis of Leser-Thrélat sign is not fully understood, there are data suggesting an association with tumor-secreting growth factors including epidermal growth factor and transforming growth factor-alpha, both of which can stimulate the epidermal growth factor receptor (8). Sudden appearance of eruptive SK is uncommon in young patie

Atopic dermatitis (AD) is a chronic, inflammatory, itchy dermatosis with periods of remissions and exacerbations. Social isolation and lockdown measures may cause increased stress which in turn may affect the skin condition of patients with AD. We aimed to investigate the impact of the COVID-19 pandemic on the course of AD and the mental health of adult patients with AD. The study was based on an anonymous online questionnaire. A total of 91 adult patients with AD participated in this survey. The study population consisted of 77 (84.6%) female and 14 (15.4%) male patients. The average age of patients was 28.3 years. Fifty-four respondents out of 91 (59.3%) noticed a worsening in the course of AD. Patients with worsened AD most often indicated exacerbating itching of the skin (92.6% of 54). Only 54 (59.3%) patients continued treatment as directed by the attending physician. Of those that did not, 13 (14.3%) took or applied fewer medications and 24 (26.4%) stopped taking or applying medications altogether. Of all respondents, 60 (65.9%) believed that their mental health had deteriorated and 11 (12.1%) patients developed suicidal thoughts during the COVID-19 pandemic. The results indicate that the COVID-19 pandemic had a negative impact on the course of AD among adult patients. Forced life changes, increased stress, and poor adherence to treatment may have been contributing factors. Increased stress may have also worsened the mental health of patients with AD, which in turn may have exacerbated AD.

Ashy dermatosis, or erythema dyschromicum perstans, is characterized by acquired grey patches distributed on the face, neck, trunk, and extremities with an unknown pathophysiology. Herein, we report a case of ashy dermatosis in a two-year-old child, possibly caused by an infection, with eventual improvement within two years, absent any treatment. To our knowledge, this is the second report of ashy dermatosis in a patient under the age of three years, and the first under the age of two years that was followed-up in the English-language literature from 2000 to 2021. Although the eruptions showed eventual improvement without any treatment in our case, all cases do not improve spontaneously. Further research is necessary to differentiate cases that eventually improve from resistant ones and determine treatment options for resistant cases.

Psoriasis is a lifelong disease with a chronic relapsing course, and treatment agent switching is a common and accepted practice in cases of primary or secondary inadequate response. Patients with prior biologic treatment failure or loss of response are a subset of individuals who most likely have more severe disease with a greater impact on quality of life. Additionally, switching between multiple biologics is associated with clinical consequences (e.g. development of anti-drug antibodies), which may limit efficacy of subsequent biologic therapies (1,2). Adalimumab and brodalumab were both shown to be highly specific and efficient while sparing the cumulative toxicity observed in conventional anti-psoriatic drugs use. An observational study on 5 patients suffering from severe plaque psoriasis provided a successful pattern on double-switching of the aforementioned biologic agents. Therapy was initiated with brodalumab, and after secondary failure or occurrence of arthritic comorbidities, a short washout period with adalimumab followed without striking results. Re-administered brodalumab showed retrieval of initial therapeutic efficacy on both skin and joints. Patients provided written informed consent. Five Caucasian patients (mean age 53.2 years), with severe plaque psoriasis (mean baseline Psoriasis Area and Severity Index (PASI) score 18.58) and seriously affected Dermatology Life Quality Index (DLQI) (median score 19.3) underwent brodalumab treatment with excellent response. However, a secondary loss of efficacy occurred within an average period of 23 months, with intense arthritic implication in three patients. A switch to adalimumab followed, which lasted 4.2 months on average. Due to inadequate response, treatment with brodalumab was resumed (Figure 1). Resumption of treatment with brodalumab, after a rather short ''washout'' period with adalimumab, led to immediate remission of psoriasis, reducing median PASI and DLQI scores to 1.84 and 2.3 respectively, while still maintaining this effectiveness for an average of 8.8 months (follow-up timepoint). The clinical course over time for patient 2 is presented from the start of brodalumab treatment to the first and second relapse before completing and maintaining treatment with this biologic agent (Figure 2). Interestingly, at the same time, comorbidities in 3 of 5 patients with the axial psoriatic arthritis type that had arisen earlier subsided with remarkable clinical amelioration. Switch therapy in patients with severe psoriasis is a common clinical practice, due to the chronic and unpredictable course of the disease, both in nonresponsive or relapsing cases, and few studies have assessed the efficacy of a second line biologic treatment in this population. A real-life, multicenter, prospective study in Italy supported the switch from anti-IL 17 to adalimumab or ustekinumab as a safe and effective therapeutic strategy, but there was a gap for patients with loss of efficacy after failure

Primary cutaneous apocrine carcinoma (PCAC), a subtype of sweat gland carcinoma, is an extremely rare malignant neoplasm. Distinguishing an apocrine carcinoma from a breast carcinoma metastasis is difficult even for a pathologist. Most arise in regions of high apocrine gland density like the axilla, and rarely on the scalp and eyelid, but they can occur elsewhere on the skin. Primary cutaneous apocrine carcinoma of the scalp is a rare malignancy most often reported in the literature as case reports or small case series. The giant form of primary cutaneous apocrine carcinoma in the frontal region has not been described in the literature, to the best of our knowledge. There are no established protocols for treatment of primary cutaneous apocrine carcinoma. We report a case of a giant primary cutaneous apocrine carcinoma localized in the frontal region. A definitive diagnosis of a primary cutaneous apocrine carcinoma was established by biopsy with microscopic and immunohistochemical analysis. Wide surgical excision and reconstruction with large local transposition flap and split thickness skin grafts for secondary defect were our therapy of choice. Primary cutaneous apocrine carcinoma is a very rare malignancy, and the giant form has not yet been described. Surgical treatment provided the patient with tumor-free status as well as satisfactory aesthetical appearance and quality of life.

Dermatitis artefacta (DA) is a psycho-dermatologic condition based on patients' behavioral patterns, characterized by an intentional production of cutaneous lesions on their own skin. The clinical presentation can be highly variable. Patients with DA seldom seek psychological support or psychiatric consultation. More often, they seek help from their primary care physician or dermatologist. This review article aims to provide a practical guide for the diagnosis and management of AD and affected patients. A broad literature search was performed using the PubMed and Google Scholar electronic online databases, using key words "dermatitis artefacta", "diagnosis", "management", and "psychodermatology". The search was limited to English and Spanish language articles and was supplemented with themed books and book chapters. DA can occur in a variety of clinical presentations, and physicians should suspect DA in patients with a history of psychiatric disorders or extensive use of healthcare services. The ultimate goal of DA treatment may be a proper referral to mental health services. However, the prognosis is poor even when successful mental health referrals are achieved, with low recovery rates. A useful approach may include the suggestion that a mental health provider can help with the anxiety and the distress generated by the lesions: in this case in this case it will be crucial to discuss this with the mental health provider after obtaining informed consent from the patient. Considering the difficulty in promoting patients' adherence to treatment, the ideal setting for DA treatment is a psycho-dermatologic clinic, where both dermatologic and psychological interventions can be seamlessly integrated.

Hand, foot, and mouth disease (HFMD) is a relatively common mild viral infection that usually affects young children, mainly occurring during the late spring, early summer, and fall months. It is most commonly caused by members of the human enterovirus (HEV) genus. Recently, HFMD has received renewed attention because of evidence that this disease could have clinical, epidemiological, and etiological characteristics different from those initially associated with it. HFMD may be associated with neurologic or cardiopulmonary complications and can, rarely, lead to death. Our study was a retrospective analysis on 83 children (<18 years of age) who were clinically diagnosed with HFMD at the Department of Dermatology of the Institute for Child and Youth Health Care of Vojvodina, in a single, tertiary-care university hospital in Novi Sad, Vojvodina province, Serbia, for the time period from January 2016 to December 2017. During the study period, HFMD was diagnosed in 83 children. Our results suggest that the outbreak of HFMD occurred in younger children (average age 3.10 years), who seem to be the most susceptible age group for HFMD infection. Taking into account that the diagnosis of HFMD is usually clinical, we believe that it is important for health professionals to be well-informed about the clinical features and the course of the disease. Good personal hygiene and the implementation of a surveillance system can help stop the spread of the disease and prevent outbreaks.

Erythema multiforme (EM) is an immune-mediated, mucocutaneous hypersensitivity syndrome that can occur as a result of various medications, including a wide range of antineoplastic and hormonal drugs. Anastrozole, a nonselective aromatase inhibitor used in breast cancer management has been associated with different cutaneous side effects, of which EM is rarely seen and usually in a minor or major form with typical target lesions. This is a short report of a patient who developed a rare cutaneous side effect after the use of aromatase inhibitor anastrozole - segmental erythema multiforme in cancer-affected area. Cutaneous adverse effects limited to cancer-affected breast are extremely rare but should be considered in everyday dermatological practice. We find this case instructive not only because of the rarity of the segmental EM, but also because, contrary to classical teaching, drug eruption due to anastrozole occurred months, not days after the initiation of therapy.

The aim of the study was to investigate the impact of atopic dermatitis (AD) in children and corticophobia on the quality of family life. Children with AD and their parents were included in a cross-sectional study. The severity of AD was self-assessed using the Patient Oriented-Scoring of Atopic Dermatitis (PO-SCORAD) index, and the severity of corticophobia using the Topical Corticosteroid Phobia (TOPICOP) score, and the general impact of AD on family quality of life using the Family Dermatology Life Quality Index (FDLQI). We included 330 parents, mostly mothers (99.4%) and children with a median age of 3 years (interquartile range, IQR 1.5-5.0 years). The median values of the PO SCORAD index and TOPICOP score were: 19.1 (IQR 13.6-24.1) and 58.3 (IQR 41.7-72.2), respectively. The median FDQLI score was 12 (IQR 7-16). The influence of independent variables such as parental age, child's age, child's gender, family history of allergies, place of residence, parental education, associated allergic disease in the child, PO SCORAD, and the TOPICOP score on the FDLQI was analysed. The significant models were the age of the parents (protective factor), the PO SCORAD index, and the TOPICOP score, which together accounted for 26.1% of the variability of FDLQI. Concusion of the study is that AD in children, its severity, and the parent's fear of chronic corticosteroid treatment impair the quality of family life.