Search

Adult T-cell leukemia/lymphoma (ATLL) is a peripheral T-cell neoplasm caused by the human T-cell leukemia virus type 1 (HTLV-1). It is characterized by a short survival time and lack of response to chemotherapy. We report a case of a 56-year-old woman, from the Brazilian northeast state of Bahia, who presented with a 2-month history of infiltrated papular and nodular skin lesions, especially on her forehead and also on her lower and upper limbs. Laboratory tests revealed positive serology for HTLV-1. Histopathological findings and the immunohistochemical profile confirmed the diagnosis of adult T-cell lymphoma/leukemia. A month after the diagnosis, the patient presented acute fatigue and pronounced paleness of the skin, dying of septic shock after her first chemotherapy cycle. The aim of this case report was to emphasize the importance of maintaining high clinical suspicion for ATLL, considering the epidemiological profile of the disease, especially for patients whose only early symptoms are cutaneous lesions.

The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) represents a new public health problem, with a total of 10.577.263 documented COVID-19 cases worldwide and 513.441 deaths up to the present date. Few cases of disease-related cutaneous manifestations have been reported in the literature, and such manifestations are scarce. Integumentary manifestations from COVID-19 include exanthemas and papular dermatoses, urticarial eruptions, atopic dermatitis, vesiculobullous lesions and skin signs of hypercoagulable states, such as acral ischaemia, livedo and retiform purpura. Most common extracutaneous manifestations from the disease include headache, cough, anosmia, ageusia, fever, dyspnoea, nausea, diarrhoea and cardiovascular events. The objectives of this review were to discuss the role of human cell receptors described as interaction targets of SARS-CoV-2, as well to understand the current state of knowledge on skin expression of these receptors, in order to substantiate future research. The authors present a thorough literature review on SARS-CoV-2 and its possible interaction with cell receptors and human tissues including the skin. They discuss a molecular hypothesis to explain the lower prevalence of dermatological manifestations from direct SARS-CoV-2 infection. Distinct human cell receptors binding the virus appear to be less expressed in the skin compared to other organs. Additionally, the presence of resolvins and the disintegrin metalloprotease ADAM17 provide a putative protection to the skin, explaining the majority of COVID-19 manifestations to be extracutaneous. This review represents an excellent opportunity for future studies using skin biopsies from COVID-19 patients to investigate molecular expression in the pathophysiology of cutaneous manifestations of the disease., (© 2020 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Background: Chronic urticaria has an expressive prevalence in general population, especially in adults, and is defined by the presence of intermittent hives for six weeks or longer. Our study aims to characterize the histological patterns of chronic spontaneous urticaria, based on the inflammatory cell infiltrate, and correlate them to laboratory exams., Methods: It was performed a retrospective analysis of laboratory, histopathology and direct immunofluorescence data of 93 patients with chronic urticaria. For histopathological analysis, cell count was performed in four fields at high magnification (×400) for each specimen. The resulting cell count medians were submitted to statistical analysis and, then, were correlated to laboratorial findings., Results: We found a female predominance (76.34%) of chronic urticaria cases, and an average age of 42.5 years (SD ± 15). Two histological groups were distinctive: 1) chronic urticaria with predominance of neutrophils or eosinophils - N (%) = 39 (42.4%) - and 2) chronic urticaria with predominance of lymphocytes - N (%) = 53 (57.6%). There was not significant correlation between histological groups and laboratorial tests. Moreover, direct immunofluorescence was positive in 21 (33,87%) from 62 patients., Conclusions: There is not enough scientific evidence to support neutrophilic urticaria as a solid, separate entity., (Copyright © 2017 Japanese Society of Allergology. Production and hosting by Elsevier B.V. All rights reserved.)

Background: Coagulation disorders contribute to the development of livedoid vasculopathy (LV). Elevated plasma levels of lipoprotein(a) [Lp(a)] are an independent risk factor for the development of cardiovascular disease and associated with hypercoagulable states. Increased serum Lp(a) levels have been reported in patients with LV and may have an important role in the pathogenesis of LV., Objectives: To investigate Lp(a) expression in skin lesions and circulating serum Lp(a) levels in patients with LV., Methods: Skin biopsy samples from 38 patients (27 women and 11 men) with active lesions diagnosed as LV and 9 samples of normal skin (5 women and 4 men) from control patients without LV were evaluated for skin expression of Lp(a) by immunohistochemistry. Plasma levels of Lp(a) were analyzed by immunoturbidimetry., Results: We found that lesional skin in patients with LV expressed 10-fold higher Lp(a) immunostaining than controls. High plasma levels of Lp(a) were observed in LV patients. We did not find a correlation (P = .02) between expression of Lp(a) in the skin and plasma levels of Lp(a) in patients with LV., Conclusions: Increased Lp(a) expression in lesional skin of LV patients suggests the role of Lp(a) in the thrombo-occlusive vasculopathy observed in this disease., (© 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Pityriasis versicolor is a superficial fungal infection caused by Malassezia spp. The aim of this study is to propose the definition of a new clinical entity: the recurrent and disseminated pityriasis versicolor (RDPV). All patients with RDPV were enrolled over an eight-month period. Clinical and epidemiological data were obtained, Malassezia (M.) species were isolated in cultures and identified by phenotypic and molecular characterization, skin biopsies were taken from active lesions, serum levels of immunoglobulin E were obtained and therapeutic schemes were evaluated. A total of 16 patients were included (11 male, 5 female). The most frequently isolated species were M. japonica (n = 3) and M. furfur (n = 3). This is the first study that isolates M. japonica in patients with pityriasis versicolor; interestingly, those were recalcitrant patients. Seven patients (43.8%) had no cure with any of the proposed treatments; among those, 5 (71.4%) had increased serum IgE levels. The most effective treatment was itraconazole 200 mg daily for 28 days. The RDPV has very different features from the classic form, including a poor response to treatment, and the isolation of different Malassezia species; therefore, we propose a hypothesis for the definition of a new clinical condition (RDPV), which could be a result of the interaction Malassezia-host., (Copyright © 2017 Elsevier Ltd. All rights reserved.)

Plasmacytoid dendritic cells (pDCs) are characterized by expression of CD123 and BDCA-2 (Blood Dendritic Cell Antigen 2) (CD303) molecules, which are important in innate and adaptive immunity. Chromoblastomycosis (CBM), lacaziosis or Jorge Lobo's disease (JLD), and paracoccidioidomycosis (PCM), are noteworthy in Latin America due to the large number of reported cases. The severity of lesions is mainly determined by the host's immune status and in situ responses. The dendritic cells studied in these fungal diseases are of myeloid origin, such as Langerhans cells and dermal dendrocytes; to our knowledge, there are no data for pDCs. Forty-three biopsies from patients with CBM, 42 from those with JLD and 46 diagnosed with PCM, were evaluated by immunohistochemistry. Plasmacytoid cells immunostained with anti-CD123 and anti-CD303 were detected in 16 cases of CBM; in those stained with anti-CD123, 24 specimens were obtained from PCM. We did not detect the presence of pDCs in any specimen using either antibody in JLD. We believe that, albeit a secondary immune response in PCM and CBM, pDCs could act as a secondary source of important cytokines. The BDCA-2 (CD303) is a c-type lectin receptor involved in cell adhesion, capture, and processing of antigens. Through the expression of the c-lectin receptor, there could be an interaction with fungi, similar to other receptors of this type, namely, CD207 in PCM and CD205 and CD209 in other fungal infections. In JLD, the absence of expression of CD123 and CD303 seems to indicate that pDCs are not involved in the immune response.

Adult-onset Still's disease (AOSD) patients typically present with arthralgia, fever, lymphadenopathy and a transient salmon maculopapular rash. Only approximately 25 cases of AOSD with urticaria were described in the literature. In this article, the authors report three additional cases of AOSD with urticarial and dermographic lesions who had a good clinical response to glucocorticoid and antihistamines. A review of the literature concerning this issue is also herein written.

Background: Few studies have evaluated the ultrastructure of the superficial skin nerves in urticaria., Objective: The objective of this study was to describe findings in superficial skin nerves in cases of drug-induced acute urticaria., Methods: Seven patients with drug-induced acute urticaria were included in the study. Skin biopsies were obtained from the urticarial lesion and from the apparently normal skin. The 14 fragments collected were processed for immunogold electron microscopy using single stains for antitryptase and anti-FXIIIa antibodies, as well as double immunogold labeling for both., Results: Some sections showed mast cells in the process of degranulation. Following double immunogold staining, 10 nm (FXIIIa) and 15 nm (Tryptase) gold particles were found together throughout the granules in mast cells, indicating that tryptase and FXIIIa are located inside each one of the granules of these cells. Interestingly, we found strong evidence of the presence of tryptase and factor XIIIa in the superficial skin nerves of these patients, both in cases of urticarial lesions (wheals) and in the apparently normal skin., Conclusions: Tryptase and FXIIIa are present in the superficial nerves of the skin in drug-induced acute urticaria. This is the first report of tryptase and FXIIIa expression in the superficial skin nerves of patients with urticaria. Tryptase may be participating in neural activation in these patients, while FXIIIa may be present in the nerves to guarantee the functional integrity of structures.

Syringomas are benign adnexal tumors derived from the intraepidermal portion of eccrine sweat ducts. Clinically, they usually appear as small yellow or skin-colored papules ranging from 1-3 mm in diameter on the lower eyelids. The condition of eruptive syringomas is a clinically distinct, uncommon variant of syringomas. This entity was first recognized by Darier and Jacquet in 1887. Since the initial description, less than 100 cases have been reported to date. Although eruptive syringomas can develop on various sites of the body, instances of widespread involvement, known as generalized eruptive syringoma, are rare. In this paper, we present a case of generalized eruptive syringoma on a 26-year-woman. She exhibited multiple, eruptive asymptomatic papules, symmetrically distributed on the face, scalp, upper and lower extremities, chest, and abdomen.

We present a 57-year-old woman with cutaneous lupus erythematosus (CLE), initially treated as acne. She noted blemishes, including nodules and facial swelling for nine months associated with discrete itching of the ears. Examination showed multiple malar nodules, comedones, pustules, atrophic scars, and hyperpigmentation. A biopsy was performed and revealed atrophic epidermis, discrete hyperkeratosis, vacuolar degeneration of basal layer, basal membrane zone with upper dermal lymphohistiocytic inflammatory infiltrate and deep perivascular and peri-adenexal lymphocytes, vascular ectasia, and mucin deposits. The acneiform presentation of CLE is commonly underdiagnosed due to the similarity with inflammatory acne. Histopathologic diagnostic in acneiform lupus is of extreme importance. This case emphasizes the relevance of knowing the notable variety of presentations of CLE and considering this diagnosis.

Summary: The SARS‐Cov‐2 is a single‐stranded RNA virus composed of 16 non‐structural proteins (NSP 1‐16) with specific roles in the replication of coronaviruses. NSP3 has the property to block host innate immune response and to promote cytokine expression. NSP5 can inhibit interferon (IFN) signalling and NSP16 prevents MAD5 recognition, depressing the innate immunity. Dendritic cells, monocytes, and macrophages are the first cell lineage against viruses' infections. The IFN type I is the danger signal for the human body during this clinical setting. Protective immune responses to viral infection are initiated by innate immune sensors that survey extracellular and intracellular space for foreign nucleic acids. In Covid‐19 the pathogenesis is not yet fully understood, but viral and host factors seem to play a key role. Important points in severe Covid‐19 are characterized by an upregulated innate immune response, hypercoagulopathy state, pulmonary tissue damage, neurological and/or gastrointestinal tract involvement, and fatal outcome in severe cases of macrophage activation syndrome, which produce a 'cytokine storm'. These systemic conditions share polymorphous cutaneous lesions where innate immune system is involved in the histopathological findings with acute respiratory distress syndrome, hypercoagulability, hyperferritinemia, increased serum levels of D‐dimer, lactic dehydrogenase, reactive‐C‐protein and serum A amyloid. It is described that several polymorphous cutaneous lesions similar to erythema pernio, urticarial rashes, diffuse or disseminated erythema, livedo racemosa, blue toe syndrome, retiform purpura, vesicles lesions, and purpuric exanthema or exanthema with clinical aspects of symmetrical drug‐related intertriginous and flexural exanthema. This review describes the complexity of Covid‐19, its pathophysiological and clinical aspects. [ABSTRACT FROM AUTHOR]