Resumo em Inglês:

Abstract: Inflammasomes are intracellular multiprotein complexes that comprise part of the innate immune response. Since their definition, inflammasome disorders have been linked to an increasing number of diseases. Autoinflammatory diseases refer to disorders in which local factors lead to the activation of innate immune cells, causing tissue damage when in the absence of autoantigens and autoantibodies. Skin symptoms include the main features of monogenic inflammasomopathies, such as Cryopyrin-Associated Periodic Syndromes (CAPS), Familial Mediterranean Fever (FMF), Schnitzler Syndrome, Hyper-IgD Syndrome (HIDS), PAPA Syndrome, and Deficiency of IL-1 Receptor Antagonist (DIRA). Concepts from other pathologies have also been reviewed in recent years, such as psoriasis, after the recognition of a combined contribution of innate and adaptive immunity in its pathogenesis. Inflammasomes are also involved in the response to various infections, malignancies, such as melanoma, autoimmune diseases, including vitiligo and lupus erythematosus, atopic and contact dermatitis, acne, hidradenitis suppurativa, among others. Inhibition of the inflammasome pathway may be a target for future therapies, as already occurs in the handling of CAPS, through the introduction of IL-1 inhibitors. This study presents a literature review focusing on the participation of inflammasomes in skin diseases.

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Abstract: Background: C-reactive protein is an inflammatory biomarker and its level increases in the serum of psoriatic patients. Its level is also associated with Psoriasis Area and Severity Index score. Objective: The aim of this study was to assess the decrement of serum C-reactive protein level with narrow-band ultraviolet B (NB-UVB) therapy. Methods: C-reactive protein serum levels in psoriasis patients were measured before and after treatment with NB-UVB and the data were analyzed in relation to the Psoriasis Area and Severity Index score improvement. Results: Baseline C-reactive protein levels among psoriatic patients were higher than normal. These levels decreased significantly after treatment (P<0.001). At the beginning of the study, patients with higher levels of C-reactive protein also had more extensive and severe skin involvement. The highest decrease in C-reactive protein was observed in patients who responded better to the treatment and achieved a higher Psoriasis Area and Severity Index 75%. There was an association between baseline Psoriasis Area and Severity Index scores and C-reactive protein levels. Conclusion: Patients with moderate to severe plaque-type psoriasis had active systemic inflammation, which was demonstrated by increased levels of C-reactive protein. Furthermore, skin disease severity was correlated with C-reactive protein levels. Phototherapy healed the psoriatic skin lesions and reduced inflammation, while decreasing C-reactive protein levels.

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Abstract: Background: Pigmented purpuric dermatosis is a chronic skin disorder of unknown aetiology characterised by symmetrical petechial and pigmented macules, often confined to the lower limbs. The aetiology of pigmented purpuric dermatosis is unknown. Dermatoscopy is a non-invasive diagnostic technique that allows the visualisation of morphological features invisible to the naked eye; it combines a method that renders the corneal layer of the skin translucent with an optical system that magnifies the image projected onto the retina. Objectives: The aim of this study is to investigate the dermatoscopic findings of pigmented purpuric dermatosis. Methods: This study enrolled patients diagnosed histopathologically with pigmented purpuric dermatosis who had dermatoscopic records. We reviewed the dermatoscopic images of PPD patients who attended the outpatient clinic in the Istanbul Dermatovenereology Department at the Bezmialem Vakıf University Medical Faculty. Results: Dermatoscopy showed: coppery-red pigmentation (97%, n = 31) in the background, a brown network (34%, n = 11), linear vessels (22%, n = 7), round to oval red dots, globules, and patches (69%, n = 22; 75%, n = 24; 34%, n = 11; respectively), brown globules (26%, n = 8) and dots (53%, n = 17), linear brown lines (22%, n = 7), and follicular openings (13%, n = 4). Conclusion: To our knowledge, this is the first study to report the dermatoscopy of pigmented purpuric dermatosis. In our opinion, dermatoscopy can be useful in the diagnosis of pigmented purpuric dermatosis.

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Abstract: Background: The morphological similarities between fibrous papules of the face and multiple sporadic oral fibromas were mentioned long ago and a relationship between them has been reported in the literature. Objective: The aim of this study was to evaluate the participation of mast cells, elastin and collagen in a series of oral fibromas and fibrous papules of the face in order to better understand the possible role of these factors in fibrosis and the formation of these lesions. Methods: Thirty cases of oral fibroma involving the buccal mucosa and 30 cases of fibrous papules of the face were selected. Tissue samples were submitted to picrosirius red staining and immunohistochemistry using anti-elastin and anti-tryptase antibodies. Results: The percentage of tryptase-positive mast cells and expression of elastin were higher in cases of fibrous papules of the face (p < 0.05). In contrast, a higher intensity of collagen deposition was observed in oral fibromas. The results showed mast cell accumulation and higher elastin synthesis in fibrous papules of the face, and mast cell accumulation with higher collagen fiber synthesis in oral fibromas. Conclusion: These findings support the hypothesis that mast cells influence the development and growth of these lesions through different mechanisms.

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Abstract: Background: Heparanase is an enzyme that cleaves heparan sulfate chains. Oligosaccharides generated by heparanase induce tumor progression. Basal cell carcinoma and squamous cell carcinoma comprise types of nonmelanoma skin cancer. Objectives: Evaluate the glycosaminoglycans profile and expression of heparanase in two human cell lines established in culture, immortalized skin keratinocyte (HaCaT) and squamous cell carcinoma (A431) and also investigate the expression of heparanase in basal cell carcinoma, squamous cell carcinoma and eyelid skin of individuals not affected by the disease (control). Methods: Glycosaminoglycans were quantified by electrophoresis and indirect ELISA method. The heparanase expression was analyzed by quantitative RT-PCR (qRTPCR). Results: The A431 strain showed significant increase in the sulfated glycosaminoglycans, increased heparanase expression and decreased hyaluronic acid, comparing to the HaCaT lineage. The mRNA expression of heparanase was significantly higher in Basal cell carcinoma and squamous cell carcinoma compared with control skin samples. It was also observed increased heparanase expression in squamous cell carcinoma compared to the Basal cell carcinoma. Conclusion: The glycosaminoglycans profile, as well as heparanase expression are different between HaCaT and A431 cell lines. The increased expression of heparanase in Basal cell carcinoma and squamous cell carcinoma suggests that this enzyme could be a marker for the diagnosis of such types of non-melanoma cancers, and may be useful as a target molecule for future alternative treatment.

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Abstract: Background: Bullous pemphigoid (BP) is a chronic, autoimmune blistering skin disease that affects patients' daily life and psychosocial well-being. Objective: The aim of the study was to evaluate the quality of life, anxiety, depression and loneliness in BP patients. Methods: Fifty-seven BP patients and fifty-seven healthy controls were recruited for the study. The quality of life of each patient was assessed using the Dermatology Life Quality Index (DLQI) scale. Moreover, they were evaluated for anxiety and depression according to the Hospital Anxiety Depression Scale (HADS-scale), while loneliness was measured through the Loneliness Scale-Version 3 (UCLA) scale. Results: The mean DLQI score was 9.45±3.34. Statistically significant differences on the HADS total scale and in HADS-depression subscale (p=0.015 and p=0.002, respectively) were documented. No statistically significant difference was found between the two groups on the HADS-anxiety subscale. Furthermore, significantly higher scores were recorded on the UCLA Scale compared with healthy volunteers (p=0.003). Conclusion: BP had a significant impact on quality of life and the psychological status of patients, probably due to the appearance of unattractive lesions on the skin, functional problems and disease chronicity.

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Abstract: Background: Topical antimicrobial drugs are indicated for limited superficial pyodermitis treatment, although they are largely used as self-prescribed medication for a variety of inflammatory dermatoses, including atopic dermatitis. Monitoring bacterial susceptibility to these drugs is difficult, given the paucity of laboratory standardization. Objective: To evaluate the prevalence of Staphylococcus aureus topical antimicrobial drug resistance in atopic dermatitis patients. Methods: We conducted a cross-sectional study of children and adults diagnosed with atopic dermatitis and S. aureus colonization. We used miscellaneous literature reported breakpoints to define S. aureus resistance to mupirocin, fusidic acid, gentamicin, neomycin and bacitracin. Results: A total of 91 patients were included and 100 S. aureus isolates were analyzed. All strains were methicillin-susceptible S. aureus. We found a low prevalence of mupirocin and fusidic acid resistance (1.1% and 5.9%, respectively), but high levels of neomycin and bacitracin resistance (42.6% and 100%, respectively). Fusidic acid resistance was associated with more severe atopic dermatitis, demonstrated by higher EASI scores (median 17.8 vs 5.7, p=.009). Our results also corroborate the literature on the absence of cross-resistance between the aminoglycosides neomycin and gentamicin. Conclusions: Our data, in a southern Brazilian sample of AD patients, revealed a low prevalence of mupirocin and fusidic acid resistance of S. aureus atopic eczema colonizer strains. However, for neomycin and bacitracin, which are commonly used topical antimicrobial drugs in Brazil, high levels of resistance were identified. Further restrictions on the use of these antimicrobials seem necessary to keep resistance as low as possible.

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Abstract: Background: Psoriasis is a multigenic and multifactorial dermatological disease linked to cardiovascular diseases. Increased levels of homocysteine in patients with psoriasis have been demonstrated in many studies. The most frequently investigated genetic defect that plays a role in homocysteine metabolism is single point substitution (C to T) located on the 677th nucleotide of the methylenetetrahydrofolate reductase gene (MTHFR). Objective: In this study, we aimed to investigate methylenetetrahydrofolate C677T polymorphism in psoriasis patients in Turkey. Methods: The study included 96 patients with psoriasis and 77 controls from southern Turkey. Methylenetetrahydrofolate C677T polymorphism was analysed using the Polymerase Chain Reaction-Restriction Fragment Length Polymorphism methods. Results: In the psoriasis group, 34 CC (35.4%), 46 CT (47.9%) and 16 TT (16.7%) genotypes were found, respectively; while in the control group, the figures were 39 (50.6%), 35 (45.5%), 3 (3.9%). Homozygote and heterozygote T alleles of methylenetetrahydrofolate C677T polymorphism were significantly higher in the psoriasis than in the control group (p=0.013). Conclusion: We firstly found a correlation between methylenetetrahydrofolate C677T polymorphism and psoriasis among the southern Turkish population.

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Abstract: Regeneration and tissue repair processes consist of a sequence of molecular and cellular events which occur after the onset of a tissue lesion in order to restore the damaged tissue. The exsudative, proliferative, and extracellular matrix remodeling phases are sequential events that occur through the integration of dynamic processes involving soluble mediators, blood cells, and parenchymal cells. Exsudative phenomena that take place after injury contribute to the development of tissue edema. The proliferative stage seeks to reduce the area of tissue injury by contracting myofibroblasts and fibroplasia. At this stage, angiogenesis and reepithelialization processes can still be observed. Endothelial cells are able to differentiate into mesenchymal components, and this difference appears to be finely orchestrated by a set of signaling proteins that have been studied in the literature. This pathway is known as Hedgehog. The purpose of this review is to describe the various cellular and molecular aspects involved in the skin healing process.

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Abstract: Mohs micrographic surgery is a technique used to excise skin tumors based on comprehensive surgical mapping, in which the surgeon removes the tumor, followed by a complete histological evaluation of the tumor's margins. The correlation of the presence of a tumor in histological examinations and its precise location on the surgical map result in a complete removal of the tumor with maximum normal tissue preservation. The present article seeks to provide general practitioners and healthcare specialists with guidelines regarding recommendations for Mohs micrographic surgery to treat skin tumors, based on the most reliable evidence available in medical literature on the subject. This bibliographic review of scientific articles in this line of research was conducted based on data collected from MEDLINE/PubMed. The search strategy used in this study was based on structured questions in the Patient, Intervention, Control, and Outcome (PICO) format. MeSH terms were used as descriptors. The indications of this technique are related to recurrence, histology, size, definition of tumor margins, and location of tumors. These guidelines attempt to establish the indications of Mohs surgery for different types of skin tumors.

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Abstract: Discrepancies in the terminology of elementary lesions persist when texts from Dermatology and Semiology books are compared, which can cause some confusion in both the teaching of undergraduate medical students and the learning acquired by professionals in the field. This review aims to compare and clarify the differences in the description of elementary lesions by many authors, used as references for specialists in dermatology.

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Abstract: Rosai-Dorfman disease is a benign histiocytic proliferative disorder of unknown etiology. The disease mainly affects lymph node tissue, although it is rarely confined to the skin. Here, we describe a 53-year-old woman with purely cutaneous Rosai-Dorfman disease. The patient presented with a large pigmented plaque on her left leg, and sparse erythematous papules on her face and arms. A complete clinical response was achieved with thalidomide, followed by recurrence at the initial site one year later. The histological examination displayed the typical features of Rosai-Dorfman disease in the recent lesions but not in the older lesions. In the setting of no lymphadenopathy, the histopathological features of Rosai-Dorfman disease are commonly misinterpreted. Therefore, awareness of the histological aspects present at different stages, not always featuring the hallmark microscopic signs of Rosai-Dorfman disease, is particularly important for a correct diagnosis of this rare disorder.

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Abstract: A 54 year-old woman with a 3-year history of rheumatoid arthritis (RA) consulted us because of weight loss, fever and skin eruption. On physical examination, erythematous plaques with a pseudo-vesicular appearance were seen on the back of both shoulders. Histological examination was consistent with rheumatoid neutrophilic dermatosis (RND). After three days of prednisone treatment, the skin eruption resolved. RND is a rare cutaneous manifestation of seropositive RA, characterized by asymptomatic, symmetrical erythematous plaques with a pseudo-vesicular appearance. Histology characteristically reveals a dense, neutrophilic infiltrate with leucocitoclasis but without other signs of vasculitis. Lesions may resolve spontaneously or with RA treatment. This case illustrates an uncommon skin manifestation of active rheumatoid arthritis.

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Abstract: Sézary syndrome is a primary cutaneous T-cell lymphoma characterized by the triad of erythroderma, lymphadenopathy and circulating atypical cells. The emergence of new molecular targets has enabled the development of drugs such as alemtuzumab, an anti-CD52 monoclonal antibody, which has shown promising results in the treatment of this entity. We report the case of a 70-year-old male with refractory Sézary syndrome in whom treatment with alemtuzumab achieved an 80% skin lesion clearance with complete haematologic and radiologic response. The treatment was discontinued after 4 months due to adverse effects, with the patient showing a sustained response without disease progression after 13 months of follow-up.

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Abstract: Eosinophilic pustular folliculitis (EPF) or Ofuji disease is a rare dermatosis, prone to recurrence and chronicity. The peak incidence occurs in the third decade of life and its exact etiology remains unknown. Evidence suggests that the expression of adhesion molecules and the production of cytokines activate the follicular unit, but the stimulus that triggers these changes remains unclear. The three clinical variants reported in the literature include classic EPF, immunosuppression-associated EPF, and infancy-associated EPF. We report a case of eosinophilic pustular folliculitis with peculiar epidemiological characteristics, which represents a challenging therapeutic scenario.

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Abstract: Necrolytic acral erythema is a rare skin disease associated with hepatitis C virus infection. We report a case of a 31-year-old woman with hepatitis C virus infection and decreased zinc serum level. Physical examination revealed scaly, lichenified plaques, well-demarcated with an erythematous peripheral rim located on the lower limbs. After blood transfusion and oral zinc supplementation the patient presented an improvement of lesions.

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Abstract: Non-steroidal, anti-inflammatory drugs, followed by antibiotics, are the main causes of fixed drug eruption. They provoke one or several round erythematous or bullous lesions that recur in the same place after taking the causative medication. A positive patch test on residual, lesional skin can replace satisfactorily oral reintroduction. We describe the case of a 74-year-old woman with numerous, rounded, erythematous lesions on the trunk and recurrent blistering on the fifth right-hand finger, which developed a few hours after taking etoricoxib. Lesional patch testing with etoricoxib was positive and reproduced the typical pattern of a fixed drug eruption upon histopathology. We emphasize the specific reactivity of the etoricoxib patch test, and the capacity to reproduce the histologic pattern of the reaction.

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Abstract: Milia-like idiopathic calcinosis cutis (MICC) is a very rare dermatological disorder characterized by multiple whitish to skin colored, milia-like papules, mostly found on the hands. MICC can disappear spontaneously by adulthood; therefore, its early recognition is crucial to avoiding unnecessary interventions. Herein, we present a case of MICC in a 6-year-old girl with Down syndrome.

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Abstract: Blue nevi are benign melanocytic lesions located in the deeper reticular dermis, consequence of failure of melanocytic migration into the dermal-epidermal junction from the neural crest. Lesions are usually asymptomatic and solitary, but may present in a multiple or agminated (grouped) pattern. The agminated subtype is formed when bluish-pigmented lesions cluster together in a well-defined area. Lesions can be flat or raised. We report the case of a patient who presented multiple bluish macules (1-3 mm in diameter) grouped on the left upper back. Dermoscopy and anatomic pathological examination were consistent with blue nevus.

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Abstract: Primary localized cutaneous amyloidosis is a skin-limited amyloidosis that does not involve internal organs. It is clinically subclassified into 3 general categories and some rare variants. However, there is considerable overlap within the classification. Though there are a variety of therapeutic measures, the treatment is often unsatisfactory, particularly when the disease is severe and extensive. We describe a rare case of primary localized cutaneous amyloidosis with lichen and poikiloderma-like lesions that showed an excellent response to systemic acitretin.

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Abstract: Generalized pustular psoriasis, or psoriasis of von Zumbusch, is an acute and severe clinical form of psoriasis, which usually occurs in patients with psoriasis undergoing aggravating factors. In this work, we report the case of a female patient, 70 years old, who developed generalized pustular psoriasis symptoms while reducing the dose of oral corticosteroids, improperly introduced for the treatment of alleged acute generalized exanthematous pustulosis. The differential diagnosis of generalized pustular psoriasis should be made with other pustular dermatoses, such as subcorneal pustulosis, IgA pemphigus and especially with acute generalized exanthematous pustulosis. Personal history of psoriasis and histopathological findings with psoriasiform changes and subcorneal pustule favored the diagnosis. She was treated with acitretin 30 mg / day, progressing to complete regression of the lesions.

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Abstract: The Henoch-Schönlein purpura is the vasculitis associated with deposits of immunoglobulin A in small vessels. Its association with cytoplasmic antineutrophil cytoplasmic antibodies is possible, but rare. This vasculitis is uncommon in adults and the main clinic manifestations are purpuric lesions in lower limbs with gastrointestinal symptoms and renal involvement. The present work describes a rare case of Henoch-Schönlein purpura in an adult with cytoplasmic antineutrophil cytoplasmic antibodies.

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Abstract: Gastric syphilis is an uncommon extracutaneous manifestation of syphilis, occurring in less than 1% of patients, presenting nonspecific clinical manifestations. In general, it occurs on secondary stage. The critical point is the recognition of the syphilitic gastric involvement, without which there may be incorrect diagnosis of malignancy of the digestive tract. In this report, a case of secondary syphilis with gastric involvement that had complete remission with benzathine penicillin will be described.

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Abstract: In Brazil, leprosy is a widespread infectious and contagious disease. Clinicians and specialists view leprosy broadly as a systemic infection, since, in its manifestations, it mimics many conditions, such as rheumatic, vascular, ENT, neurological and dermatological diseases. There are few studies that characterize the factors associated with ulcers in leprosy. These injuries should be prevented and treated promptly to avoid serious problems like secondary infections, sepsis, carcinomatous degeneration and amputations. We describe a patient with ulcers on his legs, involving late diagnosis of lepromatous leprosy.

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Abstract: The aim of the study was to identify Bacillus species from the Demodex folliculorum of patients with topical steroidinduced facial rosaceiform dermatitis. Of the 75 patients examined, 20% had clinical spinulosis, while 18.66% had dermoscopic features of Demodex: follicular plugs and tails. Of the 17.33% positive patients identified upon microscopy for Demodex, samples for bacterial culture were plated on trypticase soy Colombia agar. Identification was performed by microorganisms grown method mass spectrometry. We identified a strain of Bacillus cereus.

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Abstract: Chromoblastomycosis is a chronic fungal infection of the epidermis, dermis and subcutaneous tissue, in which the most common etiologic agent in Brazil is Fonsecaea pedrosoi. In more advanced cases we found many difficulties in their treatment, and therefore, we report a case of extensive and severe chromoblastomycosis, with therapeutical failure of first choice treatments, but good response to voriconazole.

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Abstract: Antisynthetase syndrome is a rare autoimmune disease characterized by interstitial lung disease and/or inflammatory myositis, with positive antisynthetase antibodies (anti-Jo-1, anti-PL-7, anti-PL-12, ZO, OJ, anti-KE or KS). Other symptoms described include: non-erosive arthritis, fever, Raynaud's phenomenon, and "mechanic's hands." The first therapeutic option is corticotherapy, followed by other immunosuppressants. The prognosis of the disease is quite limited when compared to other inflammatory myopathies with negative antisynthetase antibodies.

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Abstract: Due to mounting evidences of interaction between Hansen's bacilli with endothelial cells and the paucity of studies addressing the presence of nailfold capillaroscopic alterations in patients with Hansen's disease, a study was carried out in order to verify the presence of capillaroscopic alterations in patients with leprosy in its various forms and its correlation with clinical parameters. Ten patients were evaluated at a specialized university service. Sixty percent of those had some capillaroscopic change, such as micro-hemorrhages, ectatic, bushy and corkscrew capillaries. Such changes were unspecific, which suggests there is not a specific pattern for this disease.

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Abstract: Onychomycosis is the most prevalent onychopathy and it requires a correct early diagnosis. Currently, the diagnostic gold standard is the association of direct mycological test with culture; however, it shows variable sensitivity. The histopathological examination of the distal nail plate, called clipping, has shown to be an adjuvant in diagnosing onychomycosis. This is an easy-to-perform, relatively cheap examination that is little dependent of the examiner, rapidly provides results, has high sensitivity, and for patients it is painless and harmless.

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Abstract: Psoriasis is a chronic, inflammatory, immune-mediated disease affecting 1-3% of the population worldwide. This work seeks to draw a profile of patients with psoriasis, analyzing socioeconomic, anthropometric, and clinical aspects. For this, medical records from 81 individuals who received medical care in a university hospital in 2014 were consulted. It was observed that the patients were mostly dark-skinned black adult men, with a low education level and a low income, who were sedentary, former smokers, obese, with an increase in waist circumference, and who did not consume alcohol. Psoriasis vulgaris predominated, beginning mainly on the scalp, hands, and feet. In addition, many presented some type of associated comorbidity and had relatives with psoriasis.

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Abstract: Pityriasis amiantacea was first described in 1832. The disease may be secondary to any skin condition that primarily affects the scalp, including seborrheic dermatitis. Its pathogenesis remains uncertain. We aim to analyze the epidemiological and clinical profiles of patients with pityriasis amiantacea to better understand treatment responses. We identified seven cases of pityriasis amiantacea and a female predominance in a sample of 63 pediatric patients with seborrheic dermatitis followed for an average of 20.4 months. We reported a mean age of 5.9 years. Five patients were female, with a mean age of 9 years. All patients were successfully treated with topic ketoconazole.

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Abstract: Depressed facial scars are still a challenge in medical literature, despite the wide range of proposed treatments. Subcision is a technique that is frequently performed to improve this type of lesions. This article proposes a new method to release depressed scars, reported and named by the author as dermal tunneling. This study presents a simple and didactic manner to perform this method. The results in 17 patients with facial scars were considered promising. Thus, the technique was deemed to be safe and reproducible.