Human sporotrichosis: recommendations from the Brazilian Society of Dermatology for the clinical, diagnostic and therapeutic management

Abstract Background: The increase in the zoonotic epidemic of sporotrichosis caused by Sporothrix brasiliensis, which started in the late 1990s in Rio de Janeiro and is now found in almost all Brazilian states, has been equally advancing in neighboring countries of Brazil. Changes in the clinical-epidemiological profile, advances in the laboratory diagnosis of the disease, and therapeutic difficulties have been observed throughout these almost 25 years of the epidemic, although there is no national consensus. The last international guideline dates from 2007. Objectives: Update the clinical classification, diagnostic methods and recommendations on the therapeutic management of patients with sporotrichosis. Methods: Twelve experts in human sporotrichosis were selected from different Brazilian regions, and divided into three work groups: clinical, diagnosis and treatment. The bibliographic research was carried out on the EBSCOHost platform. Meetings took place via electronic mail and remote/face-to-face and hybrid settings, resulting in a questionnaire which pointed out 13 divergences, resolved based on the opinion of the majority of the participants. Results: The clinical classification and laboratory diagnosis were updated. Therapeutic recommendations were made for the different clinical forms. Conclusions: Publication of the first national recommendation, carried out by the Brazilian Society of Dermatology, aimed at the Brazilian scientific community, especially dermatologists, infectologists, pediatricians, family medicine personnel, and laboratory professionals who work in the management of human sporotrichosis.

The correct nomenclature of Zirelí sign in the propaedeutics of pityriasis versicolor (in memoriam)

Abstract Aiming at disclosing the semiotic method used in the diagnosis of pityriasis versicolor, the authors go through the history of the creation of Zirelí sign, describing the method, its usefulness and practicality in dermatological clinical practice, whether public or private, and to give credit to the author of this semiological maneuver, in memoriam.

Paracoccidioidomycosis due to Paracoccidioides brasiliensis S1 plus HIV co-infection

BACKGROUND Paracoccidioidomycosis (PCM) is one of the most important systemic mycoses in Latin America and the leading fungal cause of mortality in non-immunosuppressed individuals in Brazil. However, HIV/PCM co-infection can increase the clinical severity in these co-infected patients. This co-infection is rarely reported in the literature mainly because of the different epidemiological profiles of these infections. Furthermore, PCM is a neglected and non-notifiable disease, which may underestimate the real importance of this disease. The advent of molecular studies on the species of the genus Paracoccidioides has expanded the knowledge regarding the severity and the clinical spectrum in PCM. In this context, the development of studies to describe the association of the Paracoccidioides phylogenetic cryptic species in vulnerable populations, such as HIV-infected patients, appears relevant. OBJECTIVE To describe the clinical, epidemiological, therapeutic and prognostic aspects in HIV/PCM co-infected patients, along with the molecular identification of the Paracoccidioides species involved in these cases. METHODS The investigators performed a molecular and clinical retrospective study involving HIV/PCM co-infected patients, from a reference centre for PCM care in the endemic area of Rio de Janeiro, Brazil, from 1998 to 2015. Molecular identification of the fungal strains was done by amplification of partial sequences of arf and gp43 genes. FINDINGS Of 89 patients diagnosed with PCM by fungal isolation in the culture, a viable isolate was recovered for molecular analysis from 44 patients. Of these 44 patients, 28 (63.6%) had their serum samples submitted for enzyme immunoassay tests for screening of HIV antibodies, and 5 (17.9%) had a positive result. All cases were considered severe, with a variable clinical presentation, including mixed, acute/subacute clinical forms and a high rate of complications, requiring combination therapy. Paracoccidioides brasiliensis S1 was the species identified in all cases. CONCLUSIONS HIV/PCM co-infection can change the natural history of this fungal disease. The authors reinforce the need to include HIV screening diagnostic tests routinely for patients with PCM.

Fatal septic shock caused by Paracoccidioides brasiliensis phylogenetic species S1 in a young immunocompetent patient: a case report

Abstract The authors report the first case of fatal septic shock, a rare clinical presentation of paracoccidioidomycosis (PCM) caused by Paracoccidioides brasiliensis S1. We also provide an immunological evaluation of the patient. Severe clinical signs such as organ dysfunction and digital gangrene occurred in this case. The patient presented a remarkable cell activation profile and diminished percentage of peripheral blood T regulatory cells. A decrease in anti-inflammatory IL-1RA plasma level showed the potential for endothelium damage, probably contributing to a vasculitis process. Together with P. lutzii, P. brasiliensis appears to be involved in severe cases of PCM.

Sporotrichosis: an update on epidemiology, etiopathogenesis, laboratory and clinical therapeutics

Abstract: In the late 90's there was a change in both the route of transmission and the people at risk for sporotrichosis. This zoonotic cat-man alternative transmission route elicited changes in strategies to control the epidemic. There was a progressive increase in the number of cases involving especially children and the elderly. In addition to becoming hyperendemic, uncommon clinical pictures like immunoreactive clinical presentations or severe systemic cases have emerged. New species were identified and classified through molecular tools using more virulent clinical isolates, like S. brasiliensis, compared to the environmental isolates. Likewise, different species of Sporothrix have been associated with different geographic regions. The serological and molecular techniques are used as an auxiliary tool for the diagnosis and/or for species identification, although the isolation and the identification of Sporothrix spp. in clinical specimen is still the gold standard. Currently sporotrichosis epidemics requires the knowledge of the epidemiological-molecular profile to control the disease and the specific treatment. Itraconazole, potassium iodide, terfinafine, and amphotericin B are the available drugs in Brazil to treat sporotrichosis. The drug of choice, its posology, and treatment duration vary according to the clinical presentation, the Sporothrix species, and host immune status. New treatment choices, including a vaccine, are being developed; nevertheless, more clinical trials are required to confirm its efficacy.

Angiossarcoma do couro cabeludo: relato de caso / Angiosarcoma of the scalp: case report

O angiossarcoma é uma rara neoplasia vascular originada das células endoteliais. É muito agressivo com alta taxa de recorrência, metástases precoces e mau prognóstico. O diagnóstico precoce do angiossarcoma é um desafio, pois muitas vezes apresenta-se como uma equimose ou pápula violácea que pode ser confundida com lesão benigna como um hemangioma. O tamanho do tumor é um fator prognóstico importante e o diagnóstico precoce e tratamento combinado são fundamentais para aumentar a sobrevida desses pacientes. Esse estudo relata o caso de um homem de 58 anos de idade com angiossarcoma de couro cabeludo altamente agressivo e prognóstico reservado. (AU)

Angiosarcoma of the head and neck is a rare vascular sarcoma associated with high rates of local recurrence and distant metastasis and a poor prognosis. The diagnosis of cutaneous angiosarcoma can be challenging because it often presents insidiously as a bruiselike lesion or a purplish papule thatmay be mistaken for a benign lesion such as hemangioma. The tumor size is a important prognostic factor and a multimodality treatment approach is imperative for successful outcomes. This study reports the case of 58-years-man with angiosarcoma of the scalp aggressive and a poor prognosis. (AU)

Use of potassium iodide in Dermatology: updates on an old drug / Uso do iodeto de potássio na Dermatologia: considerações atuais de uma droga antiga

Potassium iodide, as a saturated solution, is a valuable drug in the dermatologist's therapeutic arsenal and is useful for the treatment of different diseases due to its immunomodulatory features. However, its prescription has become increasingly less frequent in dermatology practice. Little knowledge about its exact mechanism of action, lack of interest from the pharmaceutical industry, the advent of new drugs, and the toxicity caused by the use of high doses of the drug are some possible explanations for that. Consequently, there are few scientific studies on the pharmacological aspects, dosage and efficacy of this drug. Also, there is no conventional standard on how to manipulate and prescribe the saturated solution of potassium iodide, which leads to unawareness of the exact amount of the salt being delivered in grams to patients. Considering that dosage is directly related to toxicity and the immunomodulatory features of this drug, it is essential to define the amount to be prescribed and to reduce it to a minimum effective dose in order to minimize the risks of intolerance and thus improve treatment adherence. This review is relevant due to the fact that the saturated solution of potassium iodide is often the only therapeutic choice available for the treatment of some infectious, inflammatory and immune-mediated dermatoses, no matter whether the reason is specific indication, failure of a previous therapy or cost-effectiveness.

Iodeto de potássio, sob a forma de solução saturada, é um valioso medicamento no arsenal terapêutico do dermatologista. É usado há mais de um século e útil para doenças de fisiopatologias diversas em virtude de seu caráter imunomodulador. Prescrevê-lo, entretanto, tem se tornado cada vez menos frequente na prática dermatológica. O pouco conhecimento sobre seu exato mecanismo de ação, o desinteresse da indústria farmacêutica com o advento de novos fármacos, além da toxicidade do medicamento pelas altas doses utilizadas são algumas das possíveis justificativas. Dessa forma, os estudos científicos envolvendo seus aspectos farmacológicos, posológicos e de eficácia são relativamente raros. Consequentemente, não se convencionou uma padronização na forma de manipular e prescrever a solução saturada de iodeto de potássio, o que causa um verdadeiro desconhecimento da dose exata em gramas do sal que está sendo fornecida aos pacientes. Ao considerar que a dose está diretamente relacionada toxicidade e o conhecimento da característica imunomoduladora dessa droga, é importante definir a quantidade a ser fornecida, reduzindo-a até a dose mínima eficaz, de forma a diminuir a intolerância e melhorar a adesão ao tratamento. A relevância do tema se deve ao fato da solução saturada de iodeto de potássio ser, muitas vezes, a única escolha na terapêutica disponível para o tratamento de algumas dermatoses de origem infecciosa, inflamatória ou imunomediada, quer por indicação específica, por falha de outro medicamento ou por seu custo acessível.

Pityriasis amiantacea: clinical-dermatoscopic features and microscopy of hair tufts / Pitiríase amiantácea: aspectos clínicos e dermatoscópicos e microscopia dos tufos capilares

Pityriasis Amiantacea, also known as pseudotinea amiantacea, is a clinical syndrome which affects the scalp, having a reaction pattern reported to occur in several inflammatory dermatoses. The authors seek to establish the correlation between its clinical, dermoscopic and light microscopy features through the analysis of scale- encrusted hair tufts submitted to histological processing.

A pitiríase amiantácea, também chamada de pseudotínea amiantácea, é compreendida como uma síndrome clínica que acomete o couro cabeludo. Representa um padrão de reação a diversas dermatoses inflamatórias. Os autores procuram estabelecer a correlação entre características clínicas, dermatoscópicas e de microscopia ótica, através da análise do tufo de cabelos agregados pelas escamocrostas, submetido a processamento histológico.

Doença de Dowling-Degos: apresentação clínica e histopatológica clássica / Dowling-Degos disease: classic clinical and histopathological presentation

A doença de Dowling-Degos é uma genodermatose rara que consiste numa desordem pigmentar reticulada. Caracteriza-se pela presença de máculas hiperpigmentadas nas regiões flexurais com distribuição em rede; lesões tipo comedão no dorso e na região cervical; e cicatrizes cribriformes na face, particularmente periorais. Apresentamos um caso de um paciente de 51 anos, masculino, com lesões tipo macrocomedões, cicatrizes cribriformes, cistos e máculas hipercrômicas no dorso, tórax anterior, axilas, pescoço, região genital e face. Relatava ter dois filhos, três irmãos e o pai com quadro semelhante. As biópsias de pele foram características da doença de Dowling-Degos, mostrando dilatação folicular, epiderme digitiforme, com áreas de aspecto de "chifre de veado" e focos de hiperpigmentação da camada basal.

Dowling-Degos disease (DDD) is a rare genetic disease of the skin (reticulate pigmented anomaly), clinically characterized by flexural brown pigmented reticulate macules, comedo-like papules on the back, neck and pitted perioral or facial scars. We present the case of a 51 year-old man with macrocomedo-like lesions, pitted scars, cysts, hyperpigmented macules in his back, chest, axillae, neck, groin and face. The patient reported having two children, three brothers and a father with a similar condition. The histopathology of the skin biopsies was very characteristic of Dowling-Degos disease, showing dilated follicular, fingerlike projections called rete ridges (dermal pegs), with thinning of the suprapapillary plates, resulting in an "antler-like" pattern and increased pigmentation of the basal layer.

Dermatomiofibroma: relato de caso de doença rara / Dermatomyofibroma: a case report of a rare disease

O Dermatomiofibroma está incluído no grupo de lesões neoplásicas mesenquimais benignas de linhagem fibroblástica e miofibroblástica da pele. É uma doença rara, havendo aproximadamente 100 casos descritos na literatura mundial até o momento. Este artigo relata o caso de uma mulher jovem com apresentação clínica típica e diagnóstico histopatológico de dermatomiofibroma. Foram realizadas colorações especiais que mostraram preservação das fibras colágenas e a imunohistoquímica revelou positividade para vimentina e negatividade para actina e S100. Por se tratar de doença rara, os achados histopatológicos são de grande importância, mas a supeição clínica é possível em casos típicos como este.

Dermato myofibroma is included in the group of benign cutaneous mesenchymal neoplastic lesions of fibroblastic and myofibroblastic lineage. It's a rare disease and there are approximately only one hundred cases described worldwide in the medical literature up to now. The present study reports the case of a young woman with typical clinical cutaneous lesion and histopathological diagnosis of dermato myofibroma. Special stains were carried out which showed preserved collagen fibers and immunohistochemistry was positive for vimentin and negative for actin and S100. As it is a rare disease, the histopathological findings are of great importance but clinical suspicion is possible in typical cases such as this one.