Pioderma gangrenoso: atualização e orientação / Pyoderma gangrenosum: update and guidance

Introdução: O pioderma gangrenoso (PG) é uma doença neutrofílica, rara, porém de consequências danosas. O Capítulo de Feridas da Sociedade Brasileira de Cirurgia Plástica (SBCP) foi instado a compilar as melhores práticas, tanto diagnósticas como terapêuticas, junto às Sociedades Brasileiras de Dermatologia e Reumatologia para um melhor esclarecimento dos seus membros. Métodos: Ampla revisão de artigos publicados na literatura médica e compilação das novas diretrizes de diagnóstico e tratamento por dois membros indicados por cada uma das Sociedades Brasileiras de Cirurgia Plástica, Dermatologia e Reumatologia. Resultados: O PG deixou de ser uma doença de exclusão, tendo os critérios diagnósticos bem definidos e a orientação terapêutica delineada pelos autores, incluindo o uso de terapia biológica. Conclusão: O PG permanece desafiador, mas sistematizar a investigação e o uso dos novos medicamentos, bem como o manejo das feridas, abre novas perspectivas, interferindo na fisiopatologia de modo positivo, com maior precocidade e menos efeitos colaterais do que a terapia imunossupressora de forma isolada.

Introduction: The pyoderma gangrenosum (PG) is a neutrophilic disease, rare but with a poor outcome. The Capitulum of Wound treatment of the Brazilian Society of Plastic Surgery (SBCP) promoted a discussion with the Brazilian societies of Dermatology and Rheumatology to extract the best procedures in diagnostic and treatment. Methods: Broad review of published articles related to the subject and compilation of guidelines of diagnostic and treatment by two advisors of each involved society, plastic surgery, dermatology and rheumatology. Results: The PG is not an exclusion disease anymore, with well defined criteria for its diagnostic and literature based treatment, refined by the authors, including the use of biological therapies. Conclusion: The PG remains challenging, but systematizing the investigation and the use of new drugs has opened a new horizon of treatments, interfering in the pathophysiology in a positive manner with fewer side effects than immunosuppressive therapy alone.

Consensus on the diagnosis and management of chronic leg ulcers - Brazilian Society of Dermatology.

BACKGROUND: Chronic leg ulcers affect a large portion of the adult population and cause a significant social and economic impact, related to outpatient and hospital care, absence from work, social security expenses, and reduced quality of life. The correct diagnosis and therapeutic approach are essential for a favorable evolution. OBJECTIVE: To gather the experience of Brazilian dermatologists, reviewing the specialized literature to prepare recommendations for the diagnosis and treatment of the main types of chronic leg ulcers. METHODS: Seven specialists from six university centers with experience in chronic leg ulcers were appointed by the Brazilian Society of Dermatology to reach a consensus on the diagnosis and therapeutic management of these ulcers. Based on the adapted DELPHI methodology, relevant elements were considered in the diagnosis and treatment of chronic leg ulcers of the most common causes; then, the recent literature was analyzed using the best scientific evidence. RESULTS: The following themes were defined as relevant for this consensus - the most prevalent differential etiological diagnoses of chronic leg ulcers (venous, arterial, neuropathic, and hypertensive ulcers), as well as the management of each one. It also included the topic of general principles for local management, common to chronic ulcers, regardless of the etiology. CONCLUSION: This consensus addressed the main etiologies of chronic leg ulcers and their management based on scientific evidence to assist dermatologists and other health professionals and benefit the greatest number of patients with this condition.

Consensus on the diagnosis and management of chronic leg ulcers - Brazilian Society of Dermatology

Abstract Background: Chronic leg ulcers affect a large portion of the adult population and cause a significant social and economic impact, related to outpatient and hospital care, absence from work, social security expenses, and reduced quality of life. The correct diagnosis and therapeutic approach are essential for a favorable evolution. Objective: To gather the experience of Brazilian dermatologists, reviewing the specialized literature to prepare recommendations for the diagnosis and treatment of the main types of chronic leg ulcers. Methods: Seven specialists from six university centers with experience in chronic leg ulcers were appointed by the Brazilian Society of Dermatology to reach a consensus on the diagnosis and therapeutic management of these ulcers. Based on the adapted DELPHI methodology, relevant elements were considered in the diagnosis and treatment of chronic leg ulcers of the most common causes; then, the recent literature was analyzed using the best scientific evidence. Results: The following themes were defined as relevant for this consensus - the most prevalent differential etiological diagnoses of chronic leg ulcers (venous, arterial, neuropathic, and hypertensive ulcers), as well as the management of each one. It also included the topic of general principles for local management, common to chronic ulcers, regardless of the etiology. Conclusion: This consensus addressed the main etiologies of chronic leg ulcers and their management based on scientific evidence to assist dermatologists and other health professionals and benefit the greatest number of patients with this condition.

Disseminated cutaneous sporotrichosis: unusual presentation in an alcoholic patient.

Sporotrichosis is an implantation mycosis highly prevalent in Brazil, associated with soil activities and contact with infected animals. It has numerous clinical manifestations and its disseminated cutaneous form is uncommon, found in approximately 4% of cases and usually related to immunosuppressive conditions. We report an alcoholic 56-year-old male patient with no other comorbidities, presenting with multiple cutaneous nodules and ulcers. The patient was diagnosed with disseminated cutaneous sporotrichosis based on the isolation and identification of Sporothrix spp. The patient was initially treated with amphotericin B with satisfactory results and then with itraconazole.

Sweet syndrome-like cutaneous drug reaction

Abstract: Cutaneous drug reactions are adverse reactions to medications that may present with different clinical features, ranging from localized to generalized lesions. In this report we describe a case of an unusual drug reaction, resembling the morphology of Sweet syndrome lesions. The patient had a psychiatric illness and was using thioridazine hydrochloride for one year. He developed infiltrated and grouped erythematous lesions on the elbows and knees three days after commencing multiple drugs (promethazine, haloperidol, mirtazapine and levomepromazine). After suspension of these four drugs and after the use of glucocorticoids, the patient had significant clinical improvement.

Sweet syndrome-like cutaneous drug reaction.

Cutaneous drug reactions are adverse reactions to medications that may present with different clinical features, ranging from localized to generalized lesions. In this report we describe a case of an unusual drug reaction, resembling the morphology of Sweet syndrome lesions. The patient had a psychiatric illness and was using thioridazine hydrochloride for one year. He developed infiltrated and grouped erythematous lesions on the elbows and knees three days after commencing multiple drugs (promethazine, haloperidol, mirtazapine and levomepromazine). After suspension of these four drugs and after the use of glucocorticoids, the patient had significant clinical improvement.

Clinical presentation of pili torti--Case report.

Pili torti also known as 'twisted hairs' (Latin: pili=hair; torti=twisted) is a rare, congenital or acquired clinical presentation, in which the hair shaft is flattened at irregular intervals and twisted 180º along its axis. It is clinically characterized by fragile, brittle, coarse and lusterless hairs, due to uneven light reflection on the twisted hair surface. Pili torti may be associated with neurological abnormalities and ectodermal dysplasias. There is no specific treatment for this condition, but it may improve spontaneously after puberty. We report a case of pili torti in a child who presented fragile, brittle, difficult to comb hair. The patient had no comorbidities.

Traumatic neuroma of the penis after circumcision--Case report.

Traumatic neuromas are tumors resulting from hyperplasia of axons and nerve sheath cells after section or injury to the nervous tissue. We present a case of this tumor, confirmed by anatomopathological examination, in a male patient with history of circumcision. Knowledge of this entity is very important in achieving the differential diagnosis with other lesions that affect the genital area such as condyloma acuminata, bowenoid papulosis, lichen nitidus, sebaceous gland hyperplasia, achrochordon and pearly penile papules.

Traumatic neuroma of the penis after circumcision - Case report

Abstract Traumatic neuromas are tumors resulting from hyperplasia of axons and nerve sheath cells after section or injury to the nervous tissue1. We present a case of this tumor, confirmed by anatomopathological examination, in a male patient with history of circumcision. Knowledge of this entity is very important in achieving the differential diagnosis with other lesions that affect the genital area such as condyloma acuminata, bowenoid papulosis, lichen nitidus, sebaceous gland hyperplasia, achrochordon and pearly penile papules.

Clinical presentation of pili torti - Case report

Pili torti also known as ‘twisted hairs’ (Latin: pili=hair; torti=twisted) is a rare, congenital or acquired clinical presentation, in which the hair shaft is flattened at irregular intervals and twisted 180º along its axis. It is clinically characterized by fragile, brittle, coarse and lusterless hairs, due to uneven light reflection on the twisted hair surface. Pili torti may be associated with neurological abnormalities and ectodermal dysplasias. There is no specifi c treatment for this condition, but it may improve spontaneously after puberty. We report a case of pili torti in a child who presented fragile, brittle, diffi cult to comb hair. The patient had no comorbidities.

Lúpus eritematoso sistêmico bolhoso na infância: relato de caso / Bullous systemic lupus erythematosus in the childhood: case report

É apresentado caso de lúpus eritematoso bolhoso na infância. Doente do sexo feminino, com nove anos, apresentava erupção vesicobolhosa no tronco, região cervical, genital e membros. O exame anatomopatológico mostrou bolha subepidérmica com neutrófilos, a imunofluorescência direta revelou depósito linear de IgA, IgM, IgG e C3 na zona da membrana basal, e a indireta foi negativa. Os anticorpos antinucleares e o anti-Sm estavam positivos. Houve regressão do quadro com dapsona e prednisona. Trata-se de caso raro de lúpus eritematoso sistêmico (LES) na infância que se iniciou com bolhas disseminadas. O LES bolhoso deve ser incluído no diagnóstico diferencial das erupções bolhosas na infância.

We report a case of bullous systemic lupus erythematosus in a 9-year-old female, with a vesiculobullous eruption on the trunk, neck, genitals and limbs. A skin biopsy specimen showed subepidermal blister with neutrophils. Direct immunofluorescence revealed deposition of IgA, IgG, IgM and C3 at the epithelial basement membrane zone and indirect immunofluorescence was negative. Antinuclear antibodies and anti-Sm were positive. Resolution of the blisters occurred following treatment with dapsone and prednisone. This is a rare case of systemic lupus erythematous (SLE) in the childhood, whose initial manifestation was a bullous eruption. Bullous SLE should be considered in the differential diagnosis of children presenting with generalized bullous eruption.

Direct immunofluorescence on uninvolved, lesional and perilesional skin in patients with endemic pemphigus foliaceus (fogo selvagem).

BACKGROUND: Research on direct immunofluorescence in pemphigus foliaceus ("fogo selvagem") has been focused mainly on the study of perilesional and lesional skin, while little attention has been given to uninvolved skin. We analyzed the frequency of IgA, IgM, IgG and its subclasses (IgG1, IgG2, IgG3 and IgG4) and C3 complement fraction deposition in intercellular spaces (ICS) and basal membrane zone (BMZ) in uninvolved, lesional and perilesional skin from 47 fogo selvagem patients. MATERIAL/METHODS: For each patient biopsies were collected from lesional, perilesional and uninvolved skin, and then analyzed by direct immunofluorescence. The panel of antibodies consisted of IgA, IgM, IgG and its subclasses (IgG1, IgG2, IgG3 and IgG4) and C3 complement fraction. RESULTS: The results showed a predominance of IgG and IgG4 deposit in all skin samples, followed by C3 complement fraction and IgG1 deposits. The positive response for IgG on uninvolved (91.48%), lesional (93.61%) and perilesional (97.87%) skin was similar to that found for IgG4 in the same samples: 95.74%, 95.74% and 97.87%, respectively. Regarding IgG1, the uninvolved skin showed lower results (14.89%) than the lesional (29.78%) and perilesional skin (29.78%). Concerning C3 complement fraction, the perilesional skin showed higher results (40.42%) than the uninvolved and lesional skin (34.04% for both). CONCLUSIONS: The results suggest the importance of uninvolved skin for direct immunofluorescence in the diagnostics of pemphigus foliaceus. Our results suggest that any cutaneous region can demonstrate pemphigus antibodies by direct immunofluorescence.

Ceratodermia palmoplantar de Unna-Thost associada a pseudo-ainhum: relato de um caso / Unna-Thost type palmoplantar keratoderma associated with pseudoainhum: a case report

Os autores relatam um caso de ceratodermia palmoplantar hereditária de Unna-Thost, de apresentação clínica atípica, bem como complicação do tipo estrangulamento ainhumóide no quinto dedo de ambos os pés. Os autores citam ainda outras ceratodermias palmoplantares hereditárias que podem cursar com pseudo-ainhum, bem como os critérios clínicos básicos que diferenciam essas manifestações.

Lúpus eritematoso sistêmico associado a síndorme nefrótica e trombose na veia cava inferior / Systemic lupus erythematosus associated with nephrotic syndrome and vena cava inferior thrombosis

O lúpus eritematoso é doença auto-imune, cujas manifestações variam de lesões cutâneas isoladas ao envolvimento de vários sistemas. A síndrome nefrótica ocorre em carca de 25 por cento dos casos. Caracteriza-se por proteinúria, hipoaluminemia, hipercolesterolemia e edema periférico. Um estado hipercoagulável acompanha essa síndrome, freqüentemente determinando complicações trombóticas. O caso relatado refere-se a uma paciente de 32 anos que apresentava lúpus eritematoso cutâneo crônico e, após seis anos de evolução, desenvolveu síndorme nefrótica complicada por trombose na veia cava inferior. Foi tratada com prednisona, enalapril,furosemida, albumina intravenosa, heparina e, posteriormente, warfarin, apresentando boa resposta. O caso enfatiza a necessidade de seguimento dos portadores de lúpus eritematoso cutâneo crônico a fim de serem detectados sinais de sistematização da doença