Epidemiological and clinical study of cases of endemic pemphigus foliaceus and pemphigus vulgaris in a reference center in the state of Minas Gerais, Brazil.

BACKGROUND: Pemphigus constitutes a group of autoimmune bullous diseases. A reduction in the incidence of endemic pemphigus foliaceus and an increase in pemphigus vulgaris has been described, but there are no studies in Minas Gerais that address the subject. OBJECTIVE: To describe the epidemiological and clinical profile of patients with pemphigus treated at the Dermatology Service of a public University Hospital in the state of Minas Gerais, Brazil. METHODS: An observational, descriptive, and cross-sectional study was carried out of cases of endemic pemphigus foliaceus and pemphigus vulgaris, for a period of six months. A questionnaire was filled out with epidemiological and clinical data on the disease. RESULTS: A total of 122 patients were included in the study, 64 with endemic pemphigus foliaceus and 58 with pemphigus vulgaris. When comparing patients with endemic pemphigus foliaceus and those with pemphigus vulgaris, a statistical difference was observed between the median age of initial disease manifestation (p = 0.001), patient occupation (p = 0.010), area of residence (p = 0.000), forests (p = 0.000) and rivers/streams close to the dwelling (p = 0.001) and the number of systemic medications required to control the disease (p = 0.002). When comparing patients with endemic pemphigus foliaceus to those evaluated in a study carried out at the same service in 2008, there was a statistical difference in the area of residence (p = 0.030). STUDY LIMITATIONS: The assessed population comes from a tertiary care service that is not a reference for the entire state. CONCLUSIONS: Patients with endemic pemphigus foliaceus and pemphigus vulgaris maintain statistically significant differences regarding their main variables in the literature, such as age and area of residence. Historically, there has been a reduction in cases of endemic pemphigus foliaceus and an increase in cases of pemphigus vulgaris in this population.

Cutaneous lupus erythematosus: a review of etiopathogenic, clinical, diagnostic and therapeutic aspects.

Cutaneous lupus erythematosus is an autoimmune disease of varied clinical expression, which may present as an exclusively cutaneous disease or be one of the multiple manifestations of systemic lupus erythematosus. Its classification includes acute, subacute, intermittent, chronic and bullous subtypes, which are usually identified based on clinical features and histopathological and laboratory findings. Other non-specific cutaneous manifestations may be associated with systemic lupus erythematosus and are usually related to disease activity. Environmental, genetic and immunological factors play a role in the pathogenesis of skin lesions in lupus erythematosus. Recently, considerable progress has been made in elucidating the mechanisms involved in their development, which allows for foreseeing future targets for more effective treatments. This review proposes to discuss the main etiopathogenic, clinical, diagnostic and therapeutic aspects of cutaneous lupus erythematosus, aiming to update internists and specialists from different areas.

Comedogenic lupus: a rare variant of chronic cutaneous lupus erythematosus - case series.

BACKGROUND: Comedogenic lupus is an uncommon variant of cutaneous lupus, clinically characterized by the presence of comedones, papules and erythematous-infiltrated plaques, cysts and scars in photo-exposed areas, mimicking acne vulgaris and acneiform eruptions. OBJECTIVES: To report clinicopathological characteristics of patients with comedogenic lupus in a tertiary dermatology service over a 15-year period and review cases described in the literature. METHODS: Retrospective study of patients with clinical and histopathological diagnoses of comedogenic lupus between the years 2006 and 2021. The literature search was carried out in the PubMed and VHL Regional Portal databases, using the terms: "comedogenic lupus" and "acneiform lupus" in Portuguese and English. RESULTS: Five patients were diagnosed during the described period, all female, with a mean age of 56.6 years. Smoking was observed in three cases, as well as pruritus. The most affected site was the face, especially the pre-auricular, malar and chin regions. Follicular plugs, epidermal thinning and liquefaction degeneration of the basal layer were predominant histopathological findings. Hydroxychloroquine was used as the first-line treatment; however, other medications were used, such as dapsone, methotrexate, tretinoin cream, and topical corticosteroids. The literature search identified 17 cases, with a mean age of 38.9 years, 82% of which were women. Only 23% had a diagnosis of systemic lupus erythematosus. Hydroxychloroquine was the most recommended systemic medication. STUDY LIMITATIONS: Retrospective, single-center study. The literature search was carried out in two databases. CONCLUSIONS: Dermatologists should be aware of acneiform conditions with poor response to the usual treatment. Early diagnosis and treatment reduce the risk of unaesthetic scars.

Gummatous cutaneous tuberculosis associated with the use of infliximab for Crohn's disease.

As the treatment of infectious and parasitic diseases improved, the prevalence of these conditions declined. However, with the expansion of the use of immunobiologicals, opportunistic infections have emerged, especially under atypical presentations. The present study reports the case of a patient treated with infliximab for Crohn's disease, who presented diarrhea, weight loss, abdominal pain, fever, and subcutaneous erythematous nodules that evolved with spontaneous fluctuation and ulceration. With the finding of alcohol-resistant bacilli and Mycobacterium tuberculosis DNA in a cutaneous fragment, through polymerase chain reaction, the diagnosis of gummatous tuberculosis was confirmed, probably secondary to hematogenous dissemination from an intestinal focus.

Necrobiotic xanthogranuloma associated with smoldering multiple myeloma: satisfactory response to cyclophosphamide, dexamethasone, and thalidomide.

Necrobiotic xanthogranuloma is a rare chronic condition, belonging to the group C non-Langerhans cell histiocytoses, which is relevant due to the possibility of extracutaneous involvement and association with systemic diseases, particularly hematologic malignancies. The case reported here was only diagnosed after nine years of evolution and was associated with plasma cell dyscrasia. After treatment with cyclophosphamide, dexamethasone, and thalidomide, there was a reduction of cutaneous lesions and serum levels of monoclonal protein.

Scrofuloderma: a diagnostic challenge.

Cutaneous tuberculosis is a rare form of extrapulmonary tuberculosis, comprising 1-2% of cases. Caused by Mycobacterium tuberculosis or related strains, it presents a wide range of clinical manifestations, mimicking other chronic dermatoses and leading to delayed diagnosis. A case of scrofuloderma is reported, whose diagnosis and treatment were only made six years after onset of the disease.

Consensus on the treatment of autoimmune bullous dermatoses: dermatitis herpetiformis and linear IgA bullous dermatosis - Brazilian Society of Dermatology.

Dermatitis herpetiformis and linear IgA bullous dermatosis are autoimmune diseases that present with pruritic urticarial papules and plaques, with formation of vesicles and blisters of subepidermal location, mediated by IgA antibodies. Mucosal lesions are present only in linear IgA bullous dermatosis. The elaboration of this consensus consisted of a brief presentation of the different aspects of these dermatoses and, above all, of an updated literature review on the various therapeutic options that were discussed and compared with the authors' experience, aiming at the treatment orientation of these diseases in Brazil. Dermatitis herpetiformis is a cutaneous manifestation of celiac disease, and can be controlled with a gluten-free diet and dapsone. On the other hand, linear IgA bullous dermatosis arises spontaneously or is triggered by drugs, and can be controlled with dapsone, but often requires the association of systemic corticosteroids and eventually immunosuppressants.

Epidemiological and clinical study of cases of endemic pemphigus foliaceus and pemphigus vulgaris in a reference center in the state of Minas Gerais, Brazil

Abstract Background: Pemphigus constitutes a group of autoimmune bullous diseases. A reduction in the incidence of endemic pemphigus foliaceus and an increase in pemphigus vulgaris has been described, but there are no studies in Minas Gerais that address the subject. Objective: To describe the epidemiological and clinical profile of patients with pemphigus treated at the Dermatology Service of a public University Hospital in the state of Minas Gerais, Brazil. Methods: An observational, descriptive, and cross-sectional study was carried out of cases of endemic pemphigus foliaceus and pemphigus vulgaris, for a period of six months. A questionnaire was filled out with epidemiological and clinical data on the disease. Results: A total of 122 patients were included in the study, 64 with endemic pemphigus foliaceus and 58 with pemphigus vulgaris. When comparing patients with endemic pemphigus foliaceus and those with pemphigus vulgaris, a statistical difference was observed between the median age of initial disease manifestation (p = 0.001), patient occupation (p = 0.010), area of residence (p = 0.000), forests (p = 0.000) and rivers/streams close to the dwelling (p = 0.001) and the number of systemic medications required to control the disease (p = 0.002). When comparing patients with endemic pemphigus foliaceus to those evaluated in a study carried out at the same service in 2008, there was a statistical difference in the area of residence (p = 0.030). Study limitations: The assessed population comes from a tertiary care service that is not a reference for the entire state. Conclusions: Patients with endemic pemphigus foliaceus and pemphigus vulgaris maintain statistically significant differences regarding their main variables in the literature, such as age and area of residence. Historically, there has been a reduction in cases of endemic pemphigus foliaceus and an increase in cases of pemphigus vulgaris in this population.

Cutaneous lupus erythematosus: a review of etiopathogenic, clinical, diagnostic and therapeutic aspects

Abstract Cutaneous lupus erythematosus is an autoimmune disease of varied clinical expression, which may present as an exclusively cutaneous disease or be one of the multiple manifestations of systemic lupus erythematosus. Its classification includes acute, subacute, intermittent, chronic and bullous subtypes, which are usually identified based on clinical features and histopathological and laboratory findings. Other non-specific cutaneous manifestations may be associated with systemic lupus erythematosus and are usually related to disease activity. Environmental, genetic and immunological factors play a role in the pathogenesis of skin lesions in lupus erythematosus. Recently, considerable progress has been made in elucidating the mechanisms involved in their development, which allows for foreseeing future targets for more effective treatments. This review proposes to discuss the main etiopathogenic, clinical, diagnostic and therapeutic aspects of cutaneous lupus erythematosus, aiming to update internists and specialists from different areas.

Comedogenic lupus: a rare variant of chronic cutaneous lupus erythematosus - case series

Abstract Background: Comedogenic lupus is an uncommon variant of cutaneous lupus, clinically characterized by the presence of comedones, papules and erythematous-infiltrated plaques, cysts and scars in photo-exposed areas, mimicking acne vulgaris and acneiform eruptions. Objectives: To report clinicopathological characteristics of patients with comedogenic lupus in a tertiary dermatology service over a 15-year period and review cases described in the literature. Methods: Retrospective study of patients with clinical and histopathological diagnoses of comedogenic lupus between the years 2006 and 2021. The literature search was carried out in the PubMed and VHL Regional Portal databases, using the terms: ''comedogenic lupus'' and ''acneiform lupus'' in Portuguese and English. Results: Five patients were diagnosed during the described period, all female, with a mean age of 56.6 years. Smoking was observed in three cases, as well as pruritus. The most affected site was the face, especially the pre-auricular, malar and chin regions. Follicular plugs, epidermal thinning and liquefaction degeneration of the basal layer were predominant histopathological findings. Hydroxychloroquine was used as the first-line treatment; however, other medications were used, such as dapsone, methotrexate, tretinoin cream, and topical corticosteroids. The literature search identified 17 cases, with a mean age of 38.9 years, 82% of which were women. Only 23% had a diagnosis of systemic lupus erythematosus. Hydroxychloroquine was the most recommended systemic medication. Study limitations: Retrospective, single-center study. The literature search was carried out in two databases. Conclusions: Dermatologists should be aware of acneiform conditions with poor response to the usual treatment. Early diagnosis and treatment reduce the risk of unaesthetic scars.

Wells syndrome associated with chronic lymphocytic leukemia.

Eosinophilic cellulitis or Wells syndrome is an uncommon skin condition of unknown etiology that can occur alone or associated with other conditions. Typically, it presents with recurrent pruritic, erythematous and edematous plaques, but it can also show clinical polymorphism. Besides the cutaneous lesions, patients can experience systemic manifestations like fever, malaise, arthralgia and peripheral blood eosinophilia. We describe a case of this rare syndrome that presented with polymorphic cutaneous lesions associated with a serious systemic disease, which was revealed through the investigation of the cutaneous disease.

Necrobiotic xanthogranuloma associated with smoldering multiple myeloma: satisfactory response to cyclophosphamide, dexamethasone, and thalidomide

Abstract: Necrobiotic xanthogranuloma is a rare chronic condition, belonging to the group C non-Langerhans cell histiocytoses, which is relevant due to the possibility of extracutaneous involvement and association with systemic diseases, particularly hematologic malignancies. The case reported here was only diagnosed after nine years of evolution and was associated with plasma cell dyscrasia. After treatment with cyclophosphamide, dexamethasone, and thalidomide, there was a reduction of cutaneous lesions and serum levels of monoclonal protein.

Consensus on the treatment of autoimmune bullous dermatoses: dermatitis herpetiformis and linear IgA bullous dermatosis - Brazilian Society of Dermatology

Abstract: Dermatitis herpetiformis and linear IgA bullous dermatosis are autoimmune diseases that present with pruritic urticarial papules and plaques, with formation of vesicles and blisters of subepidermal location, mediated by IgA antibodies. Mucosal lesions are present only in linear IgA bullous dermatosis. The elaboration of this consensus consisted of a brief presentation of the different aspects of these dermatoses and, above all, of an updated literature review on the various therapeutic options that were discussed and compared with the authors' experience, aiming at the treatment orientation of these diseases in Brazil. Dermatitis herpetiformis is a cutaneous manifestation of celiac disease, and can be controlled with a gluten-free diet and dapsone. On the other hand, linear IgA bullous dermatosis arises spontaneously or is triggered by drugs, and can be controlled with dapsone, but often requires the association of systemic corticosteroids and eventually immunosuppressants.

Scrofuloderma: a diagnostic challenge

Abstract: Cutaneous tuberculosis is a rare form of extrapulmonary tuberculosis, comprising 1-2% of cases. Caused by Mycobacterium tuberculosis or related strains, it presents a wide range of clinical manifestations, mimicking other chronic dermatoses and leading to delayed diagnosis. A case of scrofuloderma is reported, whose diagnosis and treatment were only made six years after onset of the disease.