[Stigmas, studies, and strife: a history of Brazilian pemphigus, nineteenth and twentieth centuries]. / Estigmas, pesquisas e embates: uma história do pênfigo brasileiro, séculos XIX e XX.

This paper presents a historical narrative about the incidence of pemphigus foliaceus in Brazil in the nineteenth and twentieth centuries. This autoimmune blistering skin disease is more common in children, adolescents, and young adults who live in rural areas of endemic regions. It was first described in Brazil in 1903 by the physician Caramuru Paes Leme. The main foci of the disease are in the Federal District and the states of Goiás, Mato Grosso, Mato Grosso do Sul, Minas Gerais, Paraná, and São Paulo. This research topic, which has attracted widespread attention from medical practice, especially dermatology, has not received similar attention from historians of health and disease.

O artigo apresenta uma narrativa histórica sobre a incidência do pênfigo foliáceo no Brasil ao longo dos séculos XIX e XX. Doença bolhosa autoimune da pele que acomete com mais frequência crianças, adolescentes e adultos jovens que vivem nas áreas rurais de regiões endêmicas. Foi descrita pela primeira vez no país em 1903, pelo médico Caramuru Paes Leme. Os principais focos se situam no Distrito Federal e nos estados de Goiás, Mato Grosso, Mato Grosso do Sul, Minas Gerais, Paraná e São Paulo. Temática de pesquisa amplamente visitada pela ciência médica, em especial a dermatologia, não tem merecido a atenção peculiar por parte dos historiadores da saúde e da doença.

Estigmas, pesquisas e embates: uma história do pênfigo brasileiro, séculos XIX e XX / Stigmas, studies, and strife: a history of Brazilian pemphigus, nineteenth and twentieth centuries

Resumo O artigo apresenta uma narrativa histórica sobre a incidência do pênfigo foliáceo no Brasil ao longo dos séculos XIX e XX. Doença bolhosa autoimune da pele que acomete com mais frequência crianças, adolescentes e adultos jovens que vivem nas áreas rurais de regiões endêmicas. Foi descrita pela primeira vez no país em 1903, pelo médico Caramuru Paes Leme. Os principais focos se situam no Distrito Federal e nos estados de Goiás, Mato Grosso, Mato Grosso do Sul, Minas Gerais, Paraná e São Paulo. Temática de pesquisa amplamente visitada pela ciência médica, em especial a dermatologia, não tem merecido a atenção peculiar por parte dos historiadores da saúde e da doença.

Abstract This paper presents a historical narrative about the incidence of pemphigus foliaceus in Brazil in the nineteenth and twentieth centuries. This autoimmune blistering skin disease is more common in children, adolescents, and young adults who live in rural areas of endemic regions. It was first described in Brazil in 1903 by the physician Caramuru Paes Leme. The main foci of the disease are in the Federal District and the states of Goiás, Mato Grosso, Mato Grosso do Sul, Minas Gerais, Paraná, and São Paulo. This research topic, which has attracted widespread attention from medical practice, especially dermatology, has not received similar attention from historians of health and disease.

Estigmas, pesquisas e embates: uma história do pênfigo brasileiro, séculos XIX e XX / Stigmas, studies, and strife: a history of Brazilian pemphigus, nineteenth and twentieth centuries

O artigo apresenta uma narrativa histórica sobre a incidência do pênfigo foliáceo no Brasil ao longo dos séculos XIX e XX. Doença bolhosa autoimune da pele que acomete com mais frequência crianças, adolescentes e adultos jovens que vivem nas áreas rurais de regiões endêmicas. Foi descrita pela primeira vez no país em 1903, pelo médico Caramuru Paes Leme. Os principais focos se situam no Distrito Federal e nos estados de Goiás, Mato Grosso, Mato Grosso do Sul, Minas Gerais, Paraná e São Paulo. Temática de pesquisa amplamente visitada pela ciência médica, em especial a dermatologia, não tem merecido a atenção peculiar por parte dos historiadores da saúde e da doença.

Early Description of Diet-Induced Blistering Skin Diseases in Medieval Persia: Avicenna's Point of View.

Pemphigus is an autoimmune blistering skin disease that is strongly associated with different environmental factors. Among these, nutritional factors are considered to trigger pemphigus; however, their role may be underestimated. Investigated more recently in conventional medicine, this causative bond between dietary factors and blistering skin diseases was mentioned by Persian scholars such as Avicenna a thousand years ago. Avicenna, a well-known Persian physician and philosopher, who could be considered a pioneer in dermatology, discussed skin diseases in a chapter in The Canon of Medicine. He accounted for some nutritional triggers for skin blisters (mentioned as "hot swellings"), such as onion, garlic, leek, pepper, and wine. His precise description of causative factors based on principles of traditional Persian medicine (TPM) is appreciable and might well lead us to find more efficient ways for the prevention and treatment of blistering skin diseases.

From pemphix to desmogleins.

In the not so distant past, the word pemphigus or pemphix was common for describing various diseases characterized by blistering as well as various disorders that do not originate from a blistering pathology. Patients with these conditions were grouped in "other" skin diseases. Step by step, during the past, we were introduced to these severe conditions. First, we learned from sporadic case reports, then new differentiations were reported according to histology, later immunopathology was developed, and now there are discoveries of new molecules. Immense progress with new approaches to therapy has been achieved, but much improvement is still needed. The modern definition of pemphigus undoubtedly represents a group of rare, intraepidermal autoimmune bullous diseases characterized by intraepidermal blisters and circulating autoantibodies desmogleins against the keratinocytes cell surface.

[Nikolsky and his sign]. / Nikolsky en zijn teken.

Piotr Vasiliyevich Nikolsky (1858-1940) trained as a dermatologist and venereologist in Kiev. In 1898 he was appointed to the chair at the University of Warsaw, which moved to Rostov on the Don during the First World War. In his doctoral dissertation of 1896 he described the sign now named after him; in patients with pemphigus foliaceus, gentle rubbing of the skin causes crinkling because the superficial layer (stratum corneum) slides over the deeper layer. The same phenomenon was soon found in patients with pemphigus vulgaris, which is more common. The sign is absent in about 60% of patients with these conditions. It is also absent in patients with pemphigoid diseases, because in these patients it is the splitting of deeper layers of the skin that causes blistering.

Pénfigo herpetiforme: presentación de un caso y revisión de la literatura / Herpetiform pemphigus: case report and literature review

El pénfigo herpetiforme (PH) es una variante poco frecuente de pénfigo con rasgos clínicos, histopatológicos, inmunológicos y terapéuticos singulares. Se caracteriza por combinar figuras clínicas de dermatitis herpetiforme (DH) con inmunofluorescencia típica de pénfigo. Se expresa clínicamente como placas eritematosas, urticarianas y vesículas con distribución herpetiforme. Los hallazgos histológicos son variables, incluyendo espongiosis eosinofílica con pústulas subcórneas, asociado o no a acantolisis. Se acompaña de prurito y de buen estado general. Comparado con otros pénfigos, su curso es benigno y responde a tratamiento con dapsona, generalmente asociada a dosis bajas de corticoesteroides. Se presenta un paciente de 49 años de edad, cuyo diagnóstico definitivo de PH se realizó luego de 10 años de padecer la enfermedad (brotes extendidos en tronco y raíz de miembros de lesiones eritemato-papulo-vesiculosas, pruriginosas) y ser asistido por numerosos dermatólogos (AU)

Pénfigo herpetiforme: presentación de un caso y revisión de la literatura / Herpetiform pemphigus: case report and literature review

El pénfigo herpetiforme (PH) es una variante poco frecuente de pénfigo con rasgos clínicos, histopatológicos, inmunológicos y terapéuticos singulares. Se caracteriza por combinar figuras clínicas de dermatitis herpetiforme (DH) con inmunofluorescencia típica de pénfigo. Se expresa clínicamente como placas eritematosas, urticarianas y vesículas con distribución herpetiforme. Los hallazgos histológicos son variables, incluyendo espongiosis eosinofílica con pústulas subcórneas, asociado o no a acantolisis. Se acompaña de prurito y de buen estado general. Comparado con otros pénfigos, su curso es benigno y responde a tratamiento con dapsona, generalmente asociada a dosis bajas de corticoesteroides. Se presenta un paciente de 49 años de edad, cuyo diagnóstico definitivo de PH se realizó luego de 10 años de padecer la enfermedad (brotes extendidos en tronco y raíz de miembros de lesiones eritemato-papulo-vesiculosas, pruriginosas) y ser asistido por numerosos dermatólogos

The men behind the eponym: Francis E. Senear, Barney Usher, and the Senear-Usher syndrome.

This historical review will summarize the report of Francis Senear and Barney Usher on a disease that they called pemphigus erythematodes and which later became pemphigus erythematosus (the Senear-Usher syndrome). It will then outline the lives of these two men. Finally, it will review the literature on that condition and relate the views of various authorities as to whether pemphigus erythematosus is merely pemphigus foliaceus, a variant of pemphigus foliaceus, a syndrome combining features of lupus erythematosus and pemphigus or whether pemphigus erythematosus (Senear-Usher syndrome) is a distinct and separate disease.

History of the establishment and revision of diagnostic criteria, severity index and therapeutic guidelines for pemphigus in Japan.

We summarize the process of establishing and revising the diagnostic criteria, severity index and therapeutic guidelines for pemphigus in Japan (including the results of a nationwide survey regarding these guidelines). We also summarize the content and present an evaluation of the utility of these guidelines. Due to the publication of these documents throughout the Japanese medical community, it appears that patients with pemphigus have recently begun to receive appropriate treatment, dramatically improving their quality of life and prognosis. Continuous examination of these criteria by means of follow-up studies of patients treated according to the guidelines is necessary to determine the long-term efficacy of treatment. To this end, it is hoped that these guidelines will be more extensively disseminated among the medical community.

[Pemphigus vegetans. A historical perspective]. / Pemphigus vegetans. Eine historische Betrachtung.

Pemphigus vegetans is a rare variant of pemphigus which is mainly localized in the intertriginous areas (Neumann type, Hallopeau type) or primarily involves the mucous membranes (pyostomatitis vegetans). A 18-year-old patient with erosive stomatitis developed a vegetating plaque with papillomatous and verruciform features in her left axilla. Histopathological examination of the axilla revealed papillomatosis and acanthosis as well as suprabasal clefting with acantholytic cells. By direct immunofluorescent examination, deposits of immunoglobulin IgG and complement (C3) were found in the intercellular space of the epidermis. Serological examination by indirect immunofluorescent techniques was indicative of pemphigus autoantibodies at a titer of 1:40. This case report demonstrates that the classical differentiation of pemphigus vegetans in two types, Neumann type and Hallopeau type, is mainly of historical importance, because both entities may represent variants of the same disease. In addition, other disorders such as IgA pemphigus may also present with vegetating plaques in intertriginous sites.