Localized pemphigus vulgaris on scalp: an atypical presentation.

We present two middle-aged patients with pruritic, crusted scalp erosions. Skin biopsy showed epidermal acantholysis with IgG and C3 intercellular deposits on direct immunofluorescence, leading to the diagnosis of localized pemphigus vulgaris. Resolution of the lesions without relapse occurred after low doses of oral prednisone and intralesional triamcinolone acetonide.

Darier disease: Histopathology revisited.

ABSTRACT: Darier disease (DD) is a rare genodermatosis. Literature on this topic is overwhelmingly dominated by case reports with rare clinical presentations, which have mentioned the histopathologic features briefly. The aim of this study was to document the histopathology of DD. Skin biopsies diagnosed as Darier disease based on clinicopathologic correlation over 12 years were reviewed for various epidermal and dermal features. There were 16 patients included, who most commonly presented in the third decade, with slight female predilection. The most common clinical presentation was hyperpigmented, hyperkeratotic, papules and plaques (91%), with 69% affecting the trunk. In addition to the classic suprabasal acantholytic clefts, we noted some unusual features: absence of parakeratosis (19%), a cornoid lamella-like pattern (62%), follicular acantholysis (13%) and multiple foci of involvement within a single biopsy (63%). Features such as the presence of dyskeratotic cells and minimal dermal lymphocytic infiltrates were concordant with previous literature. The limitation of this study was the small sample size. To conclude, pathologists must be aware of the variations in histopathology of Darier's disease, especially when challenged with atypical clinical presentations. The Darier-like pattern is met within several acantholytic diseases, and clinicopathologic correlation has the last word in arriving at a diagnosis.

Enigma of Acantholytic and Clear Cell Changes in Oral Squamous Cell Carcinoma.

Only limited cases have been reported about the clear cell variant of squamous cell carcinoma occurring in the oral cavity. The present study regards the case showing the histopathological features of both the clear cell and acantholytic variants of oral squamous cell carcinoma. A review of the literature has been done to understand the pathogenesis of those changes. Also, a hypothesis has been given that the clear cell changes could be the consequences of the cascades of the acantholytic process and not a separate entity. Therefore, more research is required to confirm this hypothesis and understand the prognosis of the lesion.

Familial "benign" pemphigus? Erythroderma and fatal outcome

Abstract Hailey-Hailey disease, or familial benign pemphigus, is a rare bullous genodermatosis that usually presents with flaccid blisters, erosions, and maceration limited to flexural areas, resulting in increased morbidity and reduced quality of life for affected patients. The authors report an unusual case of generalized Hailey-Hailey disease with erythroderma and fatal outcome.

Pemphigus foliaceus as a differential diagnosis in vesicobullous lesions / Pênfigo foliáceo como diagnóstico diferencial em lesões vesicobolhosas

ABSTRACT Given the challenge of clinical diagnosis of bullous skin lesions, this report aimed to discuss the histological changes, the presentation and clinical reasoning for diagnosis of these lesions. At the same time, the importance of the pathology was reviewed to identify these clinical scenarios. In this case report, we highlighted the clinical progression of a case of pemphigus foliaceus.

RESUMO Considerando o desafio do diagnóstico clínico de lesões cutâneas de apresentação bolhosa, o presente trabalho procurou discutir as alterações histológicas, a apresentação e o raciocínio clínico para o diagnóstico de tais lesões. Paralelamente, a importância da patologia foi revisada na identificação destes quadros. Neste relato de caso, destaca-se a evolução clínica de um quadro de pênfigo foliáceo.

Scanning electron microscopy of acantholysis in pemphigus foliaceus / Microscopia eletrônica de varredura da acantólise no pênfigo foliáceo

We performed scanning electron microscopy of an inverted blister roof in a case of pemphigus foliaceus. The loss of intercellular adherence could be easily seen with low magnification. The acantholytic keratinocytes displayed an irregular and sometimes polygonal contour. Round cells, typically seen in light microscopy, were also observed. The examination of a blister roof allows ultrastructural documentation of the acantholytic changes.

Realizamos microscopia eletrônica de varredura do teto invertido de uma bolha de um caso de pênfigo foliáceo. Com pequeno aumento, a perda da adesão intercelular pôde ser vista claramente. Os queratinócitos acantolíticos demostraram um contorno irregular, algumas vezes poligonal. Células arredondadas, como vistas tipicamente na microscopia óptica, também foram observadas. O exame de um teto de bolha permite uma documentação ultraestrutural das alterações acantolíticas.

Dermatosis acantolítica, lentiginosa, fotoinducida: una nueva e infrecuente variante de la enfermedad de Grover? / Photoinduced lentiginous acantholytic dermatosis: a new and unusu al pattern of Grover´s disease?

La dermatosis acantolítica transitoria o enfermedad de Grover es una dermatosis de etiología desconocida, caracterizada por presentarse como una erupción papuloeritematosa con brotes de vesículas y costras en el tronco; tiene como hallazgo histopatológico más frecuente, la presencia de acantólisis y disqueratosis focales. Afecta principalmente a hombres en edad media o mayores. Uno de los factores desencadenantes más frecuentes descriptos en la literatura es la exposición a la luz solar. Han sido comunicados recientemente 4 casos de enfermedad de Grover con una nueva forma de presentación clínica, caracterizada por la aparición de una erupción papulosa y costrosa, luego de la exposición solar, acompañada de abundantes lesiones lentiginosas. Se trató de tres mujeres en edad media y un hombre de 60 años. Los hallazgos histopatológicos coincidieron en presentar elongación de crestas interpapilares asociada a acantólisis y disqueratosis focales. Presentamos el caso de una mujer de 38 años que presenta lesiones clínicas e histológicas compatibles con esta nueva variante lentiginosa de la enfermedad de Grover descripta por Cooper en 2004.

Transient acantholytic dermatosis or Grover´s disease, is a dermatosis ofunknown aetiology, that typically presents as an erithematous papulareruption with crops of vesicles and crusts over the trunk; in which themost common histopathological fi nding is the presence of focal acantholysisand dyskeratosis. It aff ects mostly middle-aged or elderly men.One of the triggering factors most frequently described in the literatureis the exposure to sun light.Four cases have been recently reported of Grover´s disease with a newpattern of clinical presentation, characterized by infl amed papules andcrusts following sun exposure, along with lentiginous “freckling”. Theywere three middle aged women and a 60 years old man. The histopathologicalfi ndings coincided in presenting interpapilar ridges elongationassociated with focal acantholysis and dyskeratosis. We present a 38 yearold female with clinical and histopathological lesions compatible withthis new lentiginous pattern of Grover´s disease described by Cooper in2004 (Dermatol Argent 2010;16(2):122-125).

Pênfigo foliáceo em eqüino: relato de caso / Equine pemphigus foliaceous: case report

Diagnosticou-se pênfigo foliáceo em um eqüino macho, castrado, quatro anos de idade da raça Árabe apresentando dermatite generalizada caracterizada por placas escamo-crostosas e presença de dermatite pustular acantolítica subcorneal

Pemphigus foliaceus was diagnosed in a four-year-old Arabian equine gelding, with generalized dermatitis characterized by scaling and crusting plaques and acantholytic subcorneal pustular dermatitis

Dermatosis acantolíticas: a propósito de un caso / Acantholytic dermatosis: a case report

Se presenta el caso de una paciente de 64 años que ha tenido episodios recurrentes de lesiones vesiculosas en su extremidad inferior derecha, diagnosticándose una dermatosis acantolítica. Existe un espectro de enfermedades acantolíticas que histopatológicamente presentan acantolisis, pero que no comparten factores genéticos. El diagnóstico clínico puede ser extremadamente difícil y es la suma de herencia, clínica, histopatología y evolución, la que permite llegar a un diagnóstico. Se realiza una reseña de todas estas enfermedades.

We present the case of 64-year-old female patient with recurrent episodes of vesicles in her lower right limb. Histopathological study revealed acantholytic dermatosis. There is a large spectrum of acantholytic dermatosis that histopathologically presents acantholysis but does not share genetic factors. Clinical diagnosis may be extremely difficult and results in the sum of genetics, clinical findings, histopathological studies and evolution. We also present a review of these diseases.

Enfermedad de Grover-Dermatosis acantolítica transitoria: 30 años después / Grover's disease transient acantholytic dermatosis: 30 years later

Grover (1970) describió una dermatosis transitoria, generalmente pruriginosa, papulosa, a veces hiperqueratósica o costrosa, distribuida principalmente en el tronco, que remitía espontáneamente en semanas o meses. La presencia de acantolisis focal, con cuatro patentes histológicas asociadas y formas más crónicas o persistente, fueron descritas un tiempo después. Presentamos seis nuevos casos y efectuamos una revisión de las dermatosis acantolítica transitoria (DAT) a la luz de los conocimientos actuales. Enfatizamos la experiencia y publicaciones de autores iberolatinoamericanos

Enfermedad de Grover: presentación de cuatro casos / Grover's disease: report of four cases

Se comunican cuatro casos de dermatosis acantolítica transitoria (DAT) o enfermedad de Grover, dos de los cuales presentaban lesiones de difícil diagnóstico clínico si no se tiene un alto índice de sospecha, los cuatro casos presentaron la variante histopatológica del tipo enfermedad de Darier. No se hallaron enfermedades sistémicas ni neoplásicas asociadas. Creemos que la enfermedad de Grover es mucho más frecuente de lo pensado y en nuestra experiencia también se ha correlacionado con la exposición solar, la piel seca y/o excesiva sudoración. Sospechamos que a pesar de su denominación, la mayoría de los casos de DAT poseen una evolución prolongada, siendo infrecuentes las remisiones totales

Pénfigo eritematoso: lesión única localizada en nariz / Penphigus erythematodes: single lesion localized to the nose

Se presenta un paciente de 41 años, de sexo masculino, que presentó una lesión única en la punta de la nariz. Semiológicamente se observó una placa eritematoviolácea, cubierta por una fina costra y sin síntomas. La evolución era de un año. La histología mostró acantolisis y la inmunofluorescencia depósitos de IgG y C3. La lesión se mantuvo estable. El diagnóstico fue de pénfigo eritematoso. La presentación del caso se debe a la rareza de la lesión única

Complete regression of melanocytic nevi: clues for proper diagnosis

Säo relatados os aspectos histopatológicos de uma pápula isolada do pescoço comparativamente a 80 nevos melanocíticos. Os aspectos histogenéticos sugerem eque este tumor representa o resíduo de un nevo melanocítico onde as células névicas já desapareceram, porém a infiltraçäo da derme adipócitos maduros, o espessamento fibroso da derme e os aspectos típicos dos outros elementos cutâneo permanecem. O achado de disqueratose acantolítica focal em múltiplos pontos adiciona novo elemento sugestivo de NEVO em regressäo. Lesöes com características morfológicas de nevo melanocítico, mesmo na ausência de células névicas podem ser identificadas se as alteraçöes associadas forem reconhecidas

Focal acantholytic dyskeratosis: a snare for the pathologist: report of two cases associated to psoriasis and fibrous papule of the nose

Two specimens containing clinically inapparent histologic features of acantholytic dyskeratosis (on the base of a fibrous papule of the nose and overlying a psoriatic lesion) are presented. The authors discuss the conduct to be followed by the pathologist in similar cases. The general pathologist should be well trained in dermatopathology since cutaneous biopsies account for 10 to 25% of the specimens submitted for histologic diagnosis or, even, form the major part of the workload (Berry). There is a wall between the physician who submits the skin biopsies (who may be or not a dermatologist) and the pathologist. The clinical information provided by dermatologists is scant and incomplete and physicians who are not dermatologists seldom submit any information. The histological pictures found in skin biopsies are, often, common to several nosological entities and an adequate understanding of their meaning is desirable for a thorough evaluation. We ought to assess it with the maximum scientific severity, searching to solve the puzzle without depreciating the information received. In this report the authors analyse the histopathological approach to the cutaneous lesions of two patients. They displayed the association between acantholytic dyskeratosis (AD) and another cutaneous pathology. Findings like these may obstruct the final diagnosis to be issued by the pathologist.(ABSTRACT TRUNCATED AT 250 WORDS)

Enfermedad de Grover / Grover's disease

Se presenta un caso de dermatosis acantolítica transitoria descripta por primera vez por R. Grover en 1970. Se consideran los diagnósticos diferenciales clínicos y se describen los cuatro patrones histológicos que aparecen en esta dermatosis. Se discute la etiopatología y terapéutica. Se comunica este caso de Enfermedad de Grover debido a que es una dermatosis infrecuente que obliga a plantear numerosos diagnósticos diferenciales desde el punto de vista clínico

Dermatosis acantolitica transitoria: Reporte de dos casos / Transient acantolitic dermatosis: two cases report

Se presentan dos casos de Dermatosis Acantolítica Transitoria (D.A.T.) o enfermedad de Grover, patología infrecuente pero interesante debido a su semejanza clínica e hispatológica con otras afecciones dermatológicas. Uno de los pacientes respondió al tratamiento con esteroides sistémicos El otro tuvo una evolución autolimitada. Se realiza un comentario general sobre la enfermedad y diagnósticos diferenciales

Disqueratosis acantolitica de la vulva: presentación de dos casos / Diskeratosis acantolitic of the vulva: two cases presentation

Se presentan dos casos de Disqueratosis acantolítica de la vulva y se discute la histología de la afección

Disqueratosis acantolitica focal como hallazgo histopatológico casual / Acantholitic disperatosis how histopathological occasional finding

Comunicamos 3 casos de Disqueratosis Acantolítica (D.A.F) como hallazgo histológico incidental en un epitelioma basocelular, linfoma linfocítico y dermatitis seborreica, no encontrando otras publicaciones similares en estos dois últimos casos, se destaca la importancia del reconocimiento de la D.A.F. para la correcta correlación clínico-patológica