[Case for diagnosis. Dermatofibrosarcoma protuberans]. / Haga su diagnóstico. Dermatofiborsarcoma protuberans.

A 30-year-old woman developed a slowly progressive reddish-violet multinodular rubbery lesion, measuring 20 x 10 cm in diameter, on the upper outer quadrant of her right breast four years after the surgical excision of a dermatofibrosarcoma protuberans (DFSP) in the same area. The diagnosis of DFSP was confirmed histologically and by positive immunomarkers at immunohistochemistry. A complete excision was performed and after a 2-year follow-up no recurrence was observed. It should be emphasized that DFSP of the breast is a rare occurrence.

Detection of polyomavirus major capsid antigen (VP-1) in human pilomatricomas.

The family Polyomaviridae is composed of small, non-enveloped, double-stranded DNA viruses widely used to study cell transformation in vitro and tumor induction in vivo. The development of pilomatricomas in mice experimentally infected with polyomavirus led us to detect the viral major capsid protein VP-1 in human pilomatricomas. This tumor, even uncommon, is one of the most frequent benign hair follicle tumors in humans and is composed of proliferating matrix cells that undergo keratinization, and form cystic neoplasms. The detection of VP-1 was performed using the peroxidase-antiperoxidase technique in paraffin-embedded slides with a specific primary serum. Adjacent slides treated with normal rabbit serum as a primary were employed as internal control. Positive and negative controls were also employed as well as slides of lesions caused by human papillomavirus to rule out any unspecific cross-reactivity. In 4 out of 10 cases polyomavirus VP-1 was clearly detected in nuclei of human pilomatricomas proliferating cells, in a patchy pattern of distribution. The controls confirmed the specificity of the immunocytochemical procedure. These results could indicate either an eventual infection of the virus in already developed tumors or alternatively, a direct involvement of polyomavirus in the pathogenesis of some pilomatricomas. The recent discovery of a new human polyomavirus associated with Merkel cell carcinomas has been a strong contribution to better understand the pathogenesis of some human uncommon skin cancers. Hopefully the results reported in this work will encourage further research on the role of polyomavirus in other human skin neoplasms.

Haga su diagnóstico / Case for diagnosis

Presentamos a una paciente de 30 años de edad, que desarrolla en forma lenta y progresiva una tumoración rojo violácea multinodular de consistencia duroelástica de 20cm por 10cm en el cuadrante superoexterno de su mama derecha, a los cuatro años de habérsele extirpado un dermatofiborsarcoma protuberans (DFSP) en dicha localización. El estudio histopatológico y la imnunomarcación confirmaron la recidiva. A los dos años de la nueva intervención quirúrgica no existen evidencias de lesión neoplásica. Debe considerarse infrecuente la localización mamaria de esta patología.

A 30-year-old woman developed a slowly progressive reddish-violet multinodular rubbery lesion, measuring 20 x 10 cm in diameter, on the upper outer quadrant of her right breast four years after the surgical excision of a dermatofibrosarcoma protuberans (DFSP) in the same area. The diagnosis of DFSP was confirmed histologically and by positive immunomarkers at immunohistochemistry. A complete excision was performed and after a 2-year follow-up no recurrence was observed. It should be emphasized that DFSP of the breast is a rare occurrence.

Milia en placa en varias y sucesivas áreas / Milia en plaque at multiple and successive sites

No disponible

Cutaneous true histiocytic malignancy: true histiocytic lymphoma.

True histiocytic malignancies (THM) are controversial disorders that are being re-evaluated with modern cellular and molecular biology techniques. True histiocytic lymphoma (THL) is a low-incidence, poor-prognosis THM. It mainly affects the skin, gastrointestinal tract, and bone tissues. Occasionally, skin lesions are the presenting clinical features. A patient with initially cutaneous THL is described. This and other reported cases confirm that THM are a nosologic entity by themselves.

Pitiríase rósea purpúrica: relato de caso e revisão de literatura / Purpuric pityriasis rosea: case report and literature review

A pitiríase rósea purpúrica constitui doença rara e 10 casos foram publicados na Europa e EUA. O quadro clínico cutâneo é a forma hemorrágica ou purpúrica com variável descamação marginal. Relata-se o caso de um homem de 25 anos de idade com lesões na região escapular. A revisão da literatura enfatiza o diagnóstico diferencial das lesões purpúricas. Segundo Lilacs e Medline, não foram relatados casos na literatura latino-americana

Up-regulated expression of MICA and proinflammatory cytokines in skin biopsies from patients with seborrhoeic dermatitis.

Seborrhoeic dermatitis is a disease of unknown etiopathogenesis that affects 5% of the population. In this study, we investigated expression of mRNA for IL-1 alpha, IL-6, IL-4, IFN-gamma, and the stress-inducible MICA molecule in skin biopsies from 12 patients with moderate to severe seborrhoeic dermatitis and 2 healthy volunteers by RT-PCR and hybridization with specific probes. Eight patients expressed INF-gamma, 2 expressed IL-6, 8 expressed IL-1 alpha, and 2 expressed IL-4 (1 with moderate disease). Eight patients expressed inflammatory cytokines (IL-1 alpha, IL-6, and/or IFN-gamma) in healthy skin. Higher cytokine mRNA in damaged vs healthy skin was also observed, suggesting the existence of an inflammation that predisposes healthy skin to develop overt disease. Up-regulated expression of MICA mRNA was observed in 8 patients. Although the pathogenesis of seborrhoeic dermatitis remains to be elucidated, expression of cytotoxicity-activating ligands (MICA), recruitment of NK cells, and a local pro-inflammatory microenvironment may facilitate the development of tissue injury.

Nevo de Duperrat sobre un nevo Spilus / Duperrat's nevus on a nevus spilus

Describimos un caso de nevo de Duperrat originado de un nevo Spilus )nevo lentiginosos moteado). Ponemos de relieve su asociación infrecuente y la importancia del diagnóstico diferencial con otras lesiones melanocíticas que pueden surgir de un nevo Spilus (como nevo de Spitz, el nevo azul y el melanoma maligno)

Nevo de Duperrat sobre un nevo Spilus / Duperrats nevus on a nevus spilus

Describimos un caso de nevo de Duperrat originado de un nevo Spilus )nevo lentiginosos moteado). Ponemos de relieve su asociación infrecuente y la importancia del diagnóstico diferencial con otras lesiones melanocíticas que pueden surgir de un nevo Spilus (como nevo de Spitz, el nevo azul y el melanoma maligno) (AU)

HLA haplotypes and class II molecular alleles in Argentinian patients with pemphigus vulgaris.

BACKGROUND: Pemphigus is a group of chronic blistering autoimmune diseases of which pemphigus vulgaris (PV) is the more frequent clinical form. The association of PV with the HLA serotypes suggests that there is a genetic predisposition to the disease. OBJECTIVES: To determine the frequency of class I and class II HLA antigens and the allelic variants of the class II HLA antigens, DR and DQ, in patients with PV and to determine the relative risk. METHODS: An observational, prospective, transverse, and controlled study carried out between 1995 and 1999. Forty-seven patients with a diagnosis of PV and a control sampling of 199 unselected individuals from the same ethnic group were included. The HLA alleles were determined by polymerase chain reaction. RESULTS: No significant associations were detected between HLA A, B, or C and our patients. The DR and DQ molecular alleles positively associated with PV were two different haplotypes: DRb1* 0402/DQb1* 0302 and DRb1* 1401/DQb1* 0503. CONCLUSIONS: In patients with the haplotype DRb1* 0402/DQb1* 0302 the affectation of 10%-30% of the corporal surface prevailed (ACS). In patients with DRb1* 1401/DRQb1* 0503, involvement of <10% of the ACS prevailed.

Gerodermia osteodisplásica / Geroderma osteodysplastica

La gerodermia osteodisplásica es una enfermedad genética poco frecuente que fue inicialmente descripta por Bamatter y colaboradores en una familia de origen suizo. Este autor reconoció la similitud de la misma con las creaciones de Walt Disney; como consecuencia de ello la llamaron enanismo de Walt Disney. Hasta el momento se han publicado veintiséis casos individuales provenientes de doce familias. Este síndrome presenta cambios en la piel, que sugieren un envejecimiento precoz, así como cambios óseos que incluyen la osteoporosis que puede estar asociada con fracturas y colapso vertebral. Presentamos el primer caso de gerodermia osteodisplásica en la Argentina

Gerodermia osteodisplásica / Geroderma osteodysplastica

La gerodermia osteodisplásica es una enfermedad genética poco frecuente que fue inicialmente descripta por Bamatter y colaboradores en una familia de origen suizo. Este autor reconoció la similitud de la misma con las creaciones de Walt Disney; como consecuencia de ello la llamaron enanismo de Walt Disney. Hasta el momento se han publicado veintiséis casos individuales provenientes de doce familias. Este síndrome presenta cambios en la piel, que sugieren un envejecimiento precoz, así como cambios óseos que incluyen la osteoporosis que puede estar asociada con fracturas y colapso vertebral. Presentamos el primer caso de gerodermia osteodisplásica en la Argentina (AU)

Factores predisponentes de sepsis en micosis fungoide / Risk factors of sepsis in mycosis fungoides

Introducción: La sepsis es la principal causa de mortalidad en pacientes con micosis fungoide (MF) y su aparición es favorecida por diversos factores. Objetivo: Evaluar la incidencia, etiología, factores predisponentes y evolución de la sepsis en pacientes con MF. Métodos: Se analizaron retrospectivamente en un período de 11 años (1990-2000) 62 pacientes con MF (14 con sepsis y 48 sin sepsis). Se investigaron en ambos grupos edad, sexo, clínica, neutropenia febril, compromiso de ganglios linfáticos, metástasis, células atípicas circulantes y estadificación. Resultados: 14/62 (22,5%) pacientes presentaron 21 episodios de sepsis: 15 atribuibles a Staphylococcus aureus (siete meticilinoresistente) y los restantes a Streptococcus pneumoniae y bacterias gramnegativas. Las principales fuentes de sepsis fueron úlceras cutáneas por infiltración linfomatosa (64,28%) y sepsis asociada a catéteres endovenosos (28,57%). Los 17/21 (80,95%) episodios de sepsis correlacionaron con un sitio localmente infectado. Como factores predisponentes a desarrollar sepsis mostraron una asociación significativa las ulceraciones cutáneas linfomatosas (p 5% (p < 0,02) y estadio IV (p < 0,001). El 57% de las septicemias fueron fatales antes del mes de finalizado el tratamiento antibiótico; de ellos, el 66% en estadio IV. Conclusión: La información presentada permite identificar los pacientes con MF que presentan alto riesgo de desarrollar sepsis: aquellos con eritrodermia y/o compromiso ganglionar, visceral o de sangre periférica y/o en estadio IV (AU)