Still disease with persistent atypical dermatomyositis-like skin eruption: two cases associated with macrophage activation syndrome.

The typical rash of Still disease is an asymptomatic, salmon-coloured, macular, or maculopapular eruption that appears along with the fever spikes and fades when the body temperature drops. Although not included in the diagnostic criteria, there are other frequent, persistent, pruritic and polymorphic skin manifestations of Still disease that have distinctive clinical features and specific histological findings. Among these atypical persistent pruritic eruptions (PPEs), periorbital erythema and linear flagellate papules and plaques can resemble the cutaneous manifestations of dermatomyositis (DM). The presence of these lesions in adult-onset Still disease has been associated with worse prognosis and higher mortality rates, perhaps due to the development of a secondary macrophage activation syndrome (MAS). We report two cases of Still disease with persistent atypical DM-like eruption, one in a paediatric patient (a very underreported finding) and the other in an adult. Both cases developed a secondary MAS.

Expression of Peripheral Node Addressins by Plasmacytic Plaque of Children, APACHE, TRAPP, and Primary Cutaneous Angioplasmacellular Hyperplasia.

High-endothelial venules are a common feature of 3 types of cutaneous pseudolymphomas: pretibial lymphoplasmacytic plaque (PLP) of children, acral pseudolymphomatous angiokeratoma of children (APACHE), and T-cell rich angiomatoid polypoid pseudolymphoma (TRAPP). In addition, primary cutaneous angioplasmacellular hyperplasia (PCAH) overlaps with these other 3 conditions. We intend to study the expression of peripheral node addressins in PLP, APACHE, TRAPP, and PCAH. We studied 1 case of PLP, 2 cases of APACHE, 2 cases of TRAPP, and 2 cases of PCAH. Immunostainings for MECA-79 and WT-1 were obtained in all cases. All cases showed a dense lymphohistiocytic dermal inflammatory infiltrate with abundant plasma cells. In addition, HEV were prominent in all cases. Cases of PLP, APACHE, and TRAPP expressed MECA-1. Cases of PCAH did not express MECA-1. Although PLP, APACHE, and TRAPP seem to fall under the same morphologic spectrum with different clinical representations, PCAH seems to be a different entity, with histopathologic peculiarities and a different immunophenotype.

[Erythema induratum of Bazin associated to Mycobacterium tuberculosis infection]. / Eritema indurado de Bazin asociado a infección por Mycobacterium tuberculosis.

Erythema induratum of Bazin is uncommon in children. We report the case of a 12 year-old girl from China, referred by her pediatrician with long lasting lesions of erythema nodosum and a positive tuberculin skin test (20 mm). She had no other symptoms. Physical examination showed purple nodular lesions located on both legs, mostly on the pretibial areas but also on her calves. A chest X-ray was normal. A skin biopsy was obtained, showing granulomata and septolobular panniculitis. Attempts to identify Mycobacterium tuberculosis by culture and PCR were negative. M. tuberculosis was isolated in one of three gastric aspirate samples. She was treated with isoniazid, rifampin, pyrazinamide and ethambutol. There was complete remission of the lesions. This case highlights the importance of considering erythema induratum in atypical cases of erythema nodosum and the need to perform a thorough investigation in search of active tuberculosis.

Eritema indurado de Bazin asociado a infección por Mycobacterium tuberculosis / Erythema induratum of Bazin associated to Mycobacterium tuberculosis infection

El eritema indurado de Bazin es poco frecuente en la edad pediátrica. Presentamos el caso de una niña de 12 años procedente de China remitida para estudio por sospecha de eritema nudoso de larga evolución y prueba de tuberculina positiva (20 mm). La paciente no presentaba otros síntomas. En la exploración física se observaban nódulos violáceos, localizados en ambas regiones pretibiales, pero también en ambas pantorrillas. La radiografía de tórax fue normal. Se obtuvo muestra para biopsia, que mostró una paniculitis de predominio lobulillar con presencia de granulomas. La detección de micobacterias mediante cultivo y reacción en cadena de la polimerasa en la muestra fue negativa. Se aisló Mycobacterium tuberculosis en una de tres muestras de jugo gástrico. Se trató con isoniacida, rifampicina, pirazinamida y etambutol con curación de las lesiones. Este caso resalta la importancia de sospechar eritema indurado en las formas atípicas de eritema nudoso y de investigar exhaustivamente la existencia de una tuberculosis activa en estos casos.

Erythema induratum of Bazin is uncommon in children. We report the case of a 12 year-old girl from China, referred by her pediatrician with long lasting lesions of erythema nodosum and a positive tuberculin skin test (20 mm). She had no other symptoms. Physical examination showed purple nodular lesions located on both legs, mostly on the pretibial areas but also on her calves. A chest X-ray was normal. A skin biopsy was obtained, showing granulomata and septolobular panniculitis. Attempts to identify Mycobacterium tuberculosis by culture and PCR were negative. M. tuberculosis was isolated in one of three gastric aspirate samples. She was treated with isoniazid, rifampin, pyrazinamide and ethambutol. There was complete remission of the lesions. This case highlights the importance of considering erythema induratum in atypical cases of erythema nodosum and the need to perform a thorough investigation in search of active tuberculosis.

Eritema indurado de Bazin asociado a infección por Mycobacterium tuberculosis / Erythema induratum of Bazin associated to Mycobacterium tuberculosis infection

El eritema indurado de Bazin es poco frecuente en la edad pediátrica. Presentamos el caso de una niña de 12 años procedente de China remitida para estudio por sospecha de eritema nudoso de larga evolución y prueba de tuberculina positiva (20 mm). La paciente no presentaba otros síntomas. En la exploración física se observaban nódulos violáceos, localizados en ambas regiones pretibiales, pero también en ambas pantorrillas. La radiografía de tórax fue normal. Se obtuvo muestra para biopsia, que mostró una paniculitis de predominio lobulillar con presencia de granulomas. La detección de micobacterias mediante cultivo y reacción en cadena de la polimerasa en la muestra fue negativa. Se aisló Mycobacterium tuberculosis en una de tres muestras de jugo gástrico. Se trató con isoniacida, rifampicina, pirazinamida y etambutol con curación de las lesiones. Este caso resalta la importancia de sospechar eritema indurado en las formas atípicas de eritema nudoso y de investigar exhaustivamente la existencia de una tuberculosis activa en estos casos.(AU)

Erythema induratum of Bazin is uncommon in children. We report the case of a 12 year-old girl from China, referred by her pediatrician with long lasting lesions of erythema nodosum and a positive tuberculin skin test (20 mm). She had no other symptoms. Physical examination showed purple nodular lesions located on both legs, mostly on the pretibial areas but also on her calves. A chest X-ray was normal. A skin biopsy was obtained, showing granulomata and septolobular panniculitis. Attempts to identify Mycobacterium tuberculosis by culture and PCR were negative. M. tuberculosis was isolated in one of three gastric aspirate samples. She was treated with isoniazid, rifampin, pyrazinamide and ethambutol. There was complete remission of the lesions. This case highlights the importance of considering erythema induratum in atypical cases of erythema nodosum and the need to perform a thorough investigation in search of active tuberculosis.(AU)

Eritema indurado de Bazin asociado a infección por Mycobacterium tuberculosis / Erythema induratum of Bazin associated to Mycobacterium tuberculosis infection

El eritema indurado de Bazin es poco frecuente en la edad pediátrica. Presentamos el caso de una niña de 12 años procedente de China remitida para estudio por sospecha de eritema nudoso de larga evolución y prueba de tuberculina positiva (20 mm). La paciente no presentaba otros síntomas. En la exploración física se observaban nódulos violáceos, localizados en ambas regiones pretibiales, pero también en ambas pantorrillas. La radiografía de tórax fue normal. Se obtuvo muestra para biopsia, que mostró una paniculitis de predominio lobulillar con presencia de granulomas. La detección de micobacterias mediante cultivo y reacción en cadena de la polimerasa en la muestra fue negativa. Se aisló Mycobacterium tuberculosis en una de tres muestras de jugo gástrico. Se trató con isoniacida, rifampicina, pirazinamida y etambutol con curación de las lesiones. Este caso resalta la importancia de sospechar eritema indurado en las formas atípicas de eritema nudoso y de investigar exhaustivamente la existencia de una tuberculosis activa en estos casos.(AU)

Erythema induratum of Bazin is uncommon in children. We report the case of a 12 year-old girl from China, referred by her pediatrician with long lasting lesions of erythema nodosum and a positive tuberculin skin test (20 mm). She had no other symptoms. Physical examination showed purple nodular lesions located on both legs, mostly on the pretibial areas but also on her calves. A chest X-ray was normal. A skin biopsy was obtained, showing granulomata and septolobular panniculitis. Attempts to identify Mycobacterium tuberculosis by culture and PCR were negative. M. tuberculosis was isolated in one of three gastric aspirate samples. She was treated with isoniazid, rifampin, pyrazinamide and ethambutol. There was complete remission of the lesions. This case highlights the importance of considering erythema induratum in atypical cases of erythema nodosum and the need to perform a thorough investigation in search of active tuberculosis.(AU)

Síndrome auriculotemporal de Frey. Un caso de presentación bilateral en un lactante / Frey auriculotemporal syndrome. A bilateral case in a child

El síndrome auriculotemporal o síndrome de Frey se caracteriza por la aparición de enrojecimiento, hiperhidrosis, o ambos, en la zona inervada por elnervio auriculotemporal como respuesta a un estímulo gustativo desencadenado por la ingesta de distintos alimentos. Por este motivo puede confundirseen ocasiones con alergias alimentarias. Se debe generalmente a una lesión del nervio auriculotemporal, si bien en ocasiones no existe ningún antecedentetraumático. Su aparición en la infancia y su presentación bilateral, como el caso que presentamos a continuación, son muy poco frecuentes

Frey syndrome or auriculotemporal nerve syndrome manifests as flushing, sweating, or both, localized to the distribution of the auriculotemporal nerve,in response to gustatory stimuli. Ocasionally it may be misinterpreted as foog allergy. It usually occurs as a result of injury to auriculotemporal nerve, butthere are cases with no known previous trauma. It is rarely described in children as well as the bilateral involvement, as the case we presented

[Becker's nevus associated with epidermal nevus: another example of twin spotting?]. / Nevo de Becker asociado a nevo epidérmico: 'un ejemplo más de "manchas gemelas"?

Linear epidermal nevi are believed to be caused by an autosomal dominant lethal mutation that can only be expressed by mosaicism. Becker's nevus can be explained by paradominant inheritance which is only manifested clinically by an acquired loss of heterozygosity. We present the case of a 16-year-old female with an epidermal nevus located on the left side of the neck, and also a Becker's nevus located on the ipsilateral shoulder. It is interesting to speculate that this supposed double mosaicism could be another example of "twin spotting" or non-allelic didymosis, although the possibility that this is a chance association cannot be ruled out, as the lesions are not closely associated.

Nevo de Becker asociado a nevo epidérmico: ¿un ejemplo más de «manchas gemelas»? / Becker’s nevus associated with epidermal nevus: another example of twin spotting?

El nevo epidérmico lineal se presume debido a un gen letal autosómico dominante que únicamente puede expresarse en situación de mosaicismo. El nevo de Becker puede explicarse mediante una herencia paradominante que sólo se manifiesta clínicamente por una pérdida adquirida de heterozigosidad. Presentamos una mujer de 16 años con un nevo epidérmico localizado en la zona izquierda del cuello y además un nevo de Becker localizado en el hombro ipsolateral. Resulta interesante explicar esta observación de un supuesto doble mosaicismo en términos de un ejemplo más de «manchas gemelas» o didimosis no alélica aunque no puede descartarse que dicha asociación se deba al azar, ya que las lesiones no se encuentran en íntima aposición

Linear epidermal nevi are believed to be caused by an autosomal dominant lethal mutation that can only be expressed by mosaicism. Becker's nevus can be explained by paradominant inheritance which is only manifested clinically by an acquired loss of heterozygosity. We present the case of a 16-year-old female with an epidermal nevus located on the left side of the neck, and also a Becker's nevus located on the ipsilateral shoulder. It is interesting to speculate that this supposed double mosaicism could be another example of «twin spotting» or non-allelic didymosis, although the possibility that this is a chance association cannot be ruled out, as the lesions are not closely associated

Síndrome de Kindler: aportación de un caso / Kindler syndrome: presentation of a case

El síndrome de Kindler, es una enfermedad muy poco frecuente debida a mutaciones que originan defectos en la unión actina-matriz extracelular. Suele cursar con ampollas acrales desde el nacimiento en zonas más expuestas a los traumatismos, fotosensibilidad marcada que mejora con la edad y desarrollo de poiquilodermia y atrofia cutánea. Con relativa frecuencia se describe afectación de mucosas y degeneración maligna

Kindler syndrome is a very rare disease caused by mutations resulting in defects in the extracellular matrix-actin link. It usually presents with acral blistering from birth in trauma-prone areas, pronounced photosensitivity that improves with age and the development of poikiloderma and cutaneous atrophy. Mucosal involvement and degeneration have been described with relative frequency

Prurigo pigmentoso / Prurigo pigmentosa

El prurigo pigmentoso es una dermatosis inflamatoria infrecuente de etiología desconocida caracterizada por episodios recurrentes de pápulas eritematosas y pruriginosas que evolucionan hacia una intensa pigmentación reticulada. Se ha referido más frecuentemente en mujeres adultas jóvenes en Japón. Sólo se han descrito fuera de Japón unos 30 casos. Describimos una paciente española de 32 años que desarrolló una dermatosis pruriginosa con las características clínicas y hallazgos histopatológicos del prurigo pigmentoso. Se revisan las características epidemiológicas, clínicas e histopatológicas de esta peculiar enfermedad cutánea

Prurigo pigmentosa is an infrequent inflammatory dermatosis of unknown etiology, characterized by recurrent episodes of pruritic erythematous papules which develop into intense reticulated pigmentation. It has been referenced most frequently in young women in Japan. Only thirty cases have been described outside of Japan. We describe a 32-year-old Spanish female who developed a pruritic dermatosis with the clinical characteristics and histopathological findings of prurigo pigmentosa. We review the epidemiological, clinical and histopathological characteristics of this peculiar skin disease

"Manos de mecánico" como signo cutáneo característico del síndrome de antisintetasa / Mechanic´s hands: a characteristic cutaneous sign of antisynthetase syndrome

Las «manos de mecánico» constituyen un signo cutáneo característico de miositis inflamatoria idiopática. Se describe el caso de un paciente varón de 61 años que fue diagnosticado en el año 1999 de polimiositis idiopática y enfermedad pulmonar intersticial inespecífica y que 3 años después desarrolló unas lesiones hiperqueratósicas, escamosas y fisuradas en las caras laterales y caras palmares de los primeros 3 dedos de ambas manos, escasamente pruriginosas. La presencia en el suero del paciente del anticuerpo antisintetasa anti-Jo-1, el hallazgo de lesiones cutáneas de «manos de mecánico» y el resto de manifestaciones clínicas sistémicas del paciente permiten establecer el diagnóstico de «síndrome antisintetasa»

«Mechanic’s hands» are a characteristic cutaneous sign of idiopathic inflammatory myositis. We describe the case of a 61-year-old male patient who was diagnosed with idiopathic polymyositis and non-specific interstitial lung disease in 1999, and three years later developed scaly, fissured hyperkeratotic lesions on the lateral and palmar surfaces of the first three fingers of both hands, with little pruritus. The presence of the anti-Jo-1 antisynthetase antibody in the patient’s serum, the finding of skin lesions characteristic of «mechanic’s hands» and the patient’s other systemic clinical manifestations made it possible to establish the diagnosis of «antisynthetase syndrome»

[Prurigo pigmentosa]. / Prurigo pigmentoso.

Prurigo pigmentosa is an infrequent inflammatory dermatosis of unknown etiology, characterized by recurrent episodes of pruritic erythematous papules which develop into intense reticulated pigmentation. It has been referenced most frequently in young women in Japan. Only thirty cases have been described outside of Japan. We describe a 32-year-old Spanish female who developed a pruritic dermatosis with the clinical characteristics and histopathological findings of prurigo pigmentosa. We review the epidemiological, clinical and histopathological characteristics of this peculiar skin disease.

[Kindler syndrome: presentation of a case]. / Síndrome de Kindler: aportación de un caso.

Kindler syndrome is a very rare disease caused by mutations resulting in defects in the extracellular matrix-actin link. It usually presents with acral blistering from birth in trauma-prone areas, pronounced photosensitivity that improves with age and the development of poikiloderma and cutaneous atrophy. Mucosal involvement and degeneration have been described with relative frequency.

[Mechanic's hands: a characteristic cutaneous sign of antisynthetase syndrome]. / "Manos de mecánico" como signo cutáneo característico del síndrome antisintetasa.

"Mechanic's hands" are a characteristic cutaneous sign of idiopathic inflammatory myositis. We describe the case of a 61-year-old male patient who was diagnosed with idiopathic polymyositis and non-specific interstitial lung disease in 1999, and three years later developed scaly, fissured hyperkeratotic lesions on the lateral and palmar surfaces of the first three fingers of both hands, with little pruritus. The presence of the anti-Jo-1 antisynthetase antibody in the patient's serum, the finding of skin lesions characteristic of "mechanic's hands" and the patient's other systemic clinical manifestations made it possible to establish the diagnosis of "antisynthetase syndrome."

Granulomas sarcoideos cutáneos desarrollados alrededor de cuerpos extraños durante el tratamiento con interferón Alfa y ribavirina en un paciente con hepatitis C crónica / Cutaneous sarcoid granulomas which developed around foreign bodies in a patient with chronic hepatitis C during treatment with Alpha interferon and ribavirin

La sarcoidosis es una enfermedad multisistémica caracterizada por la formación de granulomas epitelioides no caseificantes en distintos órganos y tejidos, entre ellos la piel. Su etiología aún permanece sin aclarar pero, probablemente, su origen sea multifactorial. En el caso que se presenta se imbrican varios de los factores etiopatogénicos que se han involucrado en la formación de los granulomas sarcoideos, como las alteraciones inmunológicas, en nuestro paciente relacionadas con el tratamiento con interferón y ribavirina, los cuerpos extraños y los agentes infecciosos (AU)

Dermatosis eosinofílicas (y II) / No disponible

No disponible