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.

Expanding the clinical spectrum of dermal hyperneury: report of nine new cases and a review of the literature.

AIMS: Dermal hyperneury is defined as the hypertrophy of small nerves in the dermis. It has been described in a variety of settings. We present a series of nine new cases with a distinctive clinical presentation and review the existing literature. The aim of the study was to summarise the clinical, histopathological and immunohistochemical findings in a case series of dermal hyperneury with unique clinical presentation. METHODS AND RESULTS: Nine cases were identified from the referral practice of one of the authors. Clinical characteristics, including demographic details, were collated. The histopathological features and novel immunohistochemical findings were analysed. Four cases presented with multiple skin lesions. Clinical evaluation revealed no associated syndromic stigmata. The histology in all cases was that of dermal hyperneury. Immunohistochemistry for phosphatase and tensin homologue (PTEN) and RET was supportive of the lack of syndromic association. CONCLUSION: The presentation of dermal hyperneury with multiple cutaneous lesions and no syndromic associations is distinctive, and no study with PTEN and RET immunohistochemistry has previously been reported. Comparisons with recent reports of multiple non-syndromic mucocutaneous neuromas are discussed.

Morfea profunda solitaria: Una forma infrecuente de esclerodermia localizada en la infancia. Caso clínico / Solitary morphea profunda: An unusual form of localized scleroderma in childhood. Case report

La morfea profunda solitaria es una forma infrecuente de morfea localizada en la infancia. Se caracteriza clínicamente por la presencia de un área indurada, mal delimitada y asintomática, de localización paraespinal, que no tiende a la progresión ni se asocia con manifestaciones sistémicas. Histológicamente, muestra un engrasamiento e hialinización de las fibras de colágeno dérmico, junto con infiltrados de predominio linfohistiocitario en la dermis reticular y la hipodermis. Se presenta a una paciente de 7 años con una lesión solitaria en la región dorsal media izquierda, cuyos hallazgos clínicos, ecográficos e histológicos fueron compatibles con el diagnóstico de una morfea profunda solitaria.

Solitary morphea profunda is an unusual form of localized scleroderma in childhood. It is characterized by a single, poorly defined and indurate plaque often located on the upper trunk near the spine. Solitary morphea profunda is frequently asymptomatic and shows no associated systemic involvement. Histological examination reveals dense sclerosis of collagen and a marked lymphocytic infiltrate in the reticular dermis and subcutis. We report a 7-year-old girl with a solitary asymptomatic and sclerotic plaque on the back. Clinical, ultrasonographic and histological features were consistent with solitary morphea profunda.

[Solitary morphea profunda: An unusual form of localized scleroderma in childhood. Case report]. / Morfea profunda solitaria: Una forma infrecuente de esclerodermia localizada en la infancia. Caso clínico.

Solitary morphea profunda is an unusual form of localized scleroderma in childhood. It is characterized by a single, poorly defined and indurate plaque often located on the upper trunk near the spine. Solitary morphea profunda is frequently asymptomatic and shows no associated systemic involvement. Histological examination reveals dense sclerosis of collagen and a marked lymphocytic infiltrate in the reticular dermis and subcutis. We report a 7-year-old girl with a solitary asymptomatic and sclerotic plaque on the back. Clinical, ultrasonographic and histological features were consistent with solitary morphea profunda.

La morfea profunda solitaria es una forma infrecuente de morfea localizada en la infancia. Se caracteriza clínicamente por la presencia de un área indurada, mal delimitada y asintomática, de localización paraespinal, que no tiende a la progresión ni se asocia con manifestaciones sistémicas. Histológicamente, muestra un engrasamiento e hialinización de las fibras de colágeno dérmico, junto con infiltrados de predominio linfohistiocitario en la dermis reticular y la hipodermis. Se presenta a una paciente de 7 años con una lesión solitaria en la región dorsal media izquierda, cuyos hallazgos clínicos, ecográficos e histológicos fueron compatibles con el diagnóstico de una morfea profunda solitaria.

Anetodermic pilomatricoma: clinical, histopathologic, and sonographic findings.

Pilomatricoma is a benign cutaneous tumor originatingfrom hair matrix cells. Anetodermic changes inthe skin overlying pilomatricomas are sometimesreported, although their precise mechanisms remainunknown. We present an unusual case of anetodermicpilomatricoma on the upper extremity of a 17-yearoldboy and report its clinical, histopathologic, andsonographic findings.

Vasculopatía colágena cutánea: una causa infrecuente de telangiectasias generalizadas / Cutaneous collagenous vasculopathy: A rare cause of generalized cutaneous telangiectasia

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Síndrome de Rowell: las 2 caras de la verdad / Rowell's syndrome: the two sides of the truth

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Hemangiopericitoma tipo senonasal. Presentación de un caso y revisión de la literatura / Sinonasal-type hemangiopericytoma. A report of one case and review of the literature

Según la reciente clasificación de la OMS para los tumores de partes blandas, las lesiones de tipo pericítico, han sufrido una modificación tanto en su denominación, como en su definición histopatológica. Entre dichas lesiones, sin embargo, el hemangiopericitoma tipo senonasal, sigue estando considerado prototipo de tumor pericítico. Esta neoplasia, específica del tracto aéreo superior, tiene una ligera preferencia por el sexo femenino y, aunque puede aparecer a cualquier edad, es mas frecuente en la sexta y la séptima décadas de la vida. La presentación clínica mas frecuente es la obstrucción aérea y/o epistaxis, y no se conocen factores etiológicos asociados a su desarrollo. Muestra en general un curso indolente, y la recurrencia local está habitualmente asociada a una resección quirúrgica incompleta. En ocasiones, puede tener comportamiento agresivo y producir metástasis a distancia. Presentamos un caso de este tumor, típico tanto en su presentación clínica como en su histopatología. La lesión asentaba en la fosa nasal izquierda y los senos paranasales homolaterales de una mujer de 85 años de edad, con historia de epistaxis de repetición. El tumor mostraba un crecimiento polipoide, y se resecó en su totalidad mediante cirugía endoscópica. Microscópicamente, mostraba un infiltrado celular denso y las células poseían núcleos pequeños y ovoideos, y citoplasma rosado, de límites poco definidos. Existían abundantes vasos y muchos de ellos mostraban una llamativa hialinización perivascular. No se vió atipia y la actividad mitósica era muy baja. Con técnicas de IHQ se observó positividad para la vimentina, la actina muscular específica, la actina de músculo liso y el CD34.Tras dos años de seguimiento, la paciente se encuentra bien y sin enfermedad residual

The new WHO classification of soft tissue tumours, has introduced some changes in the nomenclatura of pericytic lesions but also in its clinicopathologic definition. Among this kind of lesions, sinonasal-type hemangiopericytoma is considered a tumor with true pericytic differentiation. It is a upper aero-digestive tract neoplasm, slightly commoner in women than men, and occurs in all age groups but it is more commonly seen in the 6th and 7th decades of life. The most frequent clinical presentation is airway obstruction and/or epistaxis and there are no known etiologic factors linked with its development. In general, sinonasal-type hemangiopericytoma is an indolent tumor and local recurrence results from inadequate surgical excision. Tumors with an aggressive behaviour, locally destructive or metastatic, have been occasionally reported in the literature. We describe a case of this uncommon tumor with the characteristics clinical and pathologic findings in this lesion. A 85-year-old woman presented with a 2-year history of epistaxis. The tumor involved the left nasal cavity and also the homolateral paranasal sinuses, it was polypoid and the patient was treated by endonasal endoscopic surgery with a complete resection. Microscopically, it was constituted from sheets of uniform cells with a ovoid nuclei and indistinct cytoplasm distributed around characteristic hyalinized vascular spaces. Nuclear pleomorphism was absent and mitotic figures were identified a very low rate. The tumor was immunoreactive with vimentin, muscle specific actin, smooth muscle actin and CD34. After two-years of follow-up, the patient is alive and without evidence of disease (AU)