Angiomas en penacho en la infancia. Serie de 9 casos y revisión de la literatura / Tufted angiomas in childhood: A series of 9 cases and a literature review

INTRODUCCIÓN: El angioma en penacho o tufted angioma (TA) es una tumoración vascular benigna poco frecuente, que suele aparecer en la infancia, aunque existen casos de aparición en la edad adulta. Su presentación clínica es muy variable. Se manifiesta típicamente como una mácula, pápula o nódulo eritematovioláceo en el tronco o el cuello. Histológicamente, se caracteriza por agregados de lóbulos angiomatosos en la dermis formando pequeños penachos de capilares. PACIENTES Y MÉTODOS: Estudio retrospectivo observacional de los casos diagnosticados de TA en los últimos 20 años en nuestro centro. RESULTADOS: Presentamos un total de 9 casos de angiomas en penacho en la infancia. El 77,7% de los casos fueron congénitos, lo que representa una frecuencia superior a la descrita previamente. Nuestros pacientes presentaron regresión espontánea en el 55,5% de los casos, siendo más frecuente en el grupo de TA congénitos. A diferencia de las otras series descritas en la literatura, observamos un mayor porcentaje de pacientes con regresión espontánea, un predominio femenino (6 de los 9 niños) y una localización más frecuente en miembros los superiores. Ninguno de nuestros pacientes presentó fenómeno de Kasabach-Merritt. CONCLUSIONES: Dada la alta tasa de involución espontánea en TA congénitos o tempranos, en ausencia de otras complicaciones, la vigilancia sería una buena opción de manejo, monitorizando estrechamente al paciente

INTRODUCTION: Tufted angioma (TA) is a rare benign vascular tumor that mostly appears during infancy or early childhood, although there are cases reported in adults. Clinical presentation and evolution of TA can vary. Histologically, it takes on a classic appearance of vascular tufts ('cannon ball' like appearance). PATIENTS AND METHODS: A retrospective observational study was conducted that included all patients diagnosed with TA at our center in the last 20 years. Results A series of 9 cases of tufted angioma in childhood are presented, 77.7% of which were congenital. This represents a frequency higher than previously described. Spontaneous regression was observed in 55.5% of the cases, and was more frequent in the congenital TA group. Unlike other TA series reported in the literature, a higher proportion of patients with spontaneous regression was observed in this series, with a higher prevalence in females (6 out of 9 children) and predominantly located in the upper limbs. None of our patients had Kasabach-Merritt phenomenon. CONCLUSIONS: There are many ways of treating TA, but none are uniformly effective. Given the high rate of spontaneous regression in congenital or early TA, we suggest that, in the absence of other complications, monitoring would be a good option for management

[Tufted angiomas in childhood: A series of 9 cases and a literature review]. / Angiomas en penacho en la infancia. Serie de 9 casos y revisión de la literatura.

INTRODUCTION: Tufted angioma (TA) is a rare benign vascular tumor that mostly appears during infancy or early childhood, although there are cases reported in adults. Clinical presentation and evolution of TA can vary. Histologically, it takes on a classic appearance of vascular tufts ("cannon ball" like appearance). PATIENTS AND METHODS: A retrospective observational study was conducted that included all patients diagnosed with TA at our center in the last 20 years. RESULTS: A series of 9 cases of tufted angioma in childhood are presented, 77.7% of which were congenital. This represents a frequency higher than previously described. Spontaneous regression was observed in 55.5% of the cases, and was more frequent in the congenital TA group. Unlike other TA series reported in the literature, a higher proportion of patients with spontaneous regression was observed in this series, with a higher prevalence in females (6 out of 9 children) and predominantly located in the upper limbs. None of our patients had Kasabach-Merritt phenomenon. CONCLUSIONS: There are many ways of treating TA, but none are uniformly effective. Given the high rate of spontaneous regression in congenital or early TA, we suggest that, in the absence of other complications, monitoring would be a good option for management.

Síndrome de Buschke-Ollendorff / Buschke-Ollendorff syndrome

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Signo del collar de pelo asociado a aplasia cutis congénita del cuero cabelludo / Hair collar sign associated with scalp aplasia cutis congenita

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Cloromas cutáneos como primera manifestación de leucemia mielode aguda en un niño / Cutaneous chloromas as the presenting feature of acute myeloid leukemia in a child

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Phacomatosis pigmentokeratotica: a 20-year follow-up with malignant degeneration of both nevus components.

Phacomatosis pigmentokeratotica is a rare syndrome defined by the association of an organoid nevus occasionally with sebaceous differentiation, a speckled lentiginous nevus, and other extracutaneous anomalies. The disorder is a consequence of the so-called twin spot genetic mechanism. We describe the first occurrence involving malignant degeneration of both nevus components, giving rise to three basal cell carcinomas over the sebaceous nevus and a malignant melanoma of the superficial spreading type over the speckled lentiginous nevus. This observation, in concert with the other instances reported in the literature, points to the need for adequate patient follow-up to ensure early detection and treatment of any possible associated malignant degeneration.

Lymphangioma circumscriptum: an example of Whimster's hypothesis.

Lymphangioma circumscriptum is an uncommon hamartomatous malformation that is not only localized to an area of skin, but also spreads to subcutaneous tissue and muscle (Whimster's hypothesis). We describe a 7-year-old boy with a bluish plaque on the left side of the back and abdomen with clusters of translucent vesicles (frog spawn). Magnetic resonance imaging showed the lymphangioma had spread to muscles and even fat adjacent to the left kidney. We decided against radical surgery as it would have been too aggressive, and limited excision can produce new surface lymphangiomas.

Hiperqueratosis epidermolítica en forma de mosaico / Mosaic form of epidermolytic hyperkeratosis

Presentamos el caso de una niña de 6 años de edad con lesiones hiperqueratósicas distribuidas siguiendo las líneas de Blaschko en todo el hemicuerpo izquierdo. La biopsia cutánea demostró un patrón de hiperqueratosis epidermolítica. Se trató con acitretino a dosis de 0,5 mg/ kg/d con buena respuesta. Recordamos la importancia de este mosaicismo por la posibilidad de tener hijos afectados de eritrodermia ictiosiforme congénita ampollosa en la descendencia. (AU)

[Neutrophilic dermatosis in ulcerative colitis occurring in advanced age]. / Dermatosis neutrofílicas en colitis ulcerosa de comienzo en edad avanzada.

The Neutrophilic dermatosis (ND) is considered as an independent entity with diverse clinical manifestations among which there are: gangrenous pyoderma, nodous erythema, Sweets Syndrome, vesiculopustula eruptions associated to ulcerous colitis and intestinal short circuit syndrome with or without short circuit. Histologically, they are characterized by infiltration of polymorphonuclear neutrophils, generally at the dermic level, but also at the epidermic. They are usually associated to systemic diseases, especially to chronic intestinal inflammatory disease. Our aim was to describe two forms of clinical presentation of neutrophilic dermatosis: gangrenous pyoderma and vesiculopustula eruption, associated to ulcerous colitis starting at advances ages.

Primary soft tissue lymphoma associated with mesenteric lipodystrophy.

Skin involvement in non-Hodgkin lymphoma is usually specific and may precede systemic manifestations of the disease by months or even years. We report the case of a man with an inguinal plaque lesion of 1 year's evolution, diagnosed as polymorphous lymphoplasmocytoid lymphoma (immunocytoma). The evaluation of spread only revealed mesenteric lipodystrophy, whose association with lymphoid processes is more common than would be expected.

[Sweet's syndrome. Its association with chronic inflammatory bowel disease]. / Síndrome de Sweet. Asociado a enfermedad intestinal inflamatoria crónica.

Sweet's syndrome, or febrile neutrophilic dermatosis, is a disease first described by Sweet R.D. in 1964 as a dermatologic disease. Subsequently, it has been associated to several disease. One of those rarely describe is the association to chronic intestinal inflammatory disease. We reviewed the cases studied in our hospital since 1980 and found two cases associated to chronic intestinal inflammatory disease. We recommend the carrying out of gastrointestinal studies in patients afflicted by Sweet's syndrome to detect its association.

[Kaposi sarcoma and Hodgkin disease]. / Sarcoma de Kaposi y enfermedad de Hodgkin.

Kaposi's sarcoma is frequently associated to other primary neoplasias, especially those arising from mononuclear-phagocytic system. A case of Kaposi's sarcoma associated to Hodgkin's disease is presented.

[Plasma exchange in dermatology: apropos of a case of dermatomyositis]. / Recambio plasmático en dermatología: a propósito de un caso de dermatomiositis.

We present a 45 years old male with dermatomyositis refractory to eight months of standard treatment who responded to plasma exchange. The efficacy of the procedure is estimated from clinical follow-up (increase in strength) and serum creatine-kinase activity (decrease from 3,380 to 44 u/ml.) after five sessions of plasma exchange. The therapeutic indications for PE are reviewed in dermatological diseases with emphasis on cases where the response to steroids or cytotoxic drugs is inadequate.