Update on the Diagnosis and Management of Desmoplastic Melanoma. / Actualización en el diagnóstico y manejo del melanoma desmoplásico.

Desmoplastic melanoma (DM) accounts for 0.4% to 4% of all melanomas. These skin tumors are mainly formed by amelanotic spindled melanocytes immersed in an abundant collagen stroma and are classified as pure when the desmoplastic component accounts for at least 90% of the invasive tumor and as mixed or combined otherwise. DMs are more common in men (male to female ratio, 1.7 to 2:1), and the mean age at diagnosis is 66 to 69 years. The tumors tend to occur in chronically sun-exposed areas, often in association with lentigo maligna, and are difficult to recognize because they can resemble a scar, presenting as a firm, unpigmented papule or plaque with poorly defined borders. DMs also have a strong tendency to recur locally, and pure variants rarely spread to the lymph nodes. Nonetheless, recently published series suggest that patients with DM have a similar prognosis to those with nondesmoplastic melanoma of the same thickness. The clinical management of DM varies in certain aspects from that of other melanomas and is reviewed in this article.

Actualización en el diagnóstico y manejo del melanoma desmoplásico / Update on the Diagnosis and Management of Desmoplastic Melanoma

El melanoma desmoplásico (MD) representa entre el 0,4-4% de todos los melanomas. Se presenta como un tumor constituido predominantemente por melanocitos fusiformes amelanóticos inmersos en un estroma colágeno abundante. Se clasifica en MD puro o mixto, basándose en la proporción de melanoma desmoplásico frente a la del melanoma no desmoplásico presente en el tumor infiltrante. En el MD puro el componente desmoplásico representa más del 90% del melanoma infiltrante mientras que, en el MD combinado o mixto, el componente desmoplásico representa menos del 90%. El MD es más frecuente en varones (ratio 1,7-2:1); la edad media al diagnóstico oscila entre 66-69 años y suele localizarse en áreas de fotoexposición crónica, a menudo asociado a un lentigo maligno. Su reconocimiento clínico es difícil ya que se presenta como una pápula o placa no pigmentada, indurada y de bordes mal definidos, que recuerda a una cicatriz. El MD es un tumor con una alta tendencia a la recurrencia local y en el caso del MD puro, una baja tendencia a la diseminación ganglionar. Sin embargo, en las series más contemporáneas, su pronóstico global parece ser similar al de melanomas no desmoplásicos (MND) del mismo grosor. Su abordaje clínico posee algunos matices diferenciales, en comparación al resto de melanomas, que se revisan en el presente trabajo (AU)

Desmoplastic melanoma (DM) accounts for 0.4% to 4% of all melanomas. These skin tumors are mainly formed by amelanotic spindled melanocytes immersed in an abundant collagen stroma and are classified as pure when the desmoplastic component accounts for at least 90% of the invasive tumor and as mixed or combined otherwise. DMs are more common in men (male to female ratio, 1.7 to 2:1), and the mean age at diagnosis is 66 to 69 years. The tumors tend to occur in chronically sun-exposed areas, often in association with lentigo maligna, and are difficult to recognize because they can resemble a scar, presenting as a firm, unpigmented papule or plaque with poorly defined borders. DMs also have a strong tendency to recur locally, and pure variants rarely spread to the lymph nodes. Nonetheless, recently published series suggest that patients with DM have a similar prognosis to those with nondesmoplastic melanoma of the same thickness. The clinical management of DM varies in certain aspects from that of other melanomas and is reviewed in this article (AU)

[Translated article] Update on the Diagnosis and Management of Desmoplastic Melanoma / Actualización en el diagnóstico y manejo del melanoma desmoplásico

Desmoplastic melanoma (DM) accounts for 0.4% to 4% of all melanomas. These skin tumors are mainly formed by amelanotic spindled melanocytes immersed in an abundant collagen stroma and are classified as pure when the desmoplastic component accounts for at least 90% of the invasive tumor and as mixed or combined otherwise. DMs are more common in men (male to female ratio, 1.7 to 2:1), and the mean age at diagnosis is 66 to 69 years. The tumors tend to occur in chronically sun-exposed areas, often in association with lentigo maligna, and are difficult to recognize because they can resemble a scar, presenting as a firm, unpigmented papule or plaque with poorly defined borders. DMs also have a strong tendency to recur locally, and pure variants rarely spread to the lymph nodes. Nonetheless, recently published series suggest that patients with DM have a similar prognosis to those with nondesmoplastic melanoma of the same thickness. The clinical management of DM varies in certain aspects from that of other melanomas and is reviewed in this article (AU)

El melanoma desmoplásico (MD) representa entre el 0,4-4% de todos los melanomas. Se presenta como un tumor constituido predominantemente por melanocitos fusiformes amelanóticos inmersos en un estroma colágeno abundante. Se clasifica en MD puro o mixto, basándose en la proporción de melanoma desmoplásico frente a la del melanoma no desmoplásico presente en el tumor infiltrante. En el MD puro el componente desmoplásico representa más del 90% del melanoma infiltrante mientras que, en el MD combinado o mixto, el componente desmoplásico representa menos del 90%. El MD es más frecuente en varones (ratio 1,7-2:1); la edad media al diagnóstico oscila entre 66-69 años y suele localizarse en áreas de fotoexposición crónica, a menudo asociado a un lentigo maligno. Su reconocimiento clínico es difícil ya que se presenta como una pápula o placa no pigmentada, indurada y de bordes mal definidos, que recuerda a una cicatriz. El MD es un tumor con una alta tendencia a la recurrencia local y en el caso del MD puro, una baja tendencia a la diseminación ganglionar. Sin embargo, en las series más contemporáneas, su pronóstico global parece ser similar al de melanomas no desmoplásicos (MND) del mismo grosor. Su abordaje clínico posee algunos matices diferenciales, en comparación al resto de melanomas, que se revisan en el presente trabajo (AU)

Múltiples pápulas en ambas manos / Múltiple Paules on Both Hands

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Alopecia frontal fibrosante en el varón: presentación de 12 casos y revisión de la literatura / Frontal fibrosing alopecia in men: presentations in 12 cases and a review of the literature

INTRODUCCIÓN: La alopecia frontal fibrosante (AFF) es una alopecia cicatricial caracterizada por el retroceso de la línea de implantación del pelo, asociada a alopecia de cejas. Habitualmente afecta a mujeres en edad posmenopáusica, siendo mucho menos prevalente en varones. OBJETIVO: Describir las características clínicas de la AFF en los hombres estudiados y compararlos con los datos recogidos en la literatura. MATERIAL Y MÉTODOS: Se realizó un estudio descriptivo de los varones diagnosticados de AFF en nuestro Servicio, desde enero del 2010 hasta diciembre del 2015. Se recogieron los datos demográficos, las características clínicas y los tratamientos realizados. RESULTADOS: Se reclutó a 12 pacientes. La edad media fue de 75 años. La alopecia fue el motivo de consulta únicamente en 4 pacientes. El retroceso medio de la línea de implantación del pelo fue de 3cm. Las pápulas faciales estaban presentes en el 50% de los hombres, el 83% presentaba alopecia de cejas, extremidades y alopecia androgenética (AGA). El eritema y la hiperqueratosis folicular se veían en el 66% de los casos y solo el 25% refería prurito. El tratamiento más frecuentemente utilizado consistió en corticoide tópico en 8 pacientes (66%), asociado a minoxidil tópico en 4 de ellos (33%). CONCLUSIONES: Según los datos obtenidos en nuestra serie, las pápulas faciales, la AGA y la afectación del vello corporal son más frecuentes en los hombres con AFF que en las mujeres. Por otra parte, a diferencia de los casos de AFF en varones descritos en la literatura, la edad media es mayor en nuestra serie, lo que podría explicar la mayor incidencia de AGA asociada y que la mayoría consulte por otro motivo

BACKGROUND: Frontal fibrosing alopecia (FFA) is a scarring disease in which the hairline recedes and the eyebrows can be affected. Usually seen in postmenopausal women, FFA is much less common in men. OBJECTIVE: To describe the clinical characteristics of FFA in a case series of men and compare this series to those reported in the literature. MATERIAL AND METHODS: Men with FFA being treated in our dermatology department from January 2010 to December 2015 were included prospectively for this descriptive study. We collected patient information and clincal and treatment characteristics. RESULTS: Twelve men (mean age, 75 years) were recruited. Alopecia was the reason for seeking medical care in only 4 cases. The hairline had receded 3cm on average. Half the patients had facial papules, and 83% had androgenetic alopecia or hair loss on eyebrows or extremities. Follicular hyperkeratosis and erythema were present in 66%, and only 25% of the men reported pruritus. The most commonly prescribed treatments were topical: corticosteroids in 8 patients (66%) and minoxidil in 4 (33%). CONCLUSIONS: Facial papules, androgenetic alopecia, and loss of body hair are more often observed in men with FFA than in women. The men in this series were older on average than in other FFA case series in the literature, possibly accounting for the higher prevalence of associated androgenetic alopecia and the fact that most of these men were seeking care for conditions other than hair loss

Frontal Fibrosing Alopecia in Men: Presentations in 12 Cases and a Review of the Literature. / Alopecia frontal fibrosante en el varón: presentación de 12 casos y revisión de la literatura.

BACKGROUND: Frontal fibrosing alopecia (FFA) is a scarring disease in which the hairline recedes and the eyebrows can be affected. Usually seen in postmenopausal women, FFA is much less common in men. OBJECTIVE: To describe the clinical characteristics of FFA in a case series of men and compare this series to those reported in the literature. MATERIAL AND METHODS: Men with FFA being treated in our dermatology department from January 2010 to December 2015 were included prospectively for this descriptive study. We collected patient information and clinical and treatment characteristics. RESULTS: Twelve men (mean age, 75 years) were recruited. Alopecia was the reason for seeking medical care in only 4 cases. The hairline had receded 3cm on average. Half the patients had facial papules, and 83% had androgenetic alopecia or hair loss on eyebrows or extremities. Follicular hyperkeratosis and erythema were present in 66%, and only 25% of the men reported pruritus. The most commonly prescribed treatments were topical: corticosteroids in 8 patients (66%) and minoxidil in 4 (33%). CONCLUSIONS: Facial papules, androgenetic alopecia, and loss of body hair are more often observed in men with FFA than in women. The men in this series were older on average than in other FFA case series in the literature, possibly accounting for the higher prevalence of associated androgenetic alopecia and the fact that most of these men were seeking care for conditions other than hair loss.

Eritema necrolítico migratorio como marcador de síndrome del glucagonoma / Necrolytic migratory erythema as clue to the diagnosis of glucagonoma syndrome

El síndrome del glucagonoma es un cuadro paraneoplásico poco frecuente. Se caracteriza por la presencia de eritema necrolítico migratorio, diabetes mellitus, pérdida de peso, anemia, estomatitis, diarrea, alteraciones neuropsiquiátricas y fenómenos tromboembólicos, asociados a una tumoración pancreática de células alfa. El diagnóstico precoz es clave para poder realizar un tratamiento curativo mediante la extirpación del tumor. Presentamos el caso de una mujer de 70 años con síndrome del glucagonoma diagnosticado a partir de lesiones cutáneas en forma de eritema necrolítico migratorio (AU)

Glucagonoma syndrome is a rare paraneoplasic phenomenon. It is characterized by the existente of necrolytic migratory erythema, diabetes mellitus, weight loss, anemia, stomatitis, diarrhea, neuropsychiatric manifestations and thromboembolic events, associated to an alpha-cell tumor of the pancreas. Early detection provides the cure of the neoplasm by surgical removal. We present a 70-year-old woman with necrolytic migratory erythema as the presenting manifestation of glucagonoma syndrome (AU)

Fenómeno isotópico de Wolf: serie de 9 casos / Wolf’s Isotopic Response: A Series of 9 Cases

Introducción: El fenómeno isotópico de Wolf se define como la aparición de una enfermedad cutánea nueva en la misma localización donde previamente ha acontecido otra, ya curada, y con la que no guarda ninguna relación. En la mayoría de los casos, la primera dermatosis es un herpes zóster (HZ). Posteriormente, en esta localización pueden desarrollarse diversos procesos dermatológicos, fundamentalmente reacciones granulomatosas y liquenoides, infiltraciones específicas de enfermedades hematológicas, tumores cutáneos o infecciones. La patogenia de estas reacciones cutáneas es desconocida. Se ha sugerido que la infección viral pudiera alterar la inmunidad cutánea local, favoreciendo una hiperreactividad que determinaría el desarrollo de procesos inflamatorios, o una inmunosupresión local, que condicionaría la aparición de infiltraciones tumorales o infecciones. Material y métodos: Estudio retrospectivo de 9 pacientes diagnosticados de fenómeno isotópico de Wolf en el Servicio de Dermatología del Hospital Donostia. Cinco pacientes tenían una leucemia linfática crónica-B (LLC-B), 2 un linfoma no Hodgkin y una un carcinoma de ovario. Resultados: La dermatosis primaria en 7 casos fue un HZ, en los otros 2 una varicela y un herpes simple. Respecto a las dermatosis secundarias se diagnosticaron 4 casos de dermatitis granulomatosa, 2 de dermatitis liquenoide, 2 de infiltración específica por LLC-B y uno de infiltración por un linfoma no Hodgkin sistémico. En este último caso las lesiones cutáneas fueron el primer signo del linfoma. Conclusiones: Destacamos la necesidad de biopsiar este tipo de lesiones para descartar infiltraciones específicas tumorales, ya que en nuestra casuística fueron más frecuentes de lo esperado (AU)

Introduction: The term Wolf s isotopic response refers to the appearance of a new skin disease at the site of an already healed, unrelated disease. In most cases, the initial disease is herpes zoster. Different diseases may subsequently develop on the same site. The most common isotopic responses are granulomatous and lichenoid reactions, infiltrations of hematologic diseases, skin tumors, and infections. The pathogenesis of these skin reactions is unknown. It has been suggested that viral infection may alter local skin immunity; this would favor hyperreactivity, leading to inflammatory processes, or local immunosuppression, leading to tumor infiltrations or infections. Materials and methods: We performed a retrospective study of 9 patients diagnosed with Wolf’s isotopic response in the dermatology department of Hospital Donostia in San Sebastian, Spain. Five patients had B-cell chronic lymphocytic leukemia, 2 had a non-Hodgkin lymphoma, and 1had ovarian carcinoma. Results: The initial disease was herpes zoster in 7 cases, and chickenpox and herpes simplex in the other 2 cases. The second disease was granulomatous dermatitis in 4 cases, lichenoid dermatitis in 2 cases, infiltration by B-cell chronic lymphatic leukemia in 2 cases, and infiltration by systemic non-Hodgkin lymphoma in 1 case. In the last case, the skin lesions were the first sign of the lymphoma. Conclusions: We highlight the need to biopsy these second lesions in order to rule out tumor infiltrations, which were more frequent than expected in our series (AU)

Wolf's isotopic response: a series of 9 cases.

INTRODUCTION: The term Wolf's isotopic response refers to the appearance of a new skin disease at the site of an already healed, unrelated disease. In most cases, the initial disease is herpes zoster. Different diseases may subsequently develop on the same site. The most common isotopic responses are granulomatous and lichenoid reactions, infiltrations of hematologic diseases, skin tumors, and infections. The pathogenesis of these skin reactions is unknown. It has been suggested that viral infection may alter local skin immunity; this would favor hyperreactivity, leading to inflammatory processes, or local immunosuppression, leading to tumor infiltrations or infections. MATERIALS AND METHODS: We performed a retrospective study of 9 patients diagnosed with Wolf's isotopic response in the dermatology department of Hospital Donostia in San Sebastian, Spain. Five patients had B-cell chronic lymphocytic leukemia, 2 had a non-Hodgkin lymphoma, and 1 had ovarian carcinoma. RESULTS: The initial disease was herpes zoster in 7 cases, and chickenpox and herpes simplex in the other 2 cases. The second disease was granulomatous dermatitis in 4 cases, lichenoid dermatitis in 2 cases, infiltration by B-cell chronic lymphatic leukemia in 2 cases, and infiltration by systemic non-Hodgkin lymphoma in 1 case. In the last case, the skin lesions were the first sign of the lymphoma. CONCLUSIONS: We highlight the need to biopsy these second lesions in order to rule out tumor infiltrations, which were more frequent than expected in our series.