Lesiones cutáneas específicas en pacientes con neurosarcoidosis / Specific Cutaneous Lesions in Patients With Neurosarcoidosis

La sarcoidosis del sistema nervioso (neurosarcoidosis [NS]) es poco frecuente, pero puede ser grave y producir secuelas importantes. Analizamos los tipos de lesión cutánea específica en pacientes con NS y su utilidad para el diagnóstico de la enfermedad. De un total de 58, 16 presentaron lesiones cutáneas específicas (27,6%). De ellos, 14 eran mujeres y dos varones (edad mediana 50 años [rango 20-84]), que presentaron 24 tipos de lesiones neurológicas (siete neuropatía craneal, cuatro parenquimatosa, tres meníngea, tres mielopatía, uno hipofisaria, dos hidrocefalia y cuatro neuropatía periférica) y 20 lesiones cutáneas específicas (seis máculo-pápulas, nueve placas, uno lupus pernio y cuatro sarcoidosis de las cicatrices [dos pacientes con máculo-pápulas y dos con placas presentaron también sarcoidosis de las cicatrices]. Las lesiones cutáneas estaban presentes al diagnóstico de la NS en 13 casos. Ante la sospecha de esta enfermedad hay que descartar la presencia de lesiones cutáneas específicas cuya biopsia puede acelerar el diagnóstico. (AU)

Neurosarcoidosis is an uncommon but potentially serious disease of the central nervous system that can cause major sequelae. We analyzed the presence and diagnostic usefulness of specific cutaneous lesions in 58 patients with neurosarcoidosis. Sixteen patients (27.6%) had specific cutaneous lesions (14 men and 2 women; mean age, 50 years [range, 20-84 years]). Twenty-four types of neurological lesions were observed: cranial neuropathy (n=7), parenchymal lesions (n=4), meningeal lesions (n=3), myelopathy (n=3), pituitary lesions (n=1), hydrocephalus (n=2), and peripheral neuropathy (n=4). Twenty types of specific cutaneous lesions were observed: maculopapular lesions (n=6), plaques (n=9), lupus pernio (n=1), and scar sarcoidosis (n=4). These last lesions coexisted with maculopapular lesions in 2 patients and plaques in another 2. Specific cutaneous lesions were present at diagnosis of neurosarcoidosis in 13 patients. Recognition of specific cutaneous lesions in a patient with suspected neurosarcoidosis is important as biopsy can accelerate diagnosis. (AU)

[Translated article] Specific Cutaneous Lesions in Patients With Neurosarcoidosis / Lesiones cutáneas específicas en pacientes con neurosarcoidosis

Neurosarcoidosis is an uncommon but potentially serious disease of the central nervous system that can cause major sequelae. We analyzed the presence and diagnostic usefulness of specific cutaneous lesions in 58 patients with neurosarcoidosis. Sixteen patients (27.6%) had specific cutaneous lesions (14 men and 2 women; mean age, 50 years [range, 20-84 years]). Twenty-four types of neurological lesions were observed: cranial neuropathy (n=7), parenchymal lesions (n=4), meningeal lesions (n=3), myelopathy (n=3), pituitary lesions (n=1), hydrocephalus (n=2), and peripheral neuropathy (n=4). Twenty types of specific cutaneous lesions were observed: maculopapular lesions (n=6), plaques (n=9), lupus pernio (n=1), and scar sarcoidosis (n=4). These last lesions coexisted with maculopapular lesions in 2 patients and plaques in another 2. Specific cutaneous lesions were present at diagnosis of neurosarcoidosis in 13 patients. Recognition of specific cutaneous lesions in a patient with suspected neurosarcoidosis is important as biopsy can accelerate diagnosis. (AU)

La sarcoidosis del sistema nervioso (neurosarcoidosis [NS]) es poco frecuente, pero puede ser grave y producir secuelas importantes. Analizamos los tipos de lesión cutánea específica en pacientes con NS y su utilidad para el diagnóstico de la enfermedad. De un total de 58, 16 presentaron lesiones cutáneas específicas (27,6%). De ellos, 14 eran mujeres y dos varones (edad mediana 50 años [rango 20-84]), que presentaron 24 tipos de lesiones neurológicas (siete neuropatía craneal, cuatro parenquimatosa, tres meníngea, tres mielopatía, uno hipofisaria, dos hidrocefalia y cuatro neuropatía periférica) y 20 lesiones cutáneas específicas (seis máculo-pápulas, nueve placas, uno lupus pernio y cuatro sarcoidosis de las cicatrices [dos pacientes con máculo-pápulas y dos con placas presentaron también sarcoidosis de las cicatrices]. Las lesiones cutáneas estaban presentes al diagnóstico de la NS en 13 casos. Ante la sospecha de esta enfermedad hay que descartar la presencia de lesiones cutáneas específicas cuya biopsia puede acelerar el diagnóstico. (AU)

[Translated article] Specific Cutaneous Lesions in Patients With Neurosarcoidosis.

Neurosarcoidosis is an uncommon but potentially serious disease of the central nervous system that can cause major sequelae. We analyzed the presence and diagnostic usefulness of specific cutaneous lesions in 58 patients with neurosarcoidosis. Sixteen patients (27.6%) had specific cutaneous lesions (14 men and 2 women; mean age, 50 years [range, 20-84 years]). Twenty-four types of neurological lesions were observed: cranial neuropathy (n=7), parenchymal lesions (n=4), meningeal lesions (n=3), myelopathy (n=3), pituitary lesions (n=1), hydrocephalus (n=2), and peripheral neuropathy (n=4). Twenty types of specific cutaneous lesions were observed: maculopapular lesions (n=6), plaques (n=9), lupus pernio (n=1), and scar sarcoidosis (n=4). These last lesions coexisted with maculopapular lesions in 2 patients and plaques in another 2. Specific cutaneous lesions were present at diagnosis of neurosarcoidosis in 13 patients. Recognition of specific cutaneous lesions in a patient with suspected neurosarcoidosis is important as biopsy can accelerate diagnosis.

Specific Cutaneous Lesions in Patients With Neurosarcoidosis. / Lesiones cutáneas específicas en pacientes con neurosarcoidosis.

Neurosarcoidosis is an uncommon but potentially serious disease of the central nervous system that can cause major sequelae. We analyzed the presence and diagnostic usefulness of specific cutaneous lesions in 58 patients with neurosarcoidosis. Sixteen patients (27.6%) had specific cutaneous lesions (14 men and 2 women; mean age, 50 years [range, 20-84 years]). Twenty-four types of neurological lesions were observed: cranial neuropathy (n=7), parenchymal lesions (n=4), meningeal lesions (n=3), myelopathy (n=3), pituitary lesions (n=1), hydrocephalus (n=2), and peripheral neuropathy (n=4). Twenty types of specific cutaneous lesions were observed: maculopapular lesions (n=6), plaques (n=9), lupus pernio (n=1), and scar sarcoidosis (n=4). These last lesions coexisted with maculopapular lesions in 2 patients and plaques in another 2. Specific cutaneous lesions were present at diagnosis of neurosarcoidosis in 13 patients. Recognition of specific cutaneous lesions in a patient with suspected neurosarcoidosis is important as biopsy can accelerate diagnosis.

Acantoma de células claras. Estudio clínico de 70 pacientes / Clear Cell Acanthoma: A Series of 70 Cases

Antecedentes y objetivo El acantoma de células claras (ACC) es una lesión poco frecuente caracterizada histológicamente por la presencia de acantosis epidérmica a expensas de queratinocitos de citoplasma claro. Aunque se han descrito muchos casos clínicos de forma individual, se han publicado pocas series de pacientes con ACC. Nuestro objetivo fue analizar las características clínicas del ACC en nuestra población. Material y métodos Estudio retrospectivo de los pacientes con ACC diagnosticados en el Hospital Universitario de Bellvitge en Barcelona, España, entre 1995-2021. Se revisaron las historias clínicas para obtener los siguientes datos: edad, sexo, localización, número de lesiones, diámetro, tiempo de evolución, características clínicas de los tumores, diagnóstico clínico de sospecha y el tratamiento realizado Resultados Setenta pacientes con ACC fueron incluidos en el estudio (30 mujeres y 40 varones, edad media 62 años, DE 13). La mediana del tiempo de evolución fue de dos años, rango intercuartílico (RIQ) 4 y la del diámetro 6 mm, RIQ 5. Una paciente presentó lesiones múltiples. Las lesiones se localizaron en las extremidades inferiores en 57 pacientes (81%), en la cara posterior del tronco en ocho (11%), la cara anterior del tronco en cuatro (5%) y en las extremidades superiores en uno (1%). Se planteó el diagnóstico clínico de ACC en 40% de los pacientes atendidos por dermatólogos. Conclusiones El aspecto clínico característico del ACC es el de una lesión cupuliforme eritematosa con punteado vascular y collarete epidérmico. La proporción de ACC diagnosticados clínicamente ha mejorado respecto a series antiguas, quizás por un mayor conocimiento clínico de la entidad y un mayor uso de la dermatoscopia (AU)

Background and objective Clear cell acanthoma (CCA) is an uncommon lesion histologically characterized by the presence of epidermal acanthosis with keratinocytes containing clear cytoplasm. Although many single cases of CCA have been described, few case series have been published. The aim of this study was to describe the clinical characteristics of CCA in our practice setting. Material and methods Retrospective study of patients diagnosed with CCA at Hospital Universitario de Bellvitge in Barcelona, Spain, between 1995 and 2021. We conducted a chart review to record age, sex, number and location of lesions, diameter, time since onset, clinical characteristics, suspected clinical diagnosis, and treatment. Results Seventy patients (30 women and 40 men) with a mean (SD) age of 62 (13) years were diagnosed with CCA during the study period. Median (interquartile range) time since onset was 2 (4) years and median lesion diameter was 6 (5) mm. One woman had multiple lesions. Lesions were located on the lower extremities in 57 patients (81%), the posterior aspect of the trunk in 8 (11%), the anterior aspect of the trunk in 4 (5%), and the upper extremities in 1 (1%). CCA was clinically suspected in 40% of patients seen by dermatologists. Conclusions CCA presents as an erythematous, dome-shaped lesion with pinpoint vessels and an epidermal collarette. The accuracy of clinical diagnosis has improved relative to earlier series, possibly due to a better clinical understanding of this lesion and a greater use of dermoscopy (AU)

[Translated article] Clear Cell Acanthoma: A Series of 70 Cases / Acantoma de células claras. Estudio clínico de 70 pacientes

Background and objective Clear cell acanthoma (CCA) is an uncommon lesion histologically characterized by the presence of epidermal acanthosis with keratinocytes containing clear cytoplasm. Although many single cases of CCA have been described, few case series have been published. The aim of this study was to describe the clinical characteristics of CCA in our practice setting. Material and methods Retrospective study of patients diagnosed with CCA at Hospital Universitario de Bellvitge in Barcelona, Spain, between 1995 and 2021. We conducted a chart review to record age, sex, number and location of lesions, diameter, time since onset, clinical characteristics, suspected clinical diagnosis, and treatment. Results Seventy patients (30 women and 40 men) with a mean (SD) age of 62 (13) years were diagnosed with CCA during the study period. Median (interquartile range) time since onset was 2 (4) years and median lesion diameter was 6 (5)mm. One woman had multiple lesions. Lesions were located on the lower extremities in 57 patients (81%), the posterior aspect of the trunk in 8 (11%), the anterior aspect of the trunk in 4 (5%), and the upper extremities in 1 (1%). CCA was clinically suspected in 40% of patients seen by dermatologists. Conclusions CCA presents as an erythematous, dome-shaped lesion with pinpoint vessels and an epidermal collarette. The accuracy of clinical diagnosis has improved relative to earlier series, possibly due to a better clinical understanding of this lesion and a greater use of dermoscopy (AU)

Antecedentes y objetivo El acantoma de células claras (ACC) es una lesión poco frecuente caracterizada histológicamente por la presencia de acantosis epidérmica a expensas de queratinocitos de citoplasma claro. Aunque se han descrito muchos casos clínicos de forma individual, se han publicado pocas series de pacientes con ACC. Nuestro objetivo fue analizar las características clínicas del ACC en nuestra población. Material y métodos Estudio retrospectivo de los pacientes con ACC diagnosticados en el Hospital Universitario de Bellvitge en Barcelona, España, entre 1995-2021. Se revisaron las historias clínicas para obtener los siguientes datos: edad, sexo, localización, número de lesiones, diámetro, tiempo de evolución, características clínicas de los tumores, diagnóstico clínico de sospecha y tratamiento realizado. Resultados Setenta pacientes con ACC fueron incluidos en el estudio (30 mujeres y 40 varones, edad media: 62 años, DE: 13). La mediana del tiempo de evolución fue de 2 años, rango intercuartílico (RIQ): 4 y la del diámetro 6mm, RIQ: 5. Una paciente presentó lesiones múltiples. Las lesiones se localizaron en las extremidades inferiores en 57 pacientes (81%), en la cara posterior del tronco en 8 pacientes (11%), la cara anterior del tronco en 4 pacientes (5%) y en las extremidades superiores en un paciente (1%). Se planteó el diagnóstico clínico de ACC en el 40% de los pacientes atendidos por dermatólogos. Conclusiones El aspecto clínico característico del ACC es el de una lesión cupuliforme eritematosa con punteado vascular y collarete epidérmico. La proporción de ACC diagnosticados clínicamente ha mejorado respecto a series antiguas, quizás por un mayor conocimiento clínico de la entidad y un mayor uso de la dermatoscopia (AU)

Clear Cell Acanthoma: A Series of 70 Cases. / Acantoma de células claras. Estudio clínico de 70 pacientes.

BACKGROUND AND OBJECTIVE: Clear cell acanthoma (CCA) is an uncommon lesion histologically characterized by the presence of epidermal acanthosis with keratinocytes containing clear cytoplasm. Although many single cases of CCA have been described, few case series have been published. The aim of this study was to describe the clinical characteristics of CCA in our practice setting. MATERIAL AND METHODS: Retrospective study of patients diagnosed with CCA at Hospital Universitario de Bellvitge in Barcelona, Spain, between 1995 and 2021. We conducted a chart review to record age, sex, number and location of lesions, diameter, time since onset, clinical characteristics, suspected clinical diagnosis, and treatment. RESULTS: Seventy patients (30 women and 40 men) with a mean (SD) age of 62 (13) years were diagnosed with CCA during the study period. Median (interquartile range) time since onset was 2 (4) years and median lesion diameter was 6 (5) mm. One woman had multiple lesions. Lesions were located on the lower extremities in 57 patients (81%), the posterior aspect of the trunk in 8 (11%), the anterior aspect of the trunk in 4 (5%), and the upper extremities in 1 (1%). CCA was clinically suspected in 40% of patients seen by dermatologists. CONCLUSIONS: CCA presents as an erythematous, dome-shaped lesion with pinpoint vessels and an epidermal collarette. The accuracy of clinical diagnosis has improved relative to earlier series, possibly due to a better clinical understanding of this lesion and a greater use of dermoscopy.

Poroma. Estudio retrospectivo de 80 pacientes en un hospital terciario / Poroma: A Retrospective Series of 80 Patients at a Tertiary Care Hospital

Introducción El poroma es un tumor anexial benigno exclusivo de la piel que tiene una predilección por la piel palmoplantar.Objetivo Analizar las características clínicas del poroma en nuestra población.Material y métodosEstudio retrospectivo de los pacientes con poroma diagnosticados entre 2002 y 2021. Se revisaron las historias clínicas para obtener los siguientes datos: edad, sexo, localización, número de lesiones, diámetro, tiempo de evolución, características clínicas de los tumores, diagnóstico clínico de sospecha, estado de los márgenes de resección, desarrollo de recidiva y tiempo de seguimiento. Las variables categóricas se compararon mediante la prueba exacta de Fisher. Las variables continuas se compararon mediante la prueba t de Student cuando se confirmó la normalidad de la distribución de datos. De lo contrario, se realizó la prueba U de Mann-Whitney. ResultadosSe incluyeron 80 pacientes con poroma (31 mujeres y 49 varones, con una edad mediana de 65,5 años y rango intercuartílico [RIC] 29 años). La mediana del tiempo medio de evolución fue de 12 meses, RIC 21 meses y la mediana del diámetro 8mm, RIC 7. Ningún paciente presentó lesiones múltiples. Las lesiones se localizaron en la cabeza y el cuello en 13 casos, en el tronco en 13, en las extremidades superiores en 11 y en las extremidades inferiores en 43. Veintitrés lesiones (28,8%) se localizaron en piel acral (palmas 5, plantas 18). La localización en el cuero cabelludo fue más frecuente en el sexo femenino (p=0,041). Las lesiones acrales fueron con mayor frecuencia eritematosas (p=0,014). Cinco pacientes presentaron recidiva local. Conclusiones Aunque los poromas son especialmente frecuentes en la piel acral, sobre todo de los pies, en la mayoría de pacientes se localizan en otras regiones anatómicas (71,3%). El aspecto clínico clásico de lesión exofítica eritematosa se observa con una mayor frecuencia en las lesiones acrales. (AU)

Background Poroma is a benign, exclusively cutaneous, adnexal tumor with a predilection for palmoplantar skin. Objective To analyze the clinical characteristics of poroma in our population.Material and methodsRetrospective study of patients diagnosed with poroma between 2002 and 2021. We conducted a chart review to record age; sex; number, location, and diameter of lesions; time since onset; clinical characteristics; suspected clinical diagnosis; resection margin status; recurrences; and follow-up duration. Categorical variables were compared using the Fisher exact test. Continuous variables were compared using the t test or the Mann-Whitney U test depending on whether they were normally or nonnormally distributed. Results We studied 80 patients (31 women and 49 men) with a median (interquartile range [IQR]) age of 65.5 (29) years. Median time since onset of poroma was 12 (21) months. Median lesion diameter was 8(7)mm, and none of the patients had multiple lesions. The lesions were located on the head and neck in 13 cases, the trunk in 13, the upper extremities in 11, and the lower extremities in 43. Twenty-three lesions (28.8%) were located at acral sites (5 on the palms and 18 on the soles). Women were more likely to have scalp lesions (P=.041). Acral lesions were more likely to be erythematous (P=.014). Five patients experienced local recurrence. Conclusions Although poromas are particularly common in acral locations (especially the feet), most of the lesions in our series (71.3%) were located elsewhere. Acral lesions were more likely to show the classic clinical features of erythema and exophytic growth. (AU)

[Translated article] Poroma: A Retrospective Series of 80 Patients at a Tertiary Care Hospital / Poroma. Estudio retrospectivo de 80 pacientes en un hospital terciario

Background Poroma is a benign, exclusively cutaneous, adnexal tumor with a predilection for palmoplantar skin. Objective To analyze the clinical characteristics of poroma in our population.Material and methods Retrospective study of patients diagnosed with poroma between 2002 and 2021. We conducted a chart review to record age; sex; number, location, and diameter of lesions; time since onset; clinical characteristics; suspected clinical diagnosis; resection margin status; recurrences; and follow-up duration. Categorical variables were compared using the Fisher exact test. Continuous variables were compared using the t test or the Mann–Whitney U test depending on whether they were normally or nonnormally distributed. Results We studied 80 patients (31 women and 49 men) with a median (interquartile range [IQR]) age of 65.5 (29) years. Median time since onset of poroma was 12 (21) months. Median lesion diameter was 8 (7)mm, and none of the patients had multiple lesions. The lesions were located on the head and neck in 13 cases, the trunk in 13, the upper extremities in 11, and the lower extremities in 43. Twenty-three lesions (28.8%) were located at acral sites (5 on the palms and 18 on the soles). Women were more likely to have scalp lesions (P=.041). Acral lesions were more likely to be erythematous (P=.014). Five patients experienced local recurrence. Conclusions Although poromas are particularly common in acral locations (especially the feet), most of the lesions in our series (71.3%) were located elsewhere. Acral lesions were more likely to show the classic clinical features of erythema and exophytic growth. (AU)

Introducción El poroma es un tumor anexial benigno exclusivo de la piel que tiene predilección por la piel palmoplantar. Objetivo Analizar las características clínicas del poroma en nuestra población. Material y métodos Estudio retrospectivo de los pacientes con poroma diagnosticados entre 2002 y 2021. Se revisaron las historias clínicas para obtener los siguientes datos: edad, sexo, localización, número de lesiones, diámetro, tiempo de evolución, características clínicas de los tumores, diagnóstico clínico de sospecha, estado de los márgenes de resección, desarrollo de recidiva y tiempo de seguimiento. Las variables categóricas se compararon mediante la prueba exacta de Fisher. Las variables continuas se compararon mediante la prueba t de Student cuando se confirmó la normalidad de la distribución de datos. De lo contrario, se realizó la prueba U de Mann-Whitney. Resultados Se incluyeron 80 pacientes con poroma (31 mujeres y 49 varones, con una edad mediana de 65,5años y rango intercuartílico [RIC] de 29 años). La mediana del tiempo medio de evolución fue de 12meses, RIC 21 meses, y la mediana del diámetro 8mm, RIC 7. Ningún paciente presentó lesiones múltiples. Las lesiones se localizaron en la cabeza y en el cuello en 13 casos, en el tronco en 13, en las extremidades superiores en 11 y en las extremidades inferiores en 43. Veintitrés lesiones (28,8%) se localizaron en piel acral (palmas 5, plantas 18). La localización en el cuero cabelludo fue más frecuente en el sexo femenino (p=0,041). Las lesiones acrales fueron con mayor frecuencia eritematosas (p=0,014). Cinco pacientes presentaron recidiva local. Conclusiones Aunque los poromas son especialmente frecuentes en la piel acral, sobre todo de los pies, en la mayoría de pacientes se localizan en otras regiones anatómicas (71,3%). El aspecto clínico clásico de lesión exofítica eritematosa se observa con una mayor frecuencia en las lesiones acrales. (AU)

Dactilitis producida por sarcoidosis subcutánea como presentación de sarcoidosis / Dactylitis Due to Subcutaneous Sarcoidosis as a Manifestation of Sarcoidosis

La dactilitis o dedo en salchicha, se define como la inflamación de uno o varios dedos de las manos o de los pies. La dactilitis sarcoidea es una forma poco frecuente de dactilitis, que suele ser debida a la afectación ósea granulomatosa a nivel de las falanges subyacentes. Sin embargo, una apariencia clínica similar puede ser debida a la presencia de una sarcoidosis subcutánea en los dedos. Describimos los casos de 5 pacientes con una dactilitis por una sarcoidosis subcutánea de los dedos. En nuestros pacientes, las lesiones se localizaron en las falanges proximales a diferencia de la dactilitis sarcoidea por afectación ósea, que suele localizarse en las falanges medias y distales. Es importante el diagnóstico diferencial puesto que la dactilitis por afectación ósea suele ser mucho más persistente y asociarse a formas crónicas de sarcoidosis (AU)

Dactylitis, or sausage fingers or toes, refers to the swelling of 1 or more digits. Sarcoid dactylitis is an uncommon form that tends to be caused by granulomatous bone involvement in the underlying phalanges. Sarcoid dactylitis can, however, clinically resemble subcutaneous sarcoidosis with finger or toe involvement. We describe 5 patients with dactylitis due to subcutaneous sarcoidosis of the fingers. In all cases, the proximal phalanges were involved; sarcoid dactylitis, by contrast, tends to affect the medial and distal phalanges. Differential diagnosis is important, as dactylitis due to bone involvement tends to be much more persistent and is generally seen in chronic forms of sarcoidosis (AU)

[Translated article] Dactylitis Due to Subcutaneous Sarcoidosis as a Manifestation of Sarcoidosis / Dactilitis producida por sarcoidosis subcutánea como presentación de sarcoidosis

Dactylitis, or sausage fingers or toes, refers to the swelling of 1 or more digits. Sarcoid dactylitis is an uncommon form that tends to be caused by granulomatous bone involvement in the underlying phalanges. Sarcoid dactylitis can, however, clinically resemble subcutaneous sarcoidosis with finger or toe involvement. We describe 5 patients with dactylitis due to subcutaneous sarcoidosis of the fingers. In all cases, the proximal phalanges were involved; sarcoid dactylitis, by contrast, tends to affect the medial and distal phalanges. Differential diagnosis is important, as dactylitis due to bone involvement tends to be much more persistent and is generally seen in chronic forms of sarcoidosis (AU)

La dactilitis o dedo en salchicha, se define como la inflamación de uno o varios dedos de las manos o de los pies. La dactilitis sarcoidea es una forma poco frecuente de dactilitis, que suele ser debida a la afectación ósea granulomatosa a nivel de las falanges subyacentes. Sin embargo, una apariencia clínica similar puede ser debida a la presencia de una sarcoidosis subcutánea en los dedos. Describimos los casos de 5 pacientes con una dactilitis por una sarcoidosis subcutánea de los dedos. En nuestros pacientes, las lesiones se localizaron en las falanges proximales a diferencia de la dactilitis sarcoidea por afectación ósea, que suele localizarse en las falanges medias y distales. Es importante el diagnóstico diferencial puesto que la dactilitis por afectación ósea suele ser mucho más persistente y asociarse a formas crónicas de sarcoidosis (AU)

Dactylitis Due to Subcutaneous Sarcoidosis as a Manifestation of Sarcoidosis. / Dactilitis producida por sarcoidosis subcutánea como presentación de sarcoidosis.

Dactylitis, or sausage fingers or toes, refers to the swelling of 1 or more digits. Sarcoid dactylitis is an uncommon form that tends to be caused by granulomatous bone involvement in the underlying phalanges. Sarcoid dactylitis can, however, clinically resemble subcutaneous sarcoidosis with finger or toe involvement. We describe 5 patients with dactylitis due to subcutaneous sarcoidosis of the fingers. In all cases, the proximal phalanges were involved; sarcoid dactylitis, by contrast, tends to affect the medial and distal phalanges. Differential diagnosis is important, as dactylitis due to bone involvement tends to be much more persistent and is generally seen in chronic forms of sarcoidosis.

Poroma: A Retrospective Series of 80 Patients at a Tertiary Care Hospital. / Poroma. Estudio retrospectivo de 80 pacientes en un hospital terciario.

BACKGROUND: Poroma is a benign, exclusively cutaneous, adnexal tumor with a predilection for palmoplantar skin. OBJECTIVE: To analyze the clinical characteristics of poroma in our population. MATERIAL AND METHODS: Retrospective study of patients diagnosed with poroma between 2002 and 2021. We conducted a chart review to record age; sex; number, location, and diameter of lesions; time since onset; clinical characteristics; suspected clinical diagnosis; resection margin status; recurrences; and follow-up duration. Categorical variables were compared using the Fisher exact test. Continuous variables were compared using the t test or the Mann-Whitney U test depending on whether they were normally or nonnormally distributed. RESULTS: We studied 80 patients (31 women and 49 men) with a median (interquartile range [IQR]) age of 65.5 (29) years. Median time since onset of poroma was 12 (21) months. Median lesion diameter was 8(7)mm, and none of the patients had multiple lesions. The lesions were located on the head and neck in 13 cases, the trunk in 13, the upper extremities in 11, and the lower extremities in 43. Twenty-three lesions (28.8%) were located at acral sites (5 on the palms and 18 on the soles). Women were more likely to have scalp lesions (P=.041). Acral lesions were more likely to be erythematous (P=.014). Five patients experienced local recurrence. CONCLUSIONS: Although poromas are particularly common in acral locations (especially the feet), most of the lesions in our series (71.3%) were located elsewhere. Acral lesions were more likely to show the classic clinical features of erythema and exophytic growth.

Clinicopathological features, MCPyV status and outcomes of Merkel cell carcinoma in solid-organ transplant recipients: a retrospective, multicentre cohort study.

BACKGROUND: The proportion of Merkel cell carcinomas (MCCs) in solid-organ transplant recipients (SOTR) harbouring Merkel cell polyomavirus (MCPyV) is unknown, as are factors affecting their outcomes. OBJECTIVE: To describe clinicopathological features of MCC in SOTR, investigate the tumoral MCPyV-status and identify factors associated with tumour outcomes. METHODS: Retrospective, international, cohort-study. MCPyV-status was investigated by immunohistochemistry and polymerase chain reaction. RESULTS: A total of 30 SOTR and 44 consecutive immunocompetent patients with MCC were enrolled. SOTR were younger at diagnosis (69 vs. 78 years, P < 0.001). Thirty-three percent of SOTR MCCs were MCPyV-positive vs. 91% of immunocompetent MCCs (P = 0.001). Solid-organ transplantation was associated with an increased cumulative incidence of progression (SHR: 3.35 [1.57-7.14], P = 0.002), MCC-specific mortality (SHR: 2.55 [1.07-6.06], P = 0.034) and overall mortality (HR: 3.26 [1.54-6.9], P = 0.002). MCPyV-positivity and switching to an mTOR inhibitor (mTORi) after MCC diagnosis were associated with an increased incidence of progression (SHR: 4.3 [1.5-13], P = 0.008 and SHR: 3.6 [1.1-12], P = 0.032 respectively) in SOTR. LIMITATIONS: Retrospective design and heterogeneity of SOTR cohort. CONCLUSIONS: MCPyV appears to play a less prominent role in the aetiopathogenesis of MCC in SOTR. SOTR have a worse prognosis than their immunocompetent counterparts and switching to an mTORi after the diagnosis of MCC does not improve progression.

Tumor de células granulares. Estudio clínico de 81 pacientes / Granular Cell Tumor: A Clinical Study of 81 Patients

Introducción: El tumor de células granulares (TCG) suele desarrollarse en la piel o en la mucosa oral, pero se ha descrito en muchos otros órganos. Suele ser único, pero puede ser múltiple y asociarse a otras enfermedades. Objetivos: Analizar las características clínicas de nuestros pacientes con TCG en la piel y la mucosa oral y su posible asociación con otras afecciones. Material y métodos: Estudio retrospectivo de los pacientes con TCG diagnosticados entre 1995-2019. Se revisaron las historias clínicas para obtener los siguientes datos: edad, sexo, localización, número de lesiones, diámetro, tiempo de evolución, diagnóstico clínico de sospecha, estado de los márgenes de resección, desarrollo de recidiva, tiempo de seguimiento y enfermedades asociadas. Resultados: Ochenta y un pacientes presentaron 89 TCG en la piel y la mucosa oral (43 mujeres/38 varones, edad media 40,21años). El diámetro medio fue de 1,34cm. Contabilizando los tumores extracutáneos 5 de los 81 pacientes presentaban TCG múltiple (6,2%). La edad media de los pacientes con TCG múltiple fue significativamente inferior a la de los pacientes con un único tumor (p=0,004). Solamente un paciente presentó recidiva local y ninguno desarrolló metástasis a distancia. No hemos detectado ningún caso asociado a otras enfermedades. Conclusiones: La mayoría de TCG son benignos, y a pesar de tener los márgenes afectados no suelen presentar recidivas locales. Sin embargo, hay que tener en cuenta la posibilidad de presentar TCG múltiples, tanto en la piel y en la mucosa oral como en órganos internos, especialmente en pacientes jóvenes (AU)

Introduction: Although a granular cell tumor (GCT) usually develops on the skin or oral mucosa, it has been described in many other organs. GCT typically presents as a solitary tumor, but multiple lesions can occur. It has also been described in association with other diseases. Objective: To describe the clinical characteristics of cutaneous and oral mucosal GCTs and explore potential associations with other diseases. Material and methods: Retrospective study of patients diagnosed with GCT at our hospital between 1995 and 2019. The following information was collected from the patients’ medical records: age, sex, number of GCTs, location, diameter, time to diagnosis, tentative clinical diagnosis, surgical margin status, recurrence, follow-up time, and associated diseases. Results: We detected 89 cutaneous or oral mucosal GCTs in 81 patients (43 women, 38 men) with a mean age of 40.21 years. The mean tumor diameter was 1.34cm. Five of the 81 patients (6.2%) had multiple GCTs, including noncutaneous tumors. Patients with multiple GCTs were on average younger than those with a single tumor (P=.004). There was only a single case of local recurrence and no cases of distant metastasis. None of the patients had associated diseases. Conclusions: Most GCTs are benign and local recurrence is uncommon, even in patients with positive margins. Nevertheless, the possibility of multiple tumors affecting the skin, oral mucosa, or internal organs should be borne in mind, especially in young patients (AU)

Granular Cell Tumor: A Clinical Study of 81 Patients. / Tumor de células granulares. Estudio clínico de 81 pacientes.

INTRODUCTION: Although a granular cell tumor (GCT) usually develops on the skin or oral mucosa, it has been described in many other organs. GCT typically presents as a solitary tumor, but multiple lesions can occur. It has also been described in association with other diseases. OBJECTIVES: To describe the clinical characteristics of cutaneous and oral mucosal GCTs and explore potential associations with other diseases. MATERIAL AND METHODS: Retrospective study of patients diagnosed with GCT at our hospital between 1995 and 2019. The following information was collected from the patients' medical records: age, sex, number of GCTs, location, diameter, time to diagnosis, tentative clinical diagnosis, surgical margin status, recurrence, follow-up time, and associated diseases. RESULTS: We detected 89 cutaneous or oral mucosal GCTs in 81 patients (43 women, 38 men) with a mean age of 40.21 years. The mean tumor diameter was 1.34cm. Five of the 81 patients (6.2%) had multiple GCTs, including noncutaneous tumors. Patients with multiple GCTs were on average younger than those with a single tumor (P=.004). There was only a single case of local recurrence and no cases of distant metastasis. None of the patients had associated diseases. CONCLUSIONS: Most GCTs are benign and local recurrence is uncommon, even in patients with positive margins. Nevertheless, the possibility of multiple tumors affecting the skin, oral mucosa, or internal organs should be borne in mind, especially in young patients.