ABSTRACT
BACKGROUND: T1 melanoma substaging was recently modified by the American Joint Committee on Cancer (AJCC). Although sentinel lymph node (SLN) positivity is the most important prognostic factor in melanoma, there is a lack of consensus on whether SLN biopsy should be performed in patients with thin melanoma (≤1 mm). OBJECTIVE: The main aim of this study was to investigate predictors of SLN positivity in patients with thin melanoma, with a special emphasis on mitotic rate. A secondary aim was to evaluate survival in this group of patients. MATERIALS AND METHODS: Retrospective multicenter observational study with analysis of age, sex, tumour location, thickness, mitotic rate, regression and microscopic satellites. Predictive factors were identified using a classification and regression tree (CART) approach. Melanoma-specific survival according to SLN status was estimated using Kaplan-Meier curves. RESULTS: We analysed 203 patients with a melanoma ≤1 mm. Using the new AJCC staging criteria, the CART algorithm identified a 7.5% likelihood of SLN positivity in T1a patients. In the case of T1b melanoma, there was a 14.3% likelihood of SLN positivity in patients with a mitotic rate >1 mitosis/mm2 and a 3.2% likelihood in those with ≤1 mitoses/mm2 . None of the patients with T1b disease who had ≤1 mitoses/mm2 and regression had SLN positivity. In T1b patients, 5-year melanoma-specific survival was 98.7% in the SLN-negative group and 75% in the SLN-positive group (P = 0.05). When stratified by mitotic rate, survival was 100% for patients with a mitotic rate of ≤1 mitoses/mm2 and 91.4% for those with >1 mitosis/mm2 (P = 0.022). There were no deaths in the T1a subgroup. CONCLUSIONS: Sentinel lymph node metastasis was less common in patients with T1b melanoma who had a mitotic rate of ≤1 mitoses/mm2 . Performance of SLN biopsy should be carefully considered in this subgroup of patients, particularly considering the good prognosis.
Subject(s)
Melanoma/genetics , Mitotic Index , Skin Neoplasms/genetics , Adult , Female , Humans , Lymphatic Metastasis , Male , Melanoma/pathology , Middle Aged , Retrospective Studies , Sentinel Lymph Node , Sentinel Lymph Node Biopsy , Skin Neoplasms/pathologyABSTRACT
El lentigo maligno es actualmente la variante más frecuente de melanoma in situ. Se localiza principalmente en la cabeza y el cuello, donde sus características clínicas y dermatoscópicas están descritas ampliamente en la literatura. Presentamos una serie de 14 pacientes diagnosticados de lentigo maligno y lentigo maligno melanoma extrafacial en el Hospital General de Valencia y Hospital de Manacor, describiendo los hallazgos clínicos, dermatoscópicos e histológicos de los mismos. La localización más frecuentemente encontrada fue las extremidades superiores, seguida del tronco y las extremidades inferiores. En la dermatoscopia se hallaron patrones compatibles con lentigo maligno facial y melanoma de extensión superficial. La localización extracefálica de los lentigos malignos es poco frecuente. Clínica e histológicamente muestra características semejantes al de localización facial, aunque en la dermatoscopia podemos encontrar una mezcla de patrones de lentigo maligno y melanoma de extensión superficial, debido principalmente a las diferencias entre las características anatómicas de la piel facial y la extrafacial
Lentigo maligna is the most common form of in situ melanoma. It is most often found on the head and neck, and its clinical and dermoscopic features in this location have been extensively described in the literature. We present a series of 14 patients diagnosed with extrafacial lentigo maligna and lentigo maligna melanoma at Hospital General de Valencia and Hospital de Manacor in Spain, and describe the clinical, dermoscopic, and histologic features observed. Most of the melanomas were located on the upper limbs; the next most common locations were the trunk and the lower limbs. The dermoscopic patterns were consistent with facial lentigo maligna and superficial spreading melanoma. Extrafacial lentigo maligna is uncommon. It has similar clinical and histologic features to facial lentigo, but dermoscopy may show a mix of patterns typically seen in lentigo maligna and superficial spreading melanoma. This difference in dermoscopic features is essentially due to anatomical differences between skin on the face and on other parts of the body
Subject(s)
Humans , Male , Female , Aged , Aged, 80 and over , Middle Aged , Hutchinson's Melanotic Freckle/pathology , Skin Neoplasms/pathology , Hutchinson's Melanotic Freckle/diagnostic imaging , Dermoscopy , Extremities , Giant Cells/ultrastructure , Melanocytes/ultrastructure , Skin Neoplasms/diagnostic imaging , TorsoABSTRACT
Lentigo maligna is the most common form of in situ melanoma. It is most often found on the head and neck, and its clinical and dermoscopic features in this location have been extensively described in the literature. We present a series of 14 patients diagnosed with extrafacial lentigo maligna and lentigo maligna melanoma at Hospital General de Valencia and Hospital de Manacor in Spain, and describe the clinical, dermoscopic, and histologic features observed. Most of the melanomas were located on the upper limbs; the next most common locations were the trunk and the lower limbs. The dermoscopic patterns were consistent with facial lentigo maligna and superficial spreading melanoma. Extrafacial lentigo maligna is uncommon. It has similar clinical and histologic features to facial lentigo, but dermoscopy may show a mix of patterns typically seen in lentigo maligna and superficial spreading melanoma. This difference in dermoscopic features is essentially due to anatomical differences between skin on the face and on other parts of the body.
Subject(s)
Hutchinson's Melanotic Freckle/pathology , Skin Neoplasms/pathology , Aged , Aged, 80 and over , Dermoscopy , Extremities , Female , Giant Cells/ultrastructure , Humans , Hutchinson's Melanotic Freckle/diagnostic imaging , Male , Melanocytes/ultrastructure , Middle Aged , Skin Neoplasms/diagnostic imaging , TorsoABSTRACT
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
Subject(s)
Child , Child, Preschool , Female , Humans , Infant , Infant, Newborn , Male , Hemangioma, Capillary/diagnosis , Hemangioma, Capillary/drug therapy , Hemangioma, Capillary/pathology , Hemangioma, Capillary/complications , Kasabach-Merritt Syndrome , Aspirin/therapeutic use , Propranolol/therapeutic use , Prednisone/therapeutic use , Epidemiological Monitoring/trends , Neoplasms, Vascular Tissue/pathology , Congenital Abnormalities/pathology , Vascular System Injuries/pathology , Hemangiopericytoma , Sarcoma, Kaposi , Hemangioendothelioma , Diagnosis, Differential , Retrospective Studies , Observational Study , Spain/epidemiologyABSTRACT
No disponible
Subject(s)
Female , Humans , Middle Aged , Dermatitis, Photoallergic/diagnosis , Cheilitis/chemically induced , Chlorpromazine/adverse effects , Drug Hypersensitivity/diagnosisSubject(s)
Cheilitis/chemically induced , Chlorpromazine/adverse effects , Dermatitis, Photoallergic/etiology , Eyelid Diseases/chemically induced , Brain Injuries, Traumatic/drug therapy , Chlorpromazine/therapeutic use , Dopamine Antagonists/adverse effects , Dopamine Antagonists/therapeutic use , Drug Substitution , Female , Hand Dermatoses/chemically induced , Humans , Irritable Bowel Syndrome/drug therapy , Methotrimeprazine/therapeutic use , Middle Aged , Patch TestsABSTRACT
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.
Subject(s)
Hemangioma/diagnosis , Skin Neoplasms/diagnosis , Child , Female , Hemangioma/therapy , Humans , Infant , Infant, Newborn , Male , Neoplasm Regression, Spontaneous , Retrospective Studies , Skin Neoplasms/therapyABSTRACT
No disponible
Subject(s)
Humans , Female , Adult , Nevus/diagnosis , Nevus/pathology , Skin Neoplasms/diagnosis , Melanoma/chemically induced , Melanoma/complications , Melanoma/diagnosis , Melanoma/physiopathology , Melanocytes , Melanocytes/pathology , MelanocytesABSTRACT
INTRODUCCIÓN: Diversos estudios han demostrado el valor pronóstico de la técnica de la biopsia del ganglio centinela (BGC) y el índice mitótico (IM) en el melanoma. Sin embargo, la relación entre ambos factores no está bien establecida. OBJETIVOS: El objetivo principal del estudio es describir y analizar la relación entre el resultado de la BGC y el IM en los pacientes con melanoma atendidos en nuestro centro. MÉTODO: En total se incluyeron 139 pacientes en los que se realizó la BGC de forma consecutiva entre mayo de 2001 y mayo de 2009. La relación entre el IM y el resultado de la BGC se ha realizado mediante el test χ2 y el test exacto de Fischer. RESULTADOS: Se detectó una correlación no significativa entre estas 2 variables con p = 0,071. En el subgrupo de pacientes que tenían un espesor de Breslow entre 4 y 1 mm el resultado fue una asociación estadísticamente significativa entre el IM y el resultado de la BGC con p = 0,034. La odds ratio para tener un ganglio positivo teniendo un IM < 1 en este subgrupo es de 0,838 (IC 95%: 0,758-0,926). DISCUSIÓN: Nuestro resultado apoya la utilización del IM como factor predictivo del resultado de la BGC en melanomas de espesor intermedio y apoya la necesidad de estudiar la relación entre estos factores para melanomas finos y gruesos
BACKGROUND: The prognostic value of sentinel lymph node (SLN) biopsy findings and mitotic activity in melanoma has been confirmed in the literature, but the relation between them has not been well established. OBJECTIVES: The main objective was to describe and analyze the correlation between SLN biopsy results and the mitotic rate in patients treated for melanoma in our hospital. METHODS: A total of 139 consecutive patients who underwent SLN biopsy between May 2001 and May 2009 were included. The relation between the mitotic rate and SLN status was analyzed with the χ2 test and the Fisher exact test. RESULTS: The correlation between the 2 variables was nonsignificant (P =0.071) in the patient series overall, but a significant association was found in the subgroup of patients with tumors of Breslow thickness between 1 and 4 mm (P =0.034). The likelihood (odds ratio) of SLN positivity with a mitotic rate of less than 1 mitosis/mm2 in this subgroup was 0.838 (95% CI, 0.758-0.926). CONCLUSIONS: Our findings support use of the mitotic rate to predict SLN status in melanoma tumors of intermediate thickness. Our study also shows the need for further investigation of the relation between these 2 variables in thin and thick tumors
Subject(s)
Humans , Male , Female , Melanoma/complications , Melanoma/pathology , Melanoma/therapy , Mitotic Index/statistics & numerical data , Mitotic Index/trends , Mitotic Index , Lymphedema/mortality , Lymphedema/pathology , Seroma/pathology , Thrombophlebitis/complications , Thrombophlebitis/pathologyABSTRACT
BACKGROUND: The prognostic value of sentinel lymph node (SLN) biopsy findings and mitotic activity in melanoma has been confirmed in the literature, but the relation between them has not been well established. OBJECTIVES: The main objective was to describe and analyze the correlation between SLN biopsy results and the mitotic rate in patients treated for melanoma in our hospital. METHODS: A total of 139 consecutive patients who underwent SLN biopsy between May 2001 and May 2009 were included. The relation between the mitotic rate and SLN status was analyzed with the χ(2) test and the Fischer exact test. RESULTS: The correlation between the 2 variables was nonsignificant (P =.071) in the patient series overall, but a significant association was found in the subgroup of patients with tumors of Breslow thickness between 1 and 4mm (P =.034). The likelihood (odds ratio) of SLN positivity with a mitotic rate of less than 1 mitosis/mm2 in this subgroup was 0.838 (95% CI, 0.758-0.926). CONCLUSIONS: Our findings support use of the mitotic rate to predict SLN status in melanoma tumors of intermediate thickness. Our study also shows the need for further investigation of the relation between these 2 variables in thin and thick tumors.
Subject(s)
Lymphatic Metastasis/diagnosis , Melanoma/secondary , Mitotic Index , Skin Neoplasms/pathology , Adult , Aged , Female , Fibrosis , Hair Color , Humans , Kaplan-Meier Estimate , Longitudinal Studies , Lymphatic Metastasis/pathology , Male , Melanoma/mortality , Melanoma/pathology , Middle Aged , Neoplasm Invasiveness , Prognosis , Prospective Studies , Retrospective Studies , Sentinel Lymph Node Biopsy , Skin Neoplasms/mortality , Skin Pigmentation , Skin Ulcer/etiology , Tertiary Care CentersABSTRACT
Introducción: El nevus lentiginoso atípico (NLA) del anciano es una forma peculiar de nevus displásico, que clínicamente puede simular un melanoma maligno, y que histológicamente presenta un patrón lentiginoso con grados variables de atipia en ausencia de nidos dérmicos, que puede sugerir erróneamente el diagnóstico de lentigo melanoma o melanoma lentiginoso. Material y métodos: Hemos recogido 14 casos de nevus melanocítico lentiginoso atípico diagnosticados entre diciembre de 2007 y marzo de 2009 en el Servicio de Dermatología del Hospital General de Valencia. Hemos estudiado sus características clínicas e histopatológicas tras la tinción de las piezas con hematoxilina-eosina, melan-A y Ki67 y las hemos comparado con los datos publicados en la literatura. Resultados: Entre los datos clínicos, el 28% eran menores de 50 años, con una relación entre sexos de 1:1. La mayoría de las lesiones sugerían clínicamente un nevus atípico (8/14) y todas aparecieron en la espalda. Todos los casos presentaron hiperplasia epidérmica lentiginosa irregular, con proliferación de células melanocíticas individuales, limitadas a la membrana basal, en ausencia de nidos dérmicos; solo 4/14 tenían también ascenso epidérmico focal. Todos presentaron atipia citológica (en un 85% de los casos moderada). El índice de proliferación, valorado mediante la tinción con Ki67, fue bajo (<5%) en todos los casos estudiados. Conclusión: Los NLA son lesiones que pueden simular clínica e histológicamente un melanoma, y que se encuentran en el grupo de las lesiones pigmentadas atípicas con patrón lentiginoso: tanto en nuestra serie como en las series previamente publicadas, los hallazgos histológicos y evolución clínica de estos pacientes orientan hacia el diagnóstico de nevus displásico. Actualmente todos los pacientes estudiados están sanos y sin recidivas después de un seguimiento mínimo de 18 meses (AU)
Background: Atypical lentiginous nevus (of the elderly) is a peculiar form of dysplastic nevus. Clinically, this condition can resemble malignant melanoma and histologically, it has a lentiginous pattern with variable degrees of atypia and an absence of dermal nests. These features may lead to an erroneous diagnosis of lentigo maligna melanoma or lentiginous melanoma. Material and methods: We reviewed 14 cases of atypical lentiginous nevus diagnosed at the dermatology department of Hospital General de Valencia in Valencia, Spain between December 2007 and March 2009. We studied the clinical and histopathologic features of the lesions after hematoxylin-eosin, Melan-A, and Ki-67 staining and compared our results to data reported in the literature. Results: Four (28%) of the 14 patients (7 men, 7 women) were under 50 years of age. Clinically, most of the lesions (8/14) resembled atypical nevi and they were all located on the back. Histologically, they all had irregular lentiginous epidermal hyperplasia, with a proliferation of individual melanocytes only in the basal layer of the epidermis and an absence of dermal nests. Focal upward migration of melanocytes into the epidermis was present in just 4 cases. All the lesions had cellular atypia, which was moderate in 85% of cases. The Ki-67 proliferation index was low (<5%) in all the lesions analyzed. Conclusions: Atypical lentiginous nevi, which can be classified as atypical pigmented lesions with a lentiginous pattern, may clinically and histologically resemble melanoma. Our findings support earlier reports that both clinical and histologic findings may suggest a diagnosis of dysplastic nevus. All of the patients in our series are healthy and free of recurrence after 18 months or longer (AU)
Subject(s)
Humans , Male , Female , Adult , Dysplastic Nevus Syndrome/diagnosis , Dysplastic Nevus Syndrome/epidemiology , Dysplastic Nevus Syndrome/pathology , Hutchinson's Melanotic Freckle/diagnosis , Melanoma/diagnosis , ImmunohistochemistryABSTRACT
BACKGROUND: Atypical lentiginous nevus (of the elderly) is a peculiar form of dysplastic nevus. Clinically, this condition can resemble malignant melanoma and histologically, it has a lentiginous pattern with variable degrees of atypia and an absence of dermal nests. These features may lead to an erroneous diagnosis of lentigo maligna melanoma or lentiginous melanoma. MATERIAL AND METHODS: We reviewed 14 cases of atypical lentiginous nevus diagnosed at the dermatology department of Hospital General de Valencia in Valencia, Spain between December 2007 and March 2009. We studied the clinical and histopathologic features of the lesions after hematoxylin-eosin, Melan-A, and Ki-67 staining and compared our results to data reported in the literature. RESULTS: Four (28%) of the 14 patients (7 men, 7 women) were under 50 years of age. Clinically, most of the lesions (8/14) resembled atypical nevi and they were all located on the back. Histologically, they all had irregular lentiginous epidermal hyperplasia, with a proliferation of individual melanocytes only in the basal layer of the epidermis and an absence of dermal nests. Focal upward migration of melanocytes into the epidermis was present in just 4 cases. All the lesions had cellular atypia, which was moderate in 85% of cases. The Ki-67 proliferation index was low (<5%) in all the lesions analyzed. CONCLUSIONS: Atypical lentiginous nevi, which can be classified as atypical pigmented lesions with a lentiginous pattern, may clinically and histologically resemble melanoma. Our findings support earlier reports that both clinical and histologic findings may suggest a diagnosis of dysplastic nevus. All of the patients in our series are healthy and free of recurrence after 18 months or longer.
Subject(s)
Dysplastic Nevus Syndrome/pathology , Skin Neoplasms/pathology , Adult , Aged , Female , Humans , Male , Middle Aged , Retrospective StudiesABSTRACT
El melanoma lentiginoso acral (MLA) supone un 5% de todos los melanomas malignos, y presenta una frecuencia de metástasis loco-regionales mayoral resto de subtipos de melanoma. En la práctica clínica habitual observamos que existe un porcentaje significativo de casos de MLA que presentan recurrencias locales en zonas de piel aparentemente sanas, incluso después de haber realizado una extirpación con márgenes de seguridad adecuados, como en el caso clínico que presentamos. Esto podría estar generado por la presencia del denominado campo de células de melanoma, definido recientemente por Bastian (AU)
Acral lentiginous melanoma (MLA) represents 5% of all malignant melanomas, and has a frequency of loco-regional metastases greater than other subtypes of melanoma. In clinical practice we observed that a significant percentage of cases of MLA that recurrences in apparently healthy areas of skin, even after making excision with adequate safety margins, as in a case that we present. This could be generated by the presence of so-called melanoma field cells recently defined by Bastian (AU)
Subject(s)
Humans , Female , Aged, 80 and over , Melanoma/pathology , Lentigo/pathology , Foot/pathology , Histocytochemistry/methods , Neoplasm Recurrence, Local/pathologySubject(s)
Antibodies, Monoclonal/therapeutic use , Psoriasis/drug therapy , Antibodies, Monoclonal, Humanized , Female , Humans , Interleukin-12 Subunit p40/antagonists & inhibitors , Interleukin-12 Subunit p40/immunology , Middle Aged , Psoriasis/immunology , Quality of Life , Remission Induction , UstekinumabABSTRACT
El bupropion es un antidepresivo poco utilizado en nuestro país, cuyo uso ha sido recientemente aprobado para su uso en la deshabituación tabáquica. Presentamos el caso de una paciente de 41 años que desarrolló un exantema causado por bupropion. Se han descrito diversos efectos secundarios cutáneos de este fármaco que probablemente comencemos a ver en nuestro país tras su comercialización (AU)
Subject(s)
Bupropion/toxicity , Tobacco Use Disorder/therapyABSTRACT
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.
Subject(s)
Leukemia, Lymphocytic, Chronic, B-Cell/complications , Leukemia, Lymphocytic, Chronic, B-Cell/pathology , Lipodystrophy/complications , Lipodystrophy/pathology , Mesentery/pathology , Skin Neoplasms/complications , Skin Neoplasms/pathology , Aged , Humans , Lymphoma, Non-Hodgkin/pathology , Male , Peritoneal Diseases/complications , Peritoneal Diseases/pathologyABSTRACT
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.
Subject(s)
Hodgkin Disease/complications , Sarcoma, Kaposi/complications , Skin Neoplasms/complications , Adult , Disease Susceptibility , Female , Hodgkin Disease/diagnosis , Hodgkin Disease/immunology , Humans , Sarcoma, Kaposi/immunology , Skin Neoplasms/immunologyABSTRACT
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.
