Does the new staging system proposed for mycosis fungoides and Sézary syndrome provide reliable agreement for T1 and T2 disease?

BACKGROUND: Patch or plaque stages in mycosis fungoides (MF) have different prognoses. The recent staging system proposed for MF discriminates between patches and plaques based upon clinical features. OBJECTIVE: To estimate interdermatologist agreement on the morphological evaluation of MF lesions. METHODS: Twenty-four patients with MF were enrolled. Two dermatologists evaluated every lesion face to face and independently with respect to the patch-plaque status. Cohen's κ was used to determine the rate of agreement. RESULTS: Agreement was 67% with respect to the patch or plaque status [95% confidence interval (CI) = 49-85%; p < 0.001]. Current systemic treatment (56%; p = 0.01) was associated with lower agreement. Younger age at diagnosis [odds ratio (OR) 1.03 (95% CI 1.02-1.05)], younger age at enrolment [OR 1.03 (95% CI 1.02-1.04)] and time on systemic treatment [OR 1.02 (95% CI 1.01-1.04)] were independent risk factors for disagreement (p < 0.001). CONCLUSION: The new system for MF staging carries a significant risk of disagreement regarding patch and plaque subsets.

Treatment of stage Ia and Ib mycosis fungoides with psoralen UVA monotherapy: an observational study in tertiary hospitals in the Canary Islands.

BACKGROUND: Mycosis fungoides (MF) is the most common form of primary cutaneous T cell lymphoma. Psoralen combined with ultraviolet A (PUVA) is a first-line treatment for early-stage disease. OBJECTIVES: This study was conducted to assess the clinical effectiveness of and tolerance to PUVA monotherapy in MF. METHODS: We retrospectively reviewed the files of patients who received PUVA for stage I disease. The study included 31 patients, of whom 32% presented with stage Ia and 67% with stage Ib disease, and 68% presented with patch and 32% with plaque disease. All patients received treatment three times per week. RESULTS: Complete response (CR) was achieved in 71% of patients. The median cumulative dose of UVA at CR was 211.7 J/cm(2) . There was a significant difference in median cumulative dose at CR between patients with plaque and patch disease, respectively, but not between patients with stage Ia and Ib disease. Median disease-free survival (DFS) was 230 weeks. Patients with patch disease achieved longer DFS than those with plaque disease (P = 0.004), although DFS was similar in stage Ia and Ib patients. Of the patients who received maintenance therapy, 58% relapsed. Univariate analysis showed patch disease to be a predictive factor for CR, but no predictors of relapse were identified. A total of 71% of patients developed clinical adverse reactions. CONCLUSIONS: Psoralen with UVA is a safe and effective treatment for early-stage MF. Patch disease responds more favorably than plaque disease and is associated with a longer period of DFS. Maintenance treatment does not appear to reduce recurrence. Current evidence suggests that the proposed revision to the classification of MF, which takes into account the extent and type of disease, more accurately predicts response to PUVA.

Contact urticaria due to phenoxyethanol in an aftershave.

The occurrence of pruritus immediately after application of an aftershave product is usually due to irritant contact dermatitis. We report a case of contact urticaria in a male patient, produced by an aftershave product containing phenoxyethanol.

Office-based radiation therapy for cutaneous carcinoma: evaluation of 710 treatments.

Skin cancers are the most common malignancies and, historically, ionizing radiation has played an important role in their treatment. However, less experienced dermatologists generally consider radiation last in the line of therapeutic options. The authors sought to evaluate the effectiveness and safety of office-based elective radiation therapy for cutaneous carcinoma. A retrospective study of 604 basal cell carcinomas (BCCs) and 106 squamous cell carcinomas (SCCs) irradiated between 1971-96 was performed. The percentage of patients who developed tumor recurrence during the follow-up period was calculated using Kaplan-Meier survival curves. Cox's proportional model was used to assess the prognostic factors that might have influenced the recurrences. The recurrence rates for BCC and SCC were 11.5 and 16.5 per 1000 patient-years, respectively. The 5-year cure rates were 94.4% for BCC and 92.7% for SCC, and the 15-year cure rates were 84.8% and 78.6%, respectively. Tumor location on the nasolabial fold (OR 4.4; 95% IC, 1.3-14.7) and tumor size > or = 10 mm (OR 2.14; 95% IC, 1.03-4.45) were independent predictors of BCC recurrence. This study suggested that radiation therapy is an effective treatment for BCC and SCC and should be considered as a first option in many cases.

Dermatosis ampollosa IgA lineal del adulto tratada con colchicina / Linear IgA bullous dermatosis of adults treated with colchicine

No disponible

Lupus eritematoso neonatal: 4 casos y revisión clínica / Neonatal lupus erythematosus: 4 cases and clinical review

El lupus eritematoso neonatal (LEN) es una enfermedad poco frecuente del recién nacido debido al paso transplacentario de anticuerpos maternos anti-Ro/SSA, anti-La/ SSB y/o anti-U1RNP en la que las afectaciones cutáneas y cardiacas son las más destacadas. Realizamos un estudio retrospectivo de casos diagnosticados de LEN en los últimos 10 años en el Hospital Universitario Insular de Gran Canaria en el que se obtuvieron datos completos de 4 enfermos. En 3 casos se presentaron anticuerpos circulantes anti-Ro en la madre y en los neonatos, mientras que en el otro era anti-RNP. Dos madres estaban diagnosticadas de lupus sistémico, una de enfermedad mixta del tejido conjuntivo y otra de vasculitis leucocitoclástica. Las lesiones cutáneas consistieron en lesiones urticariformes y descamativas. Un paciente presentó ulceración. El estudio histológico de las lesiones urticariformes mostró un infiltrado perivascular inespecífico; las lesiones descamativas fueron compatibles con lupus eritematoso subagudo

Neonatal lupus erythematosus (NLE) is an infrequent disease in newborns caused by the transplacental passage of maternal Anti-Ro/SSA, Anti-La/SSB and/or Anti-U1 RNP antibodies. The most common manifestations are cutaneous and cardiac. We carried out a retrospective study of cases of NLE diagnosed in the last 10 years at the Hospital Universitario Insular in Gran Canaria. Complete data was obtained for 4 patients. Three cases had circulating Anti-Ro antibodies in the mother and in the newborns, while in the fourth case they were Anti-RNP. Two mothers were diagnosed with systemic lupus, one with mixed connective tissue disease and the other with leucocytoclastic vasculitis. The skin lesions consisted of urticaria-like and desquamative lesions. One patient presented with ulceration. The histological study of the urticaria-like lesions showed a non-specific perivascular infiltrate; the desquamative lesions were consistent with subacute lupus erythematosus

Linfoma primario cutáneo B de célula grande difuso tipo piernas según la nueva clasificación de la OMS-EORTC. Dos casos / Primary cutaneous diffuse large B-cell lymphoma of the leg according to the new WHO-EORTC classification. Two cases

Los linfomas primarios cutáneos son un grupo heterogéneo de procesos linfoproliferativos que se caracterizan por afectación cutánea sin evidencia de enfermedad sistémica en el momento del diagnóstico. Por lo general tienen un comportamiento clínico indolente, y sólo ocasionalmente suele observarse el desarrollo de enfermedad extracutánea. A partir de la década de 1980, se han considerado los linfomas primarios cutáneos de células B como un grupo específico de linfomas, diferenciándose tanto de los linfomas de células T como de los linfomas cutáneos secundarios de células B. Tanto la EORTC como la OMS han propuesto clasificaciones alternativas para estas entidades con discrepancias importantes que últimamente se han aclarado elaborando una nueva clasificación (clasificación de la OMS-EORTC de los linfomas cutáneos) que unifica criterios hasta ahora dispares. Se presentan dos nuevos casos de linfoma primario cutáneo B de célula grande difuso tipo piernas según esta nueva clasificación

Primary cutaneous lymphomas are a heterogeneous group of lymphoproliferative disorders characterized by skin involvement with no evidence of systemic disease at the time of diagnosis. Their clinical behavior is generally indolent, and only occasionally is the development of extracutaneous disease observed. Since the 1980s, primary cutaneous B-cell lymphomas have been considered a specific group of lymphomas, differentiated from both T-cell lymphomas and from secondary cutaneous B-cell lymphomas. Both the EORTC and the WHO have proposed alternative classifications for these entities, with significant discrepancies that were finally resolved through the development of a new classification (WHO-EORTC classification for cutaneous lymphomas), which standardizes criteria that had previously been different. We present two new cases of primary cutaneous diffuse large B-cell lymphoma of the leg according to the new classification

[Primary cutaneous diffuse large B-cell lymphoma of the leg according to the new WHO-EORTC classification. Two cases]. / Linfoma primario cutáneo B de célula grande difuso tipo piernas según la nueva clasificación de la OMS-EORTC. Dos casos.

Primary cutaneous lymphomas are a heterogeneous group of lymphoproliferative disorders characterized by skin involvement with no evidence of systemic disease at the time of diagnosis. Their clinical behavior is generally indolent, and only occasionally is the development of extracutaneous disease observed. Since the 1980s, primary cutaneous B-cell lymphomas have been considered a specific group of lymphomas, differentiated from both T-cell lymphomas and from secondary cutaneous B-cell lymphomas. Both the EORTC and the WHO have proposed alternative classifications for these entities, with significant discrepancies that were finally resolved through the development of a new classification (WHO-EORTC classification for cutaneous lymphomas), which standardizes criteria that had previously been different. We present two new cases of primary cutaneous diffuse large B-cell lymphoma of the leg according to the new classification.

[Neonatal lupus erythematosus: 4 cases and clinical review]. / Lupus eritematoso neonatal: 4 casos y revisión clínica.

Neonatal lupus erythematosus (NLE) is an infrequent disease in newborns caused by the transplacental passage of maternal Anti-Ro/SSA, Anti-La/SSB and/or Anti-U1 RNP antibodies. The most common manifestations are cutaneous and cardiac. We carried out a retrospective study of cases of NLE diagnosed in the last 10 years at the Hospital Universitario Insular in Gran Canaria. Complete data was obtained for 4 patients. Three cases had circulating Anti-Ro antibodies in the mother and in the newborns, while in the fourth case they were Anti-RNP. Two mothers were diagnosed with systemic lupus, one with mixed connective tissue disease and the other with leucocytoclastic vasculitis. The skin lesions consisted of urticaria-like and desquamative lesions. One patient presented with ulceration. The histological study of the urticaria-like lesions showed a non-specific perivascular infiltrate; the desquamative lesions were consistent with subacute lupus erythematosus.

Vasculopatía livedoide (atrofia blanca) generalizada en pacientes adultos con dermatomiositis / Widespread livedoid vasculopathy (white atrophy) in adult patients with dermatomyositis

La presencia de úlceras en la dermatomiositis del adulto es un hecho infrecuente, que suele asociarse a un peor pronóstico. Hasta la fecha, estos casos no han sido estudiados desde el punto de vista etiopatogénico. Se presentan 2 casos de dermatomiositis del adulto asociados a úlceras cutáneas: un varón de 42 años afectado de una dermatomiositis agresiva que le llevó a la muerte por sepsis, y una mujer de 58 años con una dermatomiositis amiopática de 3 años de evolución. Ambos casos presentaban úlceras cutáneas en distintas localizaciones que, al curar, dejaban una cicatriz atrófica y blanquecina. El estudio histopatológico mostraba trombos de fibrina en ausencia de infiltrado inflamatorio y dilataciones luminales con una pared engrosada que contenía un material hialino, hallazgos que sugerían una vasculopatía livedoide (AU)