[Discrasia linfoide epiteliotrópica de células T. Tratamiento con radiación ultravioleta].

INTRODUCTION: T-cell lymphoid dyscrasia is a group of different, infrequent, epitheliotropic inflammatory dermatosis that precede cutaneous T-cell lymphomas. Treatment with ultraviolet radiation is safe and efficacious. OBJECTIVE: To describe responses to treatment, secondary effects and complications. METHOD: Eleven patients with different T-cell lymphoid dyscrasias were treated. Two received psoralens plus ultraviolet A radiation (PUVA) and nine narrow band ultraviolet B radiation (NB-UVB). RESULTS: Seven women and four men from 5 to 30 years-old and phototypes III and IV were included. They had hypopigmented epitheliotropic lymphoid dyscrasia, chronic pityriasis lichenoides, hypopigmented parapsoriasis and pigmented purpuric dermatoses. Two received PUVA and 9 NB-UVB. Eight achieved complete response and three partial response with 10 to 119 sessions. Any patient presented neither relapse nor progression to cutaneous T-cell lymphoma during treatment or in the follow up period after 34 to 247 sessions. Secondary effects were xerosis and mild pruritus. CONCLUSIONS: The majority of patients with T-cell lymphoid dyscrasias can be successfully and safely treated with minimal secondary effects after a variable number of sessions and periods of time with ultraviolet radiation.

INTRODUCCIÓN: Las discrasias linfoides epiteliotrópicas de células T son un grupo de dermatosis inflamatorias poco frecuentes que pueden progresar a linfomas cutáneos. El tratamiento con radiación ultravioleta ha sido descrito como seguro y eficaz. OBJETIVO: Describir la respuesta al tratamiento, sus efectos secundarios y complicaciones. MÉTODO: Se administró radiación ultravioleta A con psoralenos (PUVA) a dos pacientes y radiación ultravioleta B de banda estrecha (NB-UVB) a nueve pacientes, con diferentes discrasias. RESULTADOS: Conformaron la serie siete mujeres y cuatro hombres, con edades de 5 a 30 años y con fototipos III y IV, con diagnóstico de discrasia linfoide epiteliotrópica hipopigmentada, pitiriasis liquenoide crónica, parapsoriasis hipopigmentada y púrpura pigmentada. Dos recibieron PUVA y nueve recibieron NB-UVB. Ocho alcanzaron respuesta completa y tres respuesta parcial con 10 a 119 sesiones de tratamiento. Ningún paciente presentó reactivación de la enfermedad después de 34 a 247 sesiones, y ninguno en tratamiento o seguimiento evolucionó a linfoma cutáneo. Los efectos secundarios fueron xerosis y prurito. CONCLUSIÓN: El tratamiento de las discrasias linfoides con radiación ultravioleta es eficaz y seguro, con efectos secundarios leves y con respuestas exitosas en la mayoría de los pacientes, en tiempo y número de sesiones variables.

Blocking or enhancing effects of some basic emollients in UVA penetration.

BACKGROUND: Topical agents used in combination with phototherapy or photochemotherapy may have both blocking or enhancing effects in ultraviolet rays. OBJECTIVE: In this in vivo study, the effects of topical petrolatum, basis cream, glycerine, and olive oil on the transmission of ultraviolet A radiation were investigated. METHODS: A test was performed to determine the minimal phototoxic dose on 29 volunteers with only psoralen plus ultraviolet A (PUVA) and then the same test was repeated with white petrolatum, basis cream, glycerine, olive oil, and sunscreen (0.3cc/25cm2). The effects of each agent on the minimal phototoxic dose were determined after 72 h. RESULTS: When compared to pure PUVA, there was a statistically significant increase in the mean minimal phototoxic dose values by the application of white petrolatum (P = 0.011), but there was no significant increase or decrease in the mean minimal phototoxic dose values after the application of basis cream (P = 0.326), glycerine (P = 0.611) or olive oil (P = 0.799). STUDY LIMITATIONS: Low number of patients Conclusion: The application of white petrolatum, which has a blocking effect, and also of basis cream immediately before PUVA therapy should not be recommended. Although we specify that glycerine and maybe olive oil can be used before photochemotherapy, there is a need for further research in larger series.

The efficacy and safety of phototherapy in geriatric patients: a retrospective study.

BACKGROUND: While phototherapy is a well-established treatment for many dermatoses, data from the literature regarding its use in elderly patients are quite limited. OBJECTIVE: In this study, we aimed to determine the phototherapy indications in geriatric patients and to evaluate the effectiveness and reliability of phototherapy in this group. METHODS: This study included 95 patients of 65 years of age and older who were treated in our phototherapy unit between 2006 and 2015. The data for this study were collected retrospectively from patient follow-up forms in the phototherapy unit. RESULTS: Phototherapy was administered to 28 (29.5%) patients for mycosis fungoides, 25 (26.3%) patients foplaque type psoriasis, 12 (12.6%) patients for palmoplantar psoriasis, 12 (12.6%) patients for generalized pruritus, and 18 (19%) for other dermatoses. Of the patients, 64.2% had received a narrowband UVB (NB-UVB), 21.1% oral psoralen UVA (PUVA), and 14.7% local PUVA treatment. A complete response was achieved in 76.9-85.7% of the mycosis fungoides and in 73.71-100% of the psoriasis vulgaris patients treated with NB-UVB and PUVA, respectively. All the patients with generalized pruritus were treated with NB-UVB, and 80% of these patients achieved significant improvement. The erythema rate was found to be 0.43% per session for NB-UVB treatment and 0.46% per session for PUVA treatment as a side effect. STUDY LIMITATIONS: The limitations of our study are that it was retrospective and the remission durations of the patients are not known. CONCLUSION: This study showed that phototherapy is effective and reliable in the elderly population with proper dose increases and close follow-up.

Blocking or enhancing effects of some basic emollients in UVA penetration

Abstract: Background: Topical agents used in combination with phototherapy or photochemotherapy may have both blocking or enhancing effects in ultraviolet rays. Objective: In this in vivo study, the effects of topical petrolatum, basis cream, glycerine, and olive oil on the transmission of ultraviolet A radiation were investigated. Methods: A test was performed to determine the minimal phototoxic dose on 29 volunteers with only psoralen plus ultraviolet A (PUVA) and then the same test was repeated with white petrolatum, basis cream, glycerine, olive oil, and sunscreen (0.3cc/25cm2). The effects of each agent on the minimal phototoxic dose were determined after 72 h. Results: When compared to pure PUVA, there was a statistically significant increase in the mean minimal phototoxic dose values by the application of white petrolatum (P = 0.011), but there was no significant increase or decrease in the mean minimal phototoxic dose values after the application of basis cream (P = 0.326), glycerine (P = 0.611) or olive oil (P = 0.799). Study limitations: Low number of patients Conclusion: The application of white petrolatum, which has a blocking effect, and also of basis cream immediately before PUVA therapy should not be recommended. Although we specify that glycerine and maybe olive oil can be used before photochemotherapy, there is a need for further research in larger series.

The efficacy and safety of phototherapy in geriatric patients: a retrospective study

Abstract: Background: While phototherapy is a well-established treatment for many dermatoses, data from the literature regarding its use in elderly patients are quite limited. Objective: In this study, we aimed to determine the phototherapy indications in geriatric patients and to evaluate the effectiveness and reliability of phototherapy in this group. Methods: This study included 95 patients of 65 years of age and older who were treated in our phototherapy unit between 2006 and 2015. The data for this study were collected retrospectively from patient follow-up forms in the phototherapy unit. Results: Phototherapy was administered to 28 (29.5%) patients for mycosis fungoides, 25 (26.3%) patients foplaque type psoriasis, 12 (12.6%) patients for palmoplantar psoriasis, 12 (12.6%) patients for generalized pruritus, and 18 (19%) for other dermatoses. Of the patients, 64.2% had received a narrowband UVB (NB-UVB), 21.1% oral psoralen UVA (PUVA), and 14.7% local PUVA treatment. A complete response was achieved in 76.9-85.7% of the mycosis fungoides and in 73.71-100% of the psoriasis vulgaris patients treated with NB-UVB and PUVA, respectively. All the patients with generalized pruritus were treated with NB-UVB, and 80% of these patients achieved significant improvement. The erythema rate was found to be 0.43% per session for NB-UVB treatment and 0.46% per session for PUVA treatment as a side effect. Study limitations: The limitations of our study are that it was retrospective and the remission durations of the patients are not known. Conclusion: This study showed that phototherapy is effective and reliable in the elderly population with proper dose increases and close follow-up.

Hyperpigmentation as a peculiar presentation of mycosis fungoides.

Hyperpigmented mycosis fungoides is an extremely rare subtype of mycosis fungoides. It presents as multiple pigmented macules and patches without poikilodermatous changes and characterized by a CD8+ phenotype on immunohistochemistry. This report describes a typical case of hyperpigmented mycosis fungoides in a 62-year-old woman, who presented with a 7-year history of multiple hyperpigmented macules and patches on the trunk and right leg with progression over this half a year. Histology and immunohistochemical staining of skin samples confirmed the diagnosis of mycosis fungoides. She received psoralen plus ultraviolet A (PUVA) therapy. After an 8-week treatment, the erythematous changes cleared without recurrence during a 6-month follow-up period. An intractable hyperpigmented patch should raise the clinical suspicion of mycosis fungoides with sequential skin biopsy.

Hyperpigmentation as a peculiar presentation of mycosis fungoides

Abstract Hyperpigmented mycosis fungoides is an extremely rare subtype of mycosis fungoides. It presents as multiple pigmented macules and patches without poikilodermatous changes and characterized by a CD8+ phenotype on immunohistochemistry. This report describes a typical case of hyperpigmented mycosis fungoides in a 62-year-old woman, who presented with a 7-year history of multiple hyperpigmented macules and patches on the trunk and right leg with progression over this half a year. Histology and immunohistochemical staining of skin samples confirmed the diagnosis of mycosis fungoides. She received psoralen plus ultraviolet A (PUVA) therapy. After an 8-week treatment, the erythematous changes cleared without recurrence during a 6-month follow-up period. An intractable hyperpigmented patch should raise the clinical suspicion of mycosis fungoides with sequential skin biopsy.

Phototherapy as an effective treatment for Majocchi's disease--case report.

Pigmented purpuric dermatoses are chronic and relapsing disorders characterized by a symmetrical rash of petechial and pigmentary macules, mainly confined to the lower limbs. Purpura annularis telangiectodes of Majocchi is a less common variant of Pigmented purpuric dermatoses characterized by punctate telangiectatic macules progressing to annular, hyperpigmented patches with central clearing and infrequent atrophy. A 12 year-old girl presented with asymptomatic round to oval reddish brown macules, present symmetrically over her lower and upper limbs for 3 years. Few lesions were annular in shape. Biopsy from the lesion was compatible with Pigmented purpuric dermatoses. On the basis of clinical and histopathological findings, a diagnosis of Purpura annularis telangiectodes of Majocchi was made. The patient began phototherapy thrice a week and showed excellent response.

Phototherapy as an effective treatment for Majocchi's disease - Case report

Pigmented purpuric dermatoses are chronic and relapsing disorders characterized by a symmetrical rash of petechial and pigmentary macules, mainly confined to the lower limbs. Purpura annularis telangiectodes of Majocchi is a less common variant of Pigmented purpuric dermatoses characterized by punctate telangiectatic macules progressing to annular, hyperpigmented patches with central clearing and infrequent atrophy. A 12 year-old girl presented with asymptomatic round to oval reddish brown macules, present symmetrically over her lower and upper limbs for 3 years. Few lesions were annular in shape. Biopsy from the lesion was compatible with Pigmented purpuric dermatoses. On the basis of clinical and histopathological findings, a diagnosis of Purpura annularis telangiectodes of Majocchi was made. The patient began phototherapy thrice a week and showed excellent response.

Progressive macular hypomelanosis: an epidemiological study and therapeutic response to phototherapy.

BACKGROUND: Progressive macular hypomelanosis is a common dermatosis in various continents. Its cause is unknown and proposed treatments have had little effect. OBJECTIVES: To determine epidemiological aspects of progressive macular hypomelanosis in patients referred to a phototherapy clinic between 1997 and 2008 and to evaluate therapeutic response to PUVA (psoralen + UVA) photochemotherapy or narrowband UVB phototherapy. METHODS: Eighty-four patients with progressive macular hypomelanosis were evaluated. After 16 phototherapy sessions, therapeutic response was classified as: unchanged, slightly improved (<50% of repigmentation), moderately improved (50-79% of repigmentation), much improved (80-99%) or cured (100%). After a minimum of three months, patients whose response was classified as cured or much improved were contacted by telephone to evaluate the persistence of the therapeutic response. RESULTS: Most of the patients were women (79%) and white (85%). Age at onset of progressive macular hypomelanosis ranged from 13 to 36 years. PUVA was prescribed for 27 patients and narrowband UVB phototherapy for 57. No significant difference was found between the outcomes obtained with PUVA and those obtained with narrowband UVB phototherapy (Fisher's exact test; p<0.05). The majority of patients (81%) had 50% or more repigmentation, with 65% being classified as cured or much improved. Nevertheless, there was a recurrence of the lesions in 72% of patients. CONCLUSIONS: The fact that no patients were over 40 years of age suggests that progressive macular hypomelanosis is a self-limiting disease. Both PUVA and narrowband UVB are effective therapeutic options; however, they do not prevent recurrence of the disease.

Hipomelanose macular progressiva: estudo epidemiológico e resposta terapêutica à fototerapia / Progressive macular hypomelanosis: an epidemiological study and therapeutic response to phototherapy

FUNDAMENTOS: A hipomelanose macular progressiva é uma dermatose comum em diferentes continentes. Sua causa é desconhecida e os tratamentos propostos são pouco eficazes. OBJETIVOS: Determinar aspectos epidemiológicos da hipomelanose macular progressiva em pacientes atendidos num setor de fototerapia, no período de 1997 a 2008, e avaliar a resposta terapêutica com PUVA ou UVBNB. MÉTODOS: Foram avaliados 84 pacientes com Hipomelanose Macular Progressiva. Após 16 sessões de fototerapia, a resposta terapêutica foi definida: I=inalterado, MD=melhora discreta(< 50 por cento de repigmentação), MM=melhora moderada(50-79 por cento), MI=melhora intensa(80-99 por cento) e C=cura(100 por cento). Após um tempo mínimo de três meses, pacientes com cura ou MI foram contatados por telefone para avaliar a manutenção do resultado terapêutico. RESULTADOS: Predominaram mulheres (79 por cento) e a cor branca. A idade mínima de aparecimento da Hipomelanose Macular Progressiva foi de 13 anos e a máxima de 36 anos. Fototerapia com PUVA foi indicada em 27 pacientes e UVBNB em 57. Estatisticamente, não houve diferença significante entre o tratamento com PUVA e UVBNB (teste de Fisher P>0.05). A maioria (81 por cento) dos pacientes obteve 50 por cento ou mais de repigmentação e 65 por cento tiveram cura ou MI. Entretanto, 72 por cento apresentaram recorrência das lesões. CONCLUSÃO: A ausência de pacientes, com mais de 40 anos, sugere que a Hipomelanose Macular Progressiva seja uma doença autolimitada. Tanto PUVA como UVB NB são opções terapêuticas, porém não impedem a recidiva da doença.

BACKGROUND: Progressive macular hypomelanosis is a common dermatosis in various continents. Its cause is unknown and proposed treatments have had little effect. OBJECTIVES: To determine epidemiological aspects of progressive macular hypomelanosis in patients referred to a phototherapy clinic between 1997 and 2008 and to evaluate therapeutic response to PUVA (psoralen + UVA) photochemotherapy or narrowband UVB phototherapy. METHODS: Eighty-four patients with progressive macular hypomelanosis were evaluated. After 16 phototherapy sessions, therapeutic response was classified as: unchanged, slightly improved (<50 percent of repigmentation), moderately improved (50-79 percent of repigmentation), much improved (80-99 percent) or cured (100 percent). After a minimum of three months, patients whose response was classified as cured or much improved were contacted by telephone to evaluate the persistence of the therapeutic response. RESULTS: Most of the patients were women (79 percent) and white (85 percent). Age at onset of progressive macular hypomelanosis ranged from 13 to 36 years. PUVA was prescribed for 27 patients and narrowband UVB phototherapy for 57. No significant difference was found between the outcomes obtained with PUVA and those obtained with narrowband UVB phototherapy (Fisher's exact test; p<0.05). The majority of patients (81 percent) had 50 percent or more repigmentation, with 65 percent being classified as cured or much improved. Nevertheless, there was a recurrence of the lesions in 72 percent of patients. CONCLUSIONS: The fact that no patients were over 40 years of age suggests that progressive macular hypomelanosis is a self-limiting disease. Both PUVA and narrowband UVB are effective therapeutic options; however, they do not prevent recurrence of the disease.

Evaluación del resultado de la terapia con puva en pacientes con estadios tempranos de micosis fungoides: seguimiento a 8 años / Eight years follow up of patients with early stage cutaneous T cell Lymphoma in treatment with psoralen plus UVA

Antecedentes: en la actualidad existen muy pocos datos en la literatura acerca de la evolución a largo plazo de los pacientes con micosis fungoides (MF) en tratamiento con PUVA. (fotoquimioterapia con radiación ultravioleta tipo A). Objetivos: Conocer las características epidemiológicas y clínicas de los pacientes con diagnóstico de MF en tratamiento con PUVA en el servicio de dermatología del CES-Sabaneta, y determinar qué impacto tiene la terapia de mantenimiento en la prevención de recaídas y en la sobrevida. Métodos: se realizó un estudio descriptivo y retrospectivo, con los pacientes con diagnóstico de MF desde abril de 1997 hasta junio de 2005.

Background: PUVA is considered first line therapy in early stages of mycosis fungoides. There are few reports in the literature about the long term follow up of these patients. Objective: to describe the epidemiology and clinical characteristics of patients with early stages of mycosis fungoides in PUVA therapy, in the Dermatologic Center CES-Sabaneta. We wanted to know the relationship between PUVA therapy and patient’s evolution, response to treatment, survival rates, relapse and adverse effects. Methods: a single center, retrospective, descriptive study was done. We included all patients with confirmed diagnosis of mycosis fungoides, who assisted PUVA therapy between April of 1997 and June of 2005.

Twice- vs. thrice-weekly MPD PUVA in psoriasis: a randomized-controlled efficacy study.

BACKGROUND: Oral psoralen-ultraviolet A (PUVA) has been the treatment of choice of moderate to severe forms of psoriasis, however there is no standardized worldwide protocol. Twice- and thrice-weekly regimens had been used. In this study we compare the efficacy of these regimens. METHODS: Two PUVA regimens in 28 patients with clinical diagnosis of psoriasis, with symmetric and no less than 20% of body surface area involvement, using a half-body study, were compared. Each patient body half was randomized to receive either twice- or thrice-weekly PUVA. A blinded trained dermatologist assessed the PASI score in each body half at the beginning of the study and at clearance or after 25 sessions. RESULTS: Twenty-three patients completed the study. There were no significant differences in the efficacy of both treatments considering the median percentage of PASI decrease (92.9% for the body half with the twice-weekly regimen and 94.8% for the thrice-weekly treatment, P=0.179). There were significant differences comparing the median number of sessions needed for clearance and the median cumulative doses, favouring the twice-weekly regimen (15 vs. 22, P=0.000, and 142.5 vs. 241.4 J/cm(2), P=0.001, respectively). CONCLUSION: The treatment of psoriasis patients with twice- or thrice-weekly PUVA in this study was equally effective, the number of sessions required and the cumulative doses of UVA were lower with the twice-weekly regimen.

Psoriasis y enfermedades afines: experiencia con Re-PUVA en el manejo de psoriasis moderada-severa / Psoriasis and related diseases: experience with Re-PUVA in the treatment of moderate-to-severe psoriasis

Introducción: El manejo de la psoriasis moderada-severa es complejo, y un importante número de pacientes considera que el tratamiento indicado por su médico tratante no es suficientemente agresivo. El uso de tratamientos combinados ha demostrado mayor efectividad que las monoterapias, logrando aclaración de las lesiones en menor tiempo, con dosis más bajas de agentes terapéuticos y menos efectos adversos. Objetivos: Presentar nuestra experiencia con terapia combinada de retinoides más PUVA (Re-PUVA) en pacientes con psoriasis moderada-severa. Pacientes y Métodos: Se trató a nueve pacientes con psoriasis moderada-severa, mayores de 18 años, con terapia combinada de acitretín+PUVA, evaluando respuesta clínica bajo protocolo y seguimiento fotográfico al inicio 8ª y 16ª semana de tratamiento. Se estimaron dosis requeridas, costos, y efectos adversos del tratamiento. Resultados: El tiempo de tratamiento requerido para conseguir aclaración en el 90 por ciento de los pacientes fue de siete semanas. La dosis promedio de acitretín fue de 033 mg/kg/ día y la dosis acumulada de UVA fue de 139 J/cm². El costo estimado para conseguir aclaramiento en el 90 por ciento de los pacientes fue de US$ 634. No se observaron efectos adversos severos. Conclusión: La terapia combinada Re-PUVA demostró ser una excelente opción terapéutica para psoriasis moderada-severa, lográndose óptimos resultados en un corto plazo, a un costo razonable, y sin efectos adversos severos.

Puerto Rico psoriasis study group: efficacy and safety of etanercept.

BACKGROUND: Although major advances in the understanding of its pathogenesis have been achieved, psoriasis remains an incurable disease. In April 2004, etanercept, an antagonist of TNF-alpha, was approved by the Food and Drug Administration for the treatment of chronic, moderate to severe plaque psoriasis in adults. In this study we intend to document the efficacy and further establish the safety profile of etanercept for the treatment of moderate to severe psoriasis in our population and compare our data to the Leonardi et al study published in 2003. METHODS: A total of 26 patients were followed for a period of 24 weeks. Subjects were administered 25 mg of etanercept subcutaneously twice weekly for 24 weeks. Patients were seen every 4 weeks to measure clinical improvement by means of the psoriasis area and severity index (PASI) scores. Development of side effects was also assessed. RESULTS: Ninety-two percent of the patients had an improvement of greater than 50% in their PASI score, with 79% of these patients with a PASI improvement of 75% or greater. Adverse events were uncommon and none required the permanent discontinuation of treatment. CONCLUSION: Treatment with etanercept was well-tolerated and resulted in significant sustained improvement of psoriasis throughout a period of 24 weeks. Our data strongly correlates with the findings reported by Leonardi et al in 2003.

Generalized eruptive keratoacanthoma (Grzybowski variant).

We describe a patient with generalized eruptive keratoacanthoma (KA) of Grzybowski showing the characteristic features of this extremely rare condition. Since the first description by Grzybowski in 1950, only 28 additional cases (including the present one) have been reported. This variety of KA most commonly affects patients during the fifth to seventh decade of life and appears as a generalized eruption of hundreds to thousands of follicular papules. The small pruriginous papules often have a keratotic centre and show microscopic features of KA. Marked facial involvement is characteristic and can lead to masked facies with ectropion, as in our patient. The course of the disease is chronic and the response to therapy is poor.