Hyperbaric oxygen management of recurrent cellulitis in poikiloderma with neutropenia.

Poikiloderma with neutropenia (PN), is a rare autosomal recessive condition with many associated complications and manifestations. Here we present a patient with confirmed PN who is of one-quarter Chucktaw or Cherokee heritage with no known descent from the Navajo tribe. The patient's condition was complicated by chronic bilateral lower limb cellulitis and associated osteomyelitis which was unresponsive to extensive antibiotic regimens. Subsequent treatment with hyperbaric oxygen therapy (HBOT) was successful. To date, no author has reported on the treatment of recurrent cellulitis using HBOT in this patient population. Based on our experience, HBOT should be considered in patients with PN.

Micose fungoide hipocromiante na infância: avaliação de um caso clínico / Hypopigmented mycosis fungoides in childhood: evaluation of a clinical case

Micose fungoide é uma neoplasia dos linfócitos T helper que afeta a pele e geralmente não apresenta manifestações extracutâneas por muitos anos. Já a micose fungoide hipocromiante (MFH) corresponde a um subtipo daquela com melhor prognóstico e que se apresenta com lesões hipocrômicas a acrômicas, com distribuição mais comum em tronco, regiões proximais de extremidades e cintura pélvica. O diagnóstico é firmado pela apresentação clínica aliada à histopatologia, que evidencia epidermotropismo com linfócitos atípicos. A doença geralmente apresenta boa resposta ao tratamento, o qual pode ser realizado com fototerapia com raios ultravioleta A (UVA), ultravioleta B (UVB), mostarda nitrogenada tópica, carmustina tópica e irradiação cutânea com elétrons. Descreve-se o caso de uma criança com máculas hipocrômicas que surgiram inicialmente no abdome, posteriormente se estendendo para raiz de coxas e região inguinal. As lesões apresentavam descamação fina, porém sem eritema ou prurido e ausência de linfonodomegalia palpável. A princípio o caso foi conduzido como eczemátide hipocromiante, mas o paciente apresentou agravamento importante das lesões em poucos meses apesar do tratamento, sendo assim, aventada a hipótese diagnóstica de MFH. Duas biópsias de pele foram realizadas, e os exames anatomopatológico e imunohistoquímica evidenciaram certo epidermotropismo, mas sem atipia de linfócitos. Foi orientado helioterapia domiciliar, com exposição corporal de 10 minutos no período da manhã, além do uso de emolientes e cuidados gerais com a pele, com relativa boa resposta. O acompanhamento é semestral e caso haja modificação do quadro, a biópsia com estudo histopatológico e a imunohistoquímica serão realizados novamente. (AU)

Mycosis fungoides is a type of cancer of the T helper lymphocytes that affects the skin and generally has no extracutaneous manifestations for many years. Otherwise, hypopigmented mycosis fungoides corresponds to a subtype with better prognosis and presents as hypopigmented or achromatic lesions, with more widespread distribution in the trunk, proximal parts of extremities and pelvic girdle. The diagnosis is confirmed by clinical presentation and histopathology, which shows epidermotropism and atypical lymphocytes. The disease usually show good response to treatment, which is performed with phototherapy with ultraviolet A (UVA), ultraviolet B (UVB), topical nitrogen mustard, topical carmustine and cutaneous irradiation with electrons. We describe the case of a child with hypochromic taint that initially appeared in the abdomen, later extending to root thighs and groin. The lesions had fine scaling, absence of erythema or itching, without the presence of palpable lymph node enlargement. At first the case was conducted as pytiriasis alba, but the patient had significant aggravation of injuries in a few months in spite of the treatment, thus mooted diagnosis of hypopigmented mycosis fungoides. Two skin biopsies were performed, and the histological and immunohistochemical tests showed epidermotropism but no atypia of lymphocytes. It was guided home heliotherapy, with body exposure of 10 minutes in the morning, besides the use of emollients and general skin care, with a relatively good response. The follow-up is bi-annually and if there is a modification in the condition, the biopsy with histopathological study and immunohistochemistry will be performed again. (AU)

Alexandrite Laser for the Treatment of Resistant and Hypertrophic Port Wine Stains: A Clinical, Histological and Histochemical Study / Laser de Alejandrita para el tratamiento de malformaciones capilares tipo mancha en vino de Oporto resistentes y/o hipertróficas: estudio clínico, histológico e histoquímico

BACKGROUND: Port wine stains (PWSs) are commonly treated by the pulsed dye laser. However, treatment of hypertrophic or resistant PWSs is a major therapeutic challenge. The long-pulsed Alexandrite laser could be a safe and effective treatment for resistant PWSs, due to an increase depth of penetration of 50-75% over PDL. OBJECTIVE: The aim of this study was to assess the efficacy and safety of a long-pulsed Alexandrite laser in patients with hypertrophic, dark and/or resistant PWSs. Pink pale resistant PWS were excluded from the study. METHODS: Twenty-one patients (age 20-75 years), phototypes I-IV on the Fitzpatrick scale, with PDL dark resistant PWSs were treated with long-pulsed Alexandrite laser. We excluded high phototypes and PDL pink resistant PWSs. All patients were treated with 3 laser sessions at settings of 3-ms pulse duration, 10-mm spot, 35-55J/cm2, with cooling (Dynamic Cooling Device 50 ms with delay 30 ms). Laser sessions were repeated approximately every 2 months. Three dermatologists evaluated treatment effectiveness by means of photographs of the patients before and after laser treatment (scale from 0 to 4). Adverse events were registered. Patient satisfaction was also assessed (scale from 0 to 10). RESULTS: Mean global improvement was rated as 2.28. Long-lasting side effects included minimal scarring after blistering in 1 patient. Mean patient satisfaction was 8.5. CONCLUSIONS: Our study concludes that long-pulsed Alexandrite laser was effective for treatment of resistant PWSs, although the therapeutical window is narrow with this treatment

ANTECEDENTES: Las manchas en vino de oporto (MVO) son normalmente tratadas con láser de colorante pulsado. Sin embargo, el tratamiento de MVO hipertróficas o resistentes continúa siendo un importante reto terapéutico. El láser de Alejandrita de pulso largo podría ser un método seguro y eficaz para el tratamiento de estas lesiones, debido a que la profundidad que alcanza puede superar entre un 50-75% al láser de colorante pulsado. OBJETIVO: El objetivo de este estudio fue evaluar la eficacia y la seguridad del láser de Alejandrita de pulso largo en pacientes con MVO hipertróficas y/o resistentes. Los pacientes con MVO resistentes de color rosa pálido fueron excluidos del estudio. MÉTODOS: Veintiún pacientes (Edades entre 20-75 años), fototipos I-IV en la escala Fitzpatrick, con MVO oscuras, resistentes al tratamiento con láser de colorante pulsado fueron tratados con láser de Alejandrita de pulso largo. Se excluyeron los fototipos altos y las MVO de color rosado pálido. Todos los pacientes fueron tratados con 3 sesiones de láser con los siguientes parámetros: duración de pulso de 3 ms, spot de 10 mm de diámetro, fluencias comprendidas entre 35 y 55 J/cm2, con refrigeración (Dynamic Coolong Device). El intervalo de tiempo entre sesiones fue de 2 meses aproximadamente. Tres dermatólogos evaluaron la efectividad del tratamiento a través de las fotografías de los pacientes antes y después del tratamiento con láser (escala de 0 a 4). Se registraron los eventos adversos acontecidos. La satisfacción del paciente también se evaluó (escala de 0 a 10). RESULTADOS: La mejoría global media fue de 2,28. Los efectos adversos duraderos fueron lesiones cicatriciales mínimas en un paciente. La satisfacción media de los pacientes fue de 8,5. CONCLUSIONES: Nuestro estudio concluye que el láser de Alejandrita de pulso largo puede ser eficaz en el tratamiento de MVO resistentes, aunque la ventana terapéutica es estrecha con este tratamiento