Leiomiomatosis cutánea y uterina múltiple o síndrome de Reed: estudio retrospectivo de 13 casos / Multiple Cutaneous and Uterine Leiomyomatosis or Reed Syndrome: A Retrospective Study of 13 Cases

Introducción: La leiomiomatosis cutánea y uterina múltiple (MCUL) o síndrome de Reed se caracteriza por la presencia de leiomiomas cutáneos de origen pilar, leiomiomas uterinos en las mujeres y, en algunos casos, asociación con carcinoma renal. Este síndrome, de herencia autosómica dominante, se produce por una mutación heterocigótica en la línea germinal del gen de la fumarato hidratasa, una enzima del ciclo de Krebs que actúa como supresor tumoral. Objetivo: Revisar los casos de MCUL diagnosticados en 2 hospitales universitarios durante un periodo de 5 años (2008-2013). Material y métodos: Estudio retrospectivo de 13 casos de MCUL, en el que se recogieron características demográficas, clínicas e histológicas, así como posibles asociaciones con otras enfermedades y tratamientos recibidos. Resultados: Trece pacientes fueron diagnosticados de MCUL (10 mujeres y 3 hombres, con una edad media al diagnóstico de 53 años). El 100% de los casos presentaba leiomiomas cutáneos múltiples, distribuidos de forma difusa (69%), agrupada (92%) y/o lineal (7,7%). El 90% de las mujeres presentaba además miomas uterinos y todas ellas habían precisado histerectomía porese motivo. No encontramos ningún caso de carcinoma renal en los pacientes explorados (9/13), pero sí lesiones renales benignas (4/9). Conclusión: Describimos 13 casos de MCUL, que presentan características clínicas e histológicas similares a las descritas en la literatura, siendo la manifestación cutánea más frecuente la segmentaria tipo 2. Es importante que el dermatólogo identifique los casos de leiomiomas cutáneos y conozca su posible relación con MCUL (AU)

Introduction: Multiple cutaneous and uterine leiomyomatosis (MCUL), or Reed syndrome, is characterized by the presence of cutaneous leiomyomas arising from the arrector pili muscles and, in women, by uterine leiomyomas. In some cases, MCUL is associated with renal cell carcinoma. This syndrome is an autosomal dominant disorder caused by a heterozygous germline mutation of the gene that encodes fumarate hydratase, a Krebs cycle enzyme that acts as a tumor suppressor. Objective: To review the cases of MCUL diagnosed at 2 university hospitals over a 5-year period (2008-2013). Material and methods: This was a retrospective study of 13 cases of MCUL that investigated demographic, clinical, and histologic characteristics, as well as possible associations with other diseases and treatments received. Results: We identified 13 patients (10 women and 3 men) who had been diagnosed with MCUL. The mean age at diagnosis was 53 years. All the patients had multiple cutaneous leiomyomas; in 12 (92%) the distribution was clustered and 9 (69%) also had disseminated solitary lesions. In 1 patient (7.7%), the pattern of distribution was linear. Uterine fibroids requiring hysterectomy were present in 90% of the women. Nine patients were screened for renal lesions; no cases of renal cell carcinoma were detected but benign renal lesions were found in 4 patients. Conclusion: The clinical and histologic characteristics of the 13 cases of MCUL reviewed were similar to those reported in the literature. The most common cutaneous manifestation was a type 2 segmental pattern. It is important for dermatologists to identify cutaneous leiomyomas and be aware of the possible association with MCUL (AU)

Multiple cutaneous and uterine leiomyomatosis or reed syndrome: a retrospective study of 13 cases.

INTRODUCTION: Multiple cutaneous and uterine leiomyomatosis (MCUL), or Reed syndrome, is characterized by the presence of cutaneous leiomyomas arising from the arrector pili muscles and, in women, by uterine leiomyomas. In some cases, MCUL is associated with renal cell carcinoma. This syndrome is an autosomal dominant disorder caused by a heterozygous germline mutation of the gene that encodes fumarate hydratase, a Krebs cycle enzyme that acts as a tumor suppressor. OBJECTIVE: To review the cases of MCUL diagnosed at 2 university hospitals over a 5-year period (2008-2013). MATERIAL AND METHODS: This was a retrospective study of 13 cases of MCUL that investigated demographic, clinical, and histologic characteristics, as well as possible associations with other diseases and treatments received. RESULTS: We identified 13 patients (10 women and 3 men) who had been diagnosed with MCUL. The mean age at diagnosis was 53 years. All the patients had multiple cutaneous leiomyomas; in 12 (92%) the distribution was clustered and 9 (69%) also had disseminated solitary lesions. In 1 patient (7.7%), the pattern of distribution was linear. Uterine fibroids requiring hysterectomy were present in 90% of the women. Nine patients were screened for renal lesions; no cases of renal cell carcinoma were detected but benign renal lesions were found in 4 patients. CONCLUSION: The clinical and histologic characteristics of the 13 cases of MCUL reviewed were similar to those reported in the literature. The most common cutaneous manifestation was a type 2 segmental pattern. It is important for dermatologists to identify cutaneous leiomyomas and be aware of the possible association with MCUL.

La formulación magistral en la terapéutica dermatológica actual / Compounding as a Current Therapeutic Option in Dermatology

A pesar de la gran cantidad de productos disponibles producidos por la industria farmacéutica, hoy en día la formulación magistral sigue teniendo un papel muy importante en el tratamiento de las afecciones dermatológicas. Desde los inicios de la Dermatología se ha usado, siendo muy útil en el tratamiento de enfermedades para las que no se disponía de preparados farmacéuticos específicos; sin embargo, a medida que fueron apareciendo nuevos productos comercializados se puso en duda la utilidad de la formulación, cuestionando su seguridad, estabilidad y efectividad. Esto contrasta con la tendencia actual de recuperar la formulación, haciendo que vuelva a ocupar su lugar en la práctica dermatológica habitual. En el presente artículo revisamos las patologías en las que se usa con más frecuencia la formulación, sus utilidades e inconvenientes, la legislación actual al respecto en nuestro ámbito, aportando las últimas novedades descritas en cuanto a vehículos y principios activos disponibles (AU)

Compounding continues to play a key role in the treatment of skin conditions, despite the abundance of products made by the pharmaceutical industry. Right from the earliest days of dermatology, compounding proved very useful in the treatment of diseases for which no specific drugs were available. However, as new products came onto the market, this usefulness was called into question, and doubts over safety, stability, and effectiveness were raised. Today, compounding is regaining the place it once held in routine dermatological practice. We review the advantages and disadvantages of compounding, the most common indications, current legislation in our setting, and the latest developments in active ingredients and vehicles (AU)

Dermanyssus gallinae (chicken mite): an underdiagnosed environmental infestation.

Dermanyssus gallinae is a mite that normally parasitizes small birds but may occasionally bite humans. We report an unusual case of an 82-year-old woman who presented with pruritus and bite-like lesions over her trunk. Other members of the household were also affected. On physical examination, mites < 1 mm in size were found on the patient's body. The family were residing in the city centre and had no pets, but there were pigeon nests in close proximity to the house. Most dermatologists have difficulties identifying ectoparasitosis. In the case of D. gallinae, the small size of the mites and the fact that they leave the host after feeding means that they may not be seen at presentation, thus such infestations are likely to be underdiagnosed. Physicians should be aware that infection with this mite is possible even in patients from urban areas, and it should be included in the differential diagnosis of conditions causing recurrent pruritus unresponsive to standard treatments.

Compounding as a current therapeutic option in dermatology.

Compounding continues to play a key role in the treatment of skin conditions, despite the abundance of products made by the pharmaceutical industry. Right from the earliest days of dermatology, compounding proved very useful in the treatment of diseases for which no specific drugs were available. However, as new products came onto the market, this usefulness was called into question, and doubts over safety, stability, and effectiveness were raised. Today, compounding is regaining the place it once held in routine dermatological practice. We review the advantages and disadvantages of compounding, the most common indications, current legislation in our setting, and the latest developments in active ingredients and vehicles.

Psoriasis ungueal: tratamiento con ungüento hidrófilo de tazaroteno al 0.1 por ciento / Nail Psoriasis: Treatment With Tazarotene 0.1 percent Hydrophilic Ointment

Introducción: La afectación ungueal de la psoriasis es una presentación frecuente que interfiere de manera significativa en la calidad de vida de los pacientes. Su presentación clínica va a depender del área ungueal afecta: lecho o matriz. Un 50% de los pacientes refiere dolor asociado. En este estudio evaluamos la eficacia y seguridad del tazaroteno 0,1% en ungüento hidrófilo. Material y métodos: Estudio abierto y observacional de 6 pacientes diagnosticados de psoriasis ungueal. Se aplicó ungüento de tazaroteno 0,1% (fórmula magistral) en oclusión nocturna, en su domicilio durante 6 meses, sin otro tratamiento tópico o sistémico. Se determinó el Nail Psoriasis Severity Index (NAPSI) y se evaluaron la hiperqueratosis subungueal, onicólisis, hemorragias en astilla, manchas de aceite y piquiteado ungueal, en la visita basal, a los 3 y 6 meses. Resultados: Se observó una mejoría estadísticamente significativa en todos los pacientes: NAPSI basal, media ± DE 14,3± 6,3; IC 95% 11,74-16,92; mediana 15; NAPSI a los 6 meses: media ± DE 2,3 ±1,21; IC 95% 1,84-2,83; mediana 2,5; p=0,007. El porcentaje de mejoría fue del 87,9% al final del tratamiento. No se registraron efectos adversos. Conclusión: Nuestro estudio muestra un potencial terapéutico del ungüento de tazaroteno en la psoriasis ungueal (AU)

Introduction: Nail involvement is common in psoriasis and has a considerable impact on patient quality of life. Its clinical presentation depends on which part of the nail is affected: the bed or the matrix. Fifty percent of patients report associated pain. In this study, we analyzed the safety and effectiveness of tazarotene 0.1% in a hydrophilic ointment in the treatment of nail psoriasis. Material and methods: We performed an open observational study of 6 patients diagnosed with nail psoriasis. The patients applied a compounded preparation of tazarotene 0.1% ointment under occlusion every night for 6 months in their homes. They were not receiving any other topical or systemic treatments. Nail psoriasis severity (assessed using the Nail Psoriasis Severity Index [NAPSI]), subungual hyperkeratosis, onycholysis, splinter hemorrhages, oil stains, and nail pitting were evaluated at baseline and at 3 and 6 months. Results: A statistically significant improvement between baseline and 6 months was observed in all patients: the mean (SD) NAPSI went from 14.3 (6.3; 95% CI, 11.74-16.92) to 2.3 (1.21; 95% CI, 1.84-2.3) while the median went from 15 to 2.5 (P = 0.007). The percentage improvement at the end of treatment was 87.9%. No adverse effects were observed. Conclusion: Our study shows the therapeutic potential of tazarotene ointment in nail psoriasis (AU)

Nail psoriasis: treatment with tazarotene 0.1% hydrophilic ointment.

INTRODUCTION: Nail involvement is common in psoriasis and has a considerable impact on patient quality of life. Its clinical presentation depends on which part of the nail is affected: the bed or the matrix. Fifty percent of patients report associated pain. In this study, we analyzed the safety and effectiveness of tazarotene 0.1% in a hydrophilic ointment in the treatment of nail psoriasis. MATERIAL AND METHODS: We performed an open observational study of 6 patients diagnosed with nail psoriasis. The patients applied a compounded preparation of tazarotene 0.1% ointment under occlusion every night for 6 months in their homes. They were not receiving any other topical or systemic treatments. Nail psoriasis severity (assessed using the Nail Psoriasis Severity Index [NAPSI]), subungual hyperkeratosis, onycholysis, splinter hemorrhages, oil stains, and nail pitting were evaluated at baseline and at 3 and 6 months. RESULTS: A statistically significant improvement between baseline and 6 months was observed in all patients: the mean (SD) NAPSI went from 14.3 (6.3; 95% CI, 11.74-16.92) to 2.3 (1.21; 95% CI, 1.84-2.3) while the median went from 15 to 2.5 (P = .007). The percentage improvement at the end of treatment was 87.9%. No adverse effects were observed. CONCLUSION: Our study shows the therapeutic potential of tazarotene ointment in nail psoriasis.

Nail psoriasis: a retrospective study on the effectiveness of systemic treatments (classical and biological therapy).

BACKGROUND: Nail psoriasis represents a challenge for specialists. There is no comparative study of systemic treatment's effectiveness at this site. OBJECTIVE: Evaluate the response of nail psoriasis to classical and biological therapy and to compare the effectiveness and safety of the different treatments. METHODS: We performed a retrospective study of 84 patients with moderate-severe psoriasis seen at our Department between January 2006 and January 2009. RESULTS: Psoriasis was severe in 53.4%. In 75% of cases, the fingernails were affected, and the mixed form was the most frequently subtype. The mean baseline scores on the PASI and the NAPSI were 23.12 and 14.7 respectively; the correlation between the two scores fell at weeks 12 and 24 but had risen again at week 48. The baseline NAPSI score tended to be lower in women and significantly higher in patients over 65 years of age, family history of psoriasis, severe psoriasis and nail matrix involvement. In our series, 58.3% received classical treatment (acitretin, methotrexate, cyclosporin, PUVA, NUVB, REPUVA, RENUVB) and 41.7% received biological treatment (infliximab, efalizumab, etanercept, adalimumab).Significant reductions were found (P < 0.05) in the mean NAPSI scores at 12, 24 and 48 weeks with all the antipsoriatic agents except NUVB; significantly greater with cyclosporine (P < 0.01) and biological as infliximab and adalimumab at 12 and 24 weeks (differences between treatments disappeared at 48 weeks). CONCLUSION: The response to treatment is slower in the nail lesions than in the skin lesions. The improvement of nail psoriasis is significant both with the classical treatments significantly higher in cyclosporin; and biological treatment (infliximab and adalimumab at 12 and 24 weeks).

Incontinentia pigmenti and bipolar aphthosis: an unusual combination.

Incontinentia pigmenti (IP) is an uncommon X-linked dominant multisystem disorder, lethal in the majority of affected males in utero and variably expressed in females. The cutaneous manifestations are diagnostic and classically occur in four stages: vesicular, verrucous, hyperpigmented, and atrophic. The skin lesions are typically spread along the lines of Blaschko, and they are usually present at birth. It may be variably accompanied by dental, ocular, neurologic, bones and joints, and development anomalies. The genes IP has been mapped to Xq28. Mutations in the NEMO/IKKγ gene, located at Xq28, have been found to cause expression of the disease. Behçets disease is a multisystem disorder consisting of recurrent oral aphtae, genital ulcers, pustular skin eruption, and uveitis. Occasionally there are other articular, neurological, intestinal, or vascular abnormalities. This disease is rare in children. Here, we report a case of a 16-year-old female with the rare combination of incontinentia pigmenti and an aphthosis bipolar, and we discuss the probably relationship between these two diseases.

Serum prolactin levels in psoriasis and correlation with cutaneous disease activity.

BACKGROUND: Prolactin (PRL), a neuropeptide secreted by the anterior pituitary gland, possesses a variety of physiological actions. It has been implicated as an important immunomodulator and exerts a proliferative effect in cultured human keratinocytes via specific receptors. Some studies have indicated an increase in serum PRL levels in psoriasis and exacerbation of psoriasis when a prolactinoma is present. AIM: To evaluate the correlation between serum PRL levels and Psoriasis Area and Severity Index (PASI). METHODS: Serum PRL levels were measured in 20 patients (10 mean, 10 women, age range 18-88 years) with plaque-type psoriasis before and after a 6-week period of topical treatment with tacalcitol ointment. Results were compared with a group of 20 healthy volunteers. RESULTS: Serum PRL levels were significantly increased in the psoriatic group compared with the control group (P < 0.001) and were significantly reduced after treatment (P = 0.001). There was a correlation between pretreatment serum PRL levels and PASI (r = 0.33; P = 0.02). CONCLUSIONS: These results indicate that serum PRL levels may serve as a biological marker of psoriatic disease activity.

Eritema nodoso inducido por anillo vaginal / Vaginal ring inducing erythema nodosum

El eritema nodoso (EN) es la forma de paniculitis más común, y a su vez es considerado el prototipo de paniculitis septal. Este trastorno representauna reacción de hipersensibilidad frente a diferentes antígenos, así como múltiples enfermedades sistémicas y fármacos. Aunque en la mayoríade casos se desconoce la causa. Nuevos fármacos se añaden a la lista involucrados en la etiopatogenia del EN. Reportamos dos casos de EN en relacióncon el uso del anillo vaginal (AU)

Erythema nodosum (EN) represents the commonest form of nodular panniculitis and is the prototype of septal panniculitis. This disorder representsa hypersensitivity reaction in front of different antigens, such as several systemic diseases and drug therapies. In the majority of cases, the causeremains unknown. New drugs are being added to the list of drugs implicated in the etiopathogenesis of EN. We report two cases of EN in relationto the use of the vaginal ring (AU)

Sarna ampollosa con respuesta terapéutica a la ivermectina / Bullous scabies responding to ivermectin therapy

La sarna ampollosa es una enfermedad infrecuente que se suele diagnosticar en ancianos. Los hallazgos clínicos, histológicos einmunológicos son idénticos al penfigoide ampolloso. En una búsqueda bibliográfica hemos encontrado 24 casos descritos. Aportamos un nuevo caso de sarna ampollosa en un paciente de 72 años que respondió al tratamiento con ivermectina oral y revisamos la literatura médica (AU)

Bullous scabies is a rare disease that is usually diagnosed in elderly patients. The clinical, histological, and immunological findings are identical to bullous pemphigoid. In a review of the literature, we found reports of 24 cases. We present a new case of bullous scabies in a 72-year-old man. The lesions responded to treatment with oral iver mectin (AU)

[Bullous scabies responding to ivermectin therapy]. / Sarna ampollosa con respuesta terapéutica a la ivermectina.

Bullous scabies is a rare disease that is usually diagnosed in elderly patients. The clinical, histological, and immunological findings are identical to bullous pemphigoid. In a review of the literature, we found reports of 24 cases. We present a new case of bullous scabies in a 72-year-old man. The lesions responded to treatment with oral ivermectin.

Fístula con reacción granulomatosa a cuerpo extraño secundaria a electrodos residuales de marcapasos cardiaco retirado / Fistula With Foreign Body Granulomatous Reaction Caused by Electrodes Left in Place After Pacemaker Removal

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[An update on scalp psoriasis]. / Psoriasis del cuero cabelludo.

Between 50 % and 80 % of patients with psoriasis have involvement of the scalp. The clinical presentation of scalp psoriasis can be very varied, with disease severity ranging from mild to extremely severe.The disease may have a major psychological impact. Treatment should be tailored to each individual in order to achieve a good clinical response or blanching that lasts for as long as possible, with a safe and convenient regimen. Many different treatments have been tried: phototherapy, pulsed magnetic fields, Grenz rays, keratolytics, coal tar, antifungals, dithranol, retinoids, vitamin D analogues, corticosteroids, and systemic treatment. Ideally, for scalp psoriasis, treatment should be effective; applied in the form of a lotion, foam, or emulsion; require few applications per week; and have proven long-term safety. One such treatment is potent corticosteroids and vitamin D3 analogues in combination, which has a fast onset of action and proven long-term safety.

Psoriasis del cuero cabelludo / An Update on Scalp Psoriasis

La psoriasis del cuero cabelludo afecta al 50-80 % de los pacientes psoriásicos. Su presentación clínica puede ser muy variable: desde una enfermedad leve hasta formas muy graves. Esta condición puede provocar un gran impacto psicológico. El principal objetivo del tratamiento es individualizar el mismo en cada paciente, conseguir una buena respuesta clínica o el blanqueo mediante un tratamiento seguro y cómodo, con una respuesta lo más duradera posible. Se han usado múltiples tratamientos: fototerapia, campos magnéticos pulsátiles, rayos Grenz, queratolíticos, alquitrán de hulla, antifúngicos, ditranol, retinoides, análogos de la vitamina D, corticoesteroides y tratamiento sistémico. El perfil farmacológico ideal para tratar la psoriasis del cuero cabelludo es aquel que sea efectivo, de aplicación intermitente pocas veces por semana en forma de loción, espuma o emulsión y seguro a largo plazo, como la combinación de corticoesteroides potentes y análogos de la vitamina D3, con efecto rápido inicial y seguridad a largo plazo demostrada respectivamente (AU)

Between 50 % and 80 % of patients with psoriasis have involvement of the scalp. The clinical presentation of scalp psoriasis can be very varied, with disease severity ranging from mild to extremely severe. The disease may have a major psychological impact. Treatment should be tailored to each individual in order to achieve a good clinical response or blanching that lasts for as long as possible, with a safe and convenient regimen. Many different treatments have been tried: phototherapy, pulsed magnetic fields, Grenz rays,keratolytics, coal tar, antifungals, dithranol, retinoids, vitamin D analogues, corticosteroids, and systemic treatment. Ideally, for scalp psoriasis, treatment should be effective; applied in the form of a lotion, foam, or emulsion; require few applications per week; and have proven long-term safety. One such treatment is potent corticosteroids and vitamin D3 analogues in combination, which has a fast onset of action and proven long-term safety (AU)