PIEL-L LATINOAMERICA

Lista de discusión dermatológica

 

Edición Nº 27

Viernes 01/10/2004

 

"No puede impedirse el viento. Pero pueden construirse molinos".
Proverbio holandés

 

 

Contenido: 1) Caso clínico nuevo; comentarios evolutivo. 2) Dificultades terapéuticas  3) Artículos científicos de interés  4) Generalidades  5)Anexos

 

1) Caso Clínico Presentado por el Dr. Rolando Hernández Pérez

Clínica Ntra. Señora del Pilar. Barinas. Venezuela

 

Se trata de un paciente masculino de 28 años de edad, peón de finca agropecuaria, natural y procedente de Socopo-Barinas (sur-oeste del estado Barinas) quien refiere patología dermatológica en  áreas expuestas al sol con más de 12 años de evolución aproximadamente. Al examen dermatológico placas eritemato descamativas, infiltradas, coriácea, escoriadas con costras hemáticas, moderadamente pruriginosas y con zonas  exulceradas, pruriginosas, localizadas en piel de cara, labios, zona posterior del cuello (nuca), pabellones auriculares, antebrazos, manos, en manos y antebrazo se observa pápulas duras, infiltradas, discretamente descamativa y más pruriginosas. (Favor ver foto clínica anexa DSN1795-1794). No refiere antecedentes personales relacionados con la enfermedad actual. No hay antecedentes familiares contributorios: No refiere la ingesta de medicación.

Laboratorio convencional dentro de la normalidad; RX de tórax dentro de la normalidad; Laboratorio inmunológico (AAN, ant-ADN, anti-SM, C3-C4, CH50) dentro de los valores normales.El paciente refiere empeoramiento del las lesiones al exponerse al sol.

Histopatológicamente: hiperqueratosis ortoqueratósica, paraqueratosis, acantosis irregular, espongiosis, infiltrado linfocitario, histocitario y plamocitos en dermis papilar y reticular, vasos sanguínios dilatados, edema importante de dermis.(DSCN2270)

En base a estos hallazgos se plantea tratamiento con a) Fotoprotector (Helioblock gel spf 4  tid y protector labial qid; fuorato de mometasona 1 aplicacióna al día por 12 días, luego interdiario por 12 días más, luego solo Fotoprotector solar y crema emoliente.(Favor ver resultados, DSCN2146-2145), excelente resulatdo

Comentarios: nosotros pensamos que se trata de un Eccema Fortosensible, el cual es un eccema crónico y fotosensibilidad, sin quue existan antecedente de fotoalergia y en el la dosis mínima de eritema es normal o sea sería una fotosensibilidad idiopática.Consideran ustedes que el caso esta bien manejado, ¿alguna sugerencia?, Gracias.

 

Para agregar un comentario a este caso haga click aqui

 

 


2) Dificultades Terapéuticas:

 

     Paciente de 15 años con furunculosis y foliculitis recalcitante en muslo.

2-1 El caso que quiero plantearle en esta ocasión es un joven de 15 años de edad en tratamiento con Isotretinoína oral (Roaccutan) durante meses que ha recibido hasta el presente aproximadamente 5200 Mg., y al cual pretendo llegar a su dosis máxima, el está muy bien de las lesiones que presentaba en cara y tronco, sin embargo desde hace aproximadamente 3 meses ha venido presentando furunculosis y foliculitis en caras internas de muslos.  Resistente a tratamiento antibiótico con Ciproxina (según cultivo y antibiograma).  La verdad no sé que hacer.... María Irina Daza, Barquisimeto Venezuela

 

Respuestas:

 

Omite el Roaccutan,  usaría una cefalosporina y si el cultivo dice que es resistente a las cefalosporinas, usaría dapsona, previo exámenes de laboratorio y solicitud de glucosa 6-fosfato deshidrogenasa. Localmente mupirocin y lubricación de la piel

Saludos

Jaime Piquero Martín

Caracas- Venezuela


   

 Paciente con Pénfigo Vulgar.

 2-2 Se trata de un paciente masculino de 37 años de edad, procedente de Maturín-Edo. Monagas, con diagnostico clínico e histopatológico de Pénfigo Vulgar (evolución de 6 meses aproximadamente) localizadas en boca, cara, cuero cabelludo, tronco y miembros superiores. Se inició tratamiento con Prednisona (Meticorten) 70 mg/día. Metotrexato 10 mg/semanal vo (dosis acumulada 50 mg) ; buenos resultados, pero al mes hay un estancamiento de la respuesta y persiste lesiones tanto bucales como cutáneas. Se decide aumentar la dosis de la Prednisona a 100 mg/interdiario y se infiltra las lesiones de la cara y boca con acetónido de triacinolona  (1 infiltración), continuamos con metotrexato; además se indica dieta hiposódica (interconsulta con nutrición), Rocaltrol (alfa D 3  1 mcg/día) Calcibon tid, ejercicio tipo aeróbico, y soporte psiquiátrico. RHP

Pregunto: a) considerar ustedes que esta siendo bien manejado?

           &nb sp;   b) sugieren algo más? 


2-3 Paciente con Afta mayor y leucopenia

 Paciente de 42 años de edad con cuadro clínico e histopatológico de Afta mayor recidivante y recalcitarnte en boca, con más de 10 años de evolución (estudio histopatológico reciente) resto del examen físico dentro de la normalidad, laboratorio convencional (LEUCOPENIA (3000-3800 mantenida desde enero 2004) , RX tórax normal. Se solicitó interconsulta con Hematología. RHP

 


2-4 Paciente lactante menor con Ictiosis Vulgar en tratamiento con acitretino (Neotigason oral suspensión)

Paciente lactante de 7 meses de edad (peso 10 mg) con cuadro clínico e histopatológico de Ictiosis Vulgar en tratamiento con Neotigason (2 mg/kg/peso)

        Recibe 20 mg desde hace 21 días, excelentes resultados, laboratorio convencional dentro de la normalidad; tratamiento tópico con urea y emolientes. RHP

      Pregunto: ¿hasta cuando debemos y podemos dar el acitretino?

           &nb sp;         ¿cual es la experiencia del grupo de dermatología pediátrica?

 


 

3) Artículos Científicos de interés

 

International Journal of Dermatology
Volume 43 Issue 9 Page 632  - September 2004
doi:10.1111/j.1365-4632.2004.01939.x

 

 

Report

Cutaneous manifestations associated with antiphospholipid antibodies

Maria José Nogueira Diógenes, MD, PhD, Pedro Coelho N. Diógenes, Raquel Maia de Morais Carneiro, MD, Carlos C. Ribeiro Neto, MD, Fernando B. Duarte, MD, and Rosangela R. A. Holanda, MD

Of a total of 60 patients, 47 were female and 13 were male, with a ratio of 3.6 : 1. Previous reports described a ratio of 2 : 1 in patients with PAAS, 47 and 4 : 1-5 : 1 in patients with a positive lupus anticoagulant test. 48,49 In this study, patients with primary and secondary AAS, as well as patients with PPAA, were included; all contributing to the female preponderance.

The mean age for all patients with antiphospholipid antibodies (n = 60) was 39.9 years old. For patients with AAS (n = 39) the mean age was 45.7 years, and for those with PPAA was 29.19 years (range, 15-46 years). A classical study of primary AAS reported a mean age of 38.5 years (varying from 21 to 59 years). 34 Cases in children have been described. 49,50 In our study, the patients with PPAA were slightly younger than those with an established syndrome. This may suggest that this group of patients may develop thrombotic events at older ages and should be closely followed.

All patients had positive aCL (IgG and IgM). All patients had a negative partial thromboplastin time. Fourteen patients had positive LA (KCT). Twenty patients had positive LA (dRVVT). Low levels of protein S were evidenced in two out of seven cases measured. The APTT has low sensitivity for low LA activity, missing as much as 50% of patients in one study with SLE patients and LA activity with other tests. 26 Previous data on laboratory findings in this syndrome have addressed the problem that not all patients have both LA and aCL antibodies detected, 7,47,51 with agreement of tests in only 50-75% of cases of AAS. 7,47,51 Some authors have stated that aCL antibody tests are more sensitive and have less observer error. 3,13 Almost all patients with LA have detectable aCL antibodies (90%) and almost all patients with AAS have detectable aCL antibodies. 3 Low protein C and protein S levels are described in patients with AAS and are associated with an additional risk of thrombotic events. 2832

Sixteen (40%) patients out of 39 had a dermatological manifestation related to the syndrome as the main complaint. A previous study also developed in a dermatology department described cutaneous lesions as a primary sign of AAS in 41% of cases. More serious systemic thrombosis developed in 40% of those patients. 7,46 Another study 26 showed cutaneous manifestations in 70% of catastrophic PAAS cases.

All systemic manifestations found in our group of patients have been previously described. 721 The most frequent dermatological findings were dermographism, chronic urticaria, acrocyanosis, livedo reticularis and alopecia. Dermographism was diagnosed in patients of all three groups (40%). This manifestation has not been described previously in association with the syndrome; nevertheless, as a result of its highly frequent presence among patients with antiphospholipid antibodies it will be the subject of future studies. Chronic urticaria (26% of all patients), also not described previously in association with the syndrome, was also a common manifestation. All patients with chronic urticaria were investigated for possible common causes. No cause for chronic urticaria was found in the primary AAS patients and PPAA patients, although no assay for FcRI autoantibodies was performed. Chronic urticaria is now ascribed to autoimmunity in approximately 50% of cases, with anti FcRI antibodies playing a major role. 52 Autoimmune conditions were associated more frequently with patients with chronic urticaria having functional autoantibodies than in those without. 52 As chronic urticaria is frequently autoimmune in etiology, as well as being associated with autoimmune conditions, its finding in PAAS and its occurrence in the PPAA patients without the syndrome will be a target of future studies to prove the possible association of chronic urticaria with antiphospholipid antibodies. The three cases of urticaria described in secondary antiphospholipid syndrome could ! be relat ed to the underlying disorder in these patients. Other cutaneous manifestations found in this study, such as acrocyanosis (31% of all), diffuse alopecia (18%), livedo reticularis (17%), ulcers and necrosis, purpura, nodules, Raynaud's phenomenon, pterygium ungueum and subungual hemorrhage, have already been described in patients with AAS. 7,8,12,13,2126,33 A previous report 53 described livedo reticularis as the most common dermatological manifestation (55%) in PAAS patients. In another study, 7 thrombophlebitis, presenting as an edema and erythema of the ankle and lower leg, was the most common (34%) finding among 70 patients with LA activity.

In seven (50%) of 14 secondary cases, the syndrome was associated with SLE. Similar results were described by Derksen et al. who found SLE in 49% of secondary cases. 36 Previous reports 12,13,47,51,54 have described secondary cases to be associated with the other clinical conditions diagnosed in this study such as Sjögren syndrome and rheumatoid arthritis, though thrombotic events are rare in these cases.

Histological findings of skin ulcers and necrotic areas were not specific. Three out of four skin nodules studied revealed thrombus on dermal microvessels. Biopsies from other sites revealed features of associated conditions. According to the literature, almost all findings of vascular occlusion in AAS are of thrombotic and not inflammatory nature. 2,3,13,15,54 Capillaries may coexist, as perivascular inflammatory reaction is usually a consequence and not a cause of the vessel's thrombotic event. 15

Patients with persistent positive antiphospholipid antibodies, but no systemic manifestation related to the syndrome, were included in this study because it has been shown that they are at higher risk of future thrombosis. 54 Thirty to 50% of patients with positive LA later develop thrombosis. 54 Only a small percentage of patients with antiphospholipid antibodies, especially aCL, develop thrombosis, miscarriages or thrombocytopenia, 3 although high titers of aCL IgG have been linked with an increased risk of future thrombosis. 28 As only a minority of patients develop thrombosis, they should not be treated initially, but should be carefully followed up. 3 In one case of this study, the patient presented with a nonthrombotic skin nodule and later developed the primary syndrome, justifying the careful follow up of patients with persistent antiphospholipid antibodies and no documented evidence of thrombosis.

Dermatological complaints are frequent in patients with AAS and may be the first clue to the syndrome. So, every dermatologist should investigate the possibility of AAS, not only when facing dermatological findings related to the development of thrombus and microthrombus, but also when observing acrocyanosis and livedo reticularis.


 

4) Generalidades

 

FLOR DE PIEL

 

Esta piel de mis poros

y mis alegrías

esta piel de mis pecas

y mis pecados

de mis lunares

y cicatrices

de mis erizos

y picazones

esta piel de mis venas

y tus caricias

de hora en hora

se vuelve arrugas

con plan

con método

sin retroceso

 

Dentro de quince

de veinte años

dentro de veinte

treinta minutos

será un hollejo

será una pasa

un viejo odre

sin vino nuevo.

 

"Mario Benedetti"

Inventario (La casa y el Ladrillo)

 


 

5) Anexos

 

5.1 Anexo 1: DSCN1794.jpg

Si no ve la foto DSCN1794.jpg click aquí

 

5.2 Anexo 2: DSCN1795.jpg

 

Si no ve la foto DSCN1795.jpg clic aqui

 

5.3 Anexo 3: DSCN2145.jpg

 

Si no ve la foto DSCN2145.jpg clic aqui

 

5.4 Anexo 4: DSCN2146.jpg

 

Si no ve la foto DSCN2146.jpg clic aqui

 

5.5 Anexo 5: DSCN2270.jpg

 

Si no ve la foto DSCN2270.jpg clic aqui

 


 

 

Saludos y hasta nuestra póxima edición el martes 05/10/2004

Los editores.

 

 

 

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