PIEL-L LATINOAMERICA

Lista de discusi�n dermatol�gica

 

Edici�n N� 27

Viernes 01/10/2004

 

"No puede impedirse el viento. Pero pueden construirse molinos".
Proverbio holand�s

 

 

Contenido: 1) Caso cl�nico nuevo; comentarios evolutivo. 2) Dificultades terap�uticas  3) Art�culos cient�ficos de inter�s  4) Generalidades  5)Anexos

 

1) Caso Cl�nico Presentado por el Dr. Rolando Hern�ndez P�rez

Cl�nica Ntra. Se�ora del Pilar. Barinas. Venezuela

 

Se trata de un paciente masculino de 28 a�os de edad, pe�n de finca agropecuaria, natural y procedente de Socopo-Barinas (sur-oeste del estado Barinas) quien refiere patolog�a dermatol�gica en  �reas expuestas al sol con m�s de 12 a�os de evoluci�n aproximadamente. Al examen dermatol�gico placas eritemato descamativas, infiltradas, cori�cea, escoriadas con costras hem�ticas, 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 p�pulas duras, infiltradas, discretamente descamativa y m�s pruriginosas. (Favor ver foto cl�nica anexa DSN1795-1794). No refiere antecedentes personales relacionados con la enfermedad actual. No hay antecedentes familiares contributorios: No refiere la ingesta de medicaci�n.

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

Histopatol�gicamente: hiperqueratosis ortoquerat�sica, paraqueratosis, acantosis irregular, espongiosis, infiltrado linfocitario, histocitario y plamocitos en dermis papilar y reticular, vasos sangu�nios 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 aplicaci�na al d�a por 12 d�as, luego interdiario por 12 d�as m�s, 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 cr�nico y fotosensibilidad, sin quue existan antecedente de fotoalergia y en el la dosis m�nima de eritema es normal o sea ser�a una fotosensibilidad idiop�tica.Consideran ustedes que el caso esta bien manejado, �alguna sugerencia?, Gracias.

 

Para agregar un comentario a este caso haga click aqui

 

 


2) Dificultades Terap�uticas:

 

     Paciente de 15 a�os con furunculosis y foliculitis recalcitante en muslo.

2-1 El caso que quiero plantearle en esta ocasi�n es un joven de 15 a�os de edad en tratamiento con Isotretino�na oral (Roaccutan) durante meses que ha recibido hasta el presente aproximadamente 5200 Mg., y al cual pretendo llegar a su dosis m�xima, el est� 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 antibi�tico con Ciproxina (seg�n cultivo y antibiograma).  La verdad no s� que hacer.... Mar�a Irina Daza, Barquisimeto Venezuela

 

Respuestas:

 

Omite el Roaccutan,  usar�a una cefalosporina y si el cultivo dice que es resistente a las cefalosporinas, usar�a dapsona, previo ex�menes de laboratorio y solicitud de glucosa 6-fosfato deshidrogenasa. Localmente mupirocin y lubricaci�n de la piel

Saludos

Jaime Piquero Mart�n

Caracas- Venezuela


   

 Paciente con P�nfigo Vulgar.

 2-2 Se trata de un paciente masculino de 37 a�os de edad, procedente de Matur�n-Edo. Monagas, con diagnostico cl�nico e histopatol�gico de P�nfigo Vulgar (evoluci�n de 6 meses aproximadamente) localizadas en boca, cara, cuero cabelludo, tronco y miembros superiores. Se inici� tratamiento con Prednisona (Meticorten) 70 mg/d�a. 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 cut�neas. Se decide aumentar la dosis de la Prednisona a 100 mg/interdiario y se infiltra las lesiones de la cara y boca con acet�nido de triacinolona  (1 infiltraci�n), continuamos con metotrexato; adem�s se indica dieta hipos�dica (interconsulta con nutrici�n), Rocaltrol (alfa D 3  1 mcg/d�a) Calcibon tid, ejercicio tipo aer�bico, y soporte psiqui�trico. RHP

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

           &nb sp;   b) sugieren algo m�s? 


2-3 Paciente con Afta mayor y leucopenia

 Paciente de 42 a�os de edad con cuadro cl�nico e histopatol�gico de Afta mayor recidivante y recalcitarnte en boca, con m�s de 10 a�os de evoluci�n (estudio histopatol�gico reciente) resto del examen f�sico dentro de la normalidad, laboratorio convencional (LEUCOPENIA (3000-3800 mantenida desde enero 2004) , RX t�rax normal. Se solicit� interconsulta con Hematolog�a. RHP

 


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

Paciente lactante de 7 meses de edad (peso 10 mg) con cuadro cl�nico e histopatol�gico de Ictiosis Vulgar en tratamiento con Neotigason (2 mg/kg/peso)

        Recibe 20 mg desde hace 21 d�as, excelentes resultados, laboratorio convencional dentro de la normalidad; tratamiento t�pico con urea y emolientes. RHP

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

           &nb sp;         �cual es la experiencia del grupo de dermatolog�a pedi�trica?

 


 

3) Art�culos Cient�ficos de inter�s

 

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 Jos� Nogueira Di�genes, MD, PhD, Pedro Coelho N. Di�genes, 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 Sj�gren 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 alegr�as

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 m�todo

sin retroceso

 

Dentro de quince

de veinte a�os

dentro de veinte

treinta minutos

ser� un hollejo

ser� 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 aqu�

 

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 p�xima edici�n el martes 05/10/2004

Los editores.

 

 

 

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