URTICARIA VASCULITIS

Urticaria Vasculitis
Entidad clnico patolgica caracterizada por: Lesiones que simulan urticaria, pero en su evolucin son ms prolongadas Dejan hiperpigmentacin residual Pueden o no presentar prurito Relacin 2:1 ms en mujeres

Urticaria Vasculitis
Manifestacin de una enfermedad sistmica.

Proceso no asociado a enfermedad subyacente.

Urticaria Vasculitis
CLNICA TRADA CARACTERSTICA Erupcin de ronchas eritematosas que simulan urticaria pero histolgicamente muestran vasculitis leucocitoclsica Duran ms de 24 hs Lesiones fijas

Urticaria Vasculitis
CLNICA Erupcin compuesta por la asociacin de ppulas y placas de aspecto urticariano acompaada de sensacin de dolor o quemazn. Petequias ocasionales. Hiperpigmentacin post inflamatoria Lesiones fijas, de ms de 24 hs.

Urticaria Vasculitis
Sntomas sistmicos asociados: Artralgias Angioedema Fiebre Dolor abdominal y/o torcico Enf. Pulmonar Enf. Renal Epiescleritis-Uvetis

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Reaccin de hipersensibiidad tipo III: Complejos Ag Ac son depositados en la luz vascular. Activacin del complemento y quimiotaxis de los neutrfilos con liberacin de enzimas que causan dao tisular.

Urticaria Vasculitis
Formacin de complejos inmunes que se depositan en las paredes de los vasos. 30 a 75 % de pacientes con U V El antgeno parece ser una regin colgeno-like sobre C1q. El anticucuerpo es referido como C1q precipitina

Urticaria Vasculitis
CAUSAS: Idioptica. Inducida por drogas: Inh. de ECA, penicilinas, sulfonmidas, fluoxetina, tiazidas. Enf. Reumticas: LES, Sme. De Sjgren, gamapata monoclonal Ig A, Ig M. Crioglobulinemias Neoplasias: Hematolgicas o slidas. Infecciones: Hepatitis B, C, Mononucleosis infecciosa.

Urticaria Vasculitis
Sndrome de Urticaria Vasculitis Hipocomplementmica Urticaria Vasculitis Hipocomplementmica Urticaria Vasculitis Normocomplementmica

Urticaria Vasculitis
UV HIPOCOMPLEMENTMICA: Asociada a sntomas sistmicos Enf. del tejido conectivo (LES) Angioedema Enfermedad Pulmonar Obstructiva Crnica

URTICARIA VASCULITIS NORMOCOMPLEMENT NORMOCOMPLEMENTMICA

URTICARIA VASCULITIS HIPOCOMPLEMENT HIPOCOMPLEMENTMICA + Frecuencia de lesiones purp purpricas y secuelas pigmentarias Artralgias: 75 % Glomerulonefritis: 13 % Afectaci Afectacin ocular: 21 % Afectaci Afectacin pulmonar obstructiva o asma: 17 % Dolores abdominales: 35 % Infiltrado dermico intersticial de polimorfonucleares Dep Depsitos granulares de Ig M, Ig G, o Ig A a lo largo de la membrana basal: 96 % VSG Aumentada: 50 % Incierto. Posibilidad de LES Corticoides y/o antihistam antihistamnicos, AINES, inmunosupresores

SIGNOS CUTNEOS

Artralgias: 25 % Glomerulonefritis: 0 % Afectaci Afectacin ocular: 0 % Afectaci Afectacin pulmonar obstructiva o asma: 5 % Dolores abdominales: 15 %

SIGNOS EXTRACUT EXTRACUTNEOS

HISTOLOG HISTOLOGA INMUNOFLUORESCENCIA CUT CUTNEA LABORATORIO PRON PRONSTICO TRATAMIENTO

Dep Depsitos granulares de Ig M, Ig G, o Ig A a lo largo de la membrana basal: 1 % VSG Aumentada: 25 % Bueno Antihistam Antihistamnicos, AINES, Colchicina, Corticoides

Urticaria Vasculitis
Laboratorio CH 50, C3, C4, C1q y Ac anti C1q Funcin renal y orina completa Ac Antinucleares y serologa para LES Inmunofluorescencia Ac Anticitoplasma de neutrofilos

Urticaria Vasculitis
Rx Trax: En pacientes con sntomas pulmonares Biopsia: Lesin de menos de 48 hs. Lesin de 24 hs, mejor para inmunofluorescencia

Urticaria Vasculitis
Anatoma Patolgica: VASCULITIS LEUCOCITOCLSTICA: Dao en pequeos vasos de dermis papilar y reticular

Urticaria Vasculitis
Lesin temprana: Infiltrado neutroflico perivascular, vnulas postcapilares. Leucocitoclasia. Presencia de eosinfilos. Depsitos fibrinoides en la pared y endotelio intacto. Extravasacin de glbulos rojos

Urticaria Vasculitis
Lesin tarda: Infiltrado mixto, linfocitos y neutrfilos Inmunofluorescencia: Depsitos de fibrina y complemento Ig M, Ig G e Ig A a lo largo de la membrana basal

Urticaria Vasculitis
Curso crnico Formas hipocomplementmica el pronstico lo marca la afectacin pulmonar y la renal

Urticaria Vasculitis
TRATAMIENTO: Se basa en le afectacin sistmica de base Afectacin slo cutnea: Antihistamnicos y AINES Otras drogas utilizadas son: Colchicina, Hidroxicloroquina o Dapsona. Afectacin sistmica: Corticoides Azatioprina, Micofenolatomofetil, Rituximab.

HIDROXICINA

Drug Name

Description

Hydroxyzine (Atarax, Vistaril) Antagonizes H1 receptors in periphery. May suppress histamine activity in subcortical region of CNS. Can be used for symptomatic control. The recommended antihistamine for pregnant patients is diphenhydramine. Has been used safely in children. 0.5 mg/kg PO q6h or 25-100 mg PO qd/qid; not to exceed 50 mg PO q6h 0.5-0.6 mg/kg/dose PO q6h Documented hypersensitivity CNS depression may increase with alcohol or other CNS depressants

Adult Dose Pediatric Dose Contraindications Interactions

Antagonista de R H1 perifricos

DIFENHIDRAMINA

Drug Name Description Adult Dose Pediatric Dose Contraindications Interactions

Diphenhydramine (Benadryl, Benylin, Diphen, AllerMax) For symptomatic relief of symptoms caused by release of histamine in hypersensitivity reactions. In pregnancy, use 25-50 mg PO q6h prn. 25-50 mg PO q6-8h prn; 10-50 mg IV/IM q6-8h prn; not to exceed 400 mg/d 5 mg/kg/d PO/IV/IM divided q6-8h; not to exceed 300 mg/d Documented hypersensitivity, MAOIs Potentiates effect of CNS depressants; because of alcohol content, do not administer syrup form to patient taking medications that can cause disulfiramlike reactions

Antagonista de R H1 perifricos

COLCHICINA

Description

Alkaloid extract that inhibits microtubule formation. Often used for treatment of acute gout. Has been reported effective for urticarial vasculitis. Concentrates well in leukocytes and reduces neutrophilic chemotaxis and motility. Histologically, urticarial vasculitis presents with neutrophil involvement; therefore, colchicine possibly is useful. However, drug's effect has not been proven in clinical trials. 0.6 mg PO bid/tid Children: Not established Adolescents: 0.5 mg/kg PO divided bid/tid Documented hypersensitivity; severe renal, hepatic, GI, or cardiac disorders; blood dyscrasias

Adult Dose Pediatric Dose Contraindications

Modula el sistema inmune y reducen la inflamacin Disminuye la quimiotaxis de neutrfilos

SULFONAS

Drug Name

Description

Adult Dose Pediatric Dose

Dapsone (Avlosulfon) Preferred sulfone. Other sulfones must be metabolized to dapsone for their effect. Mechanism of action is similar to that of sulfonamides in which competitive antagonists of PABA prevent formation of folic acid, inhibiting bacterial growth. Dosing guidelines for dermatologic use have been well described in dermatitis herpetiformis. Most case reports about effect in urticarial vasculitis use dermatitis herpetiformis dosing guidelines. Has been used extensively in chronic bullous disease of childhood. 50 mg/d PO initial; can be increased by 50 mg/wk to 300 mg/d 1-2 mg/kg/d PO Absolute: Documented hypersensitivity Relative: G-6-PD deficiency (especially in African Americans, persons of Middle Eastern heritage, and Asians); significant cardiopulmonary disease; significant hematologic disease; sulfa allergy (cautious use in patients with sulfa allergy may be attempted; crossreactivity is relatively rare and mild)

Contraindications

Modula el sistema inmune y reducen la inflamacin Inhibe la formacin de radicales libres por neutrfilos

ANTIMALRICOS

Drug Name

Hydroxychloroquine (Plaquenil) Preferred antimalarial agent because of its low toxicity and high effectiveness profile. Usually well tolerated if carefully monitored by prescribing physician. Therapy is required for 4-8 weeks before evaluating effectiveness. 6.5 mg/kg PO or 400 mg/d PO, whichever is less 3-5 mg/kg/d PO divided bid; not to exceed 400 mg/d Absolute: Documented hypersensitivity; retinopathy from any cause Relative: Pregnancy or breastfeeding; retinal or visualfield changes; severe blood dyscrasias; psoriasis; G-6-PD deficiency (caution advocated, but routine G-6-PD screening not recommended; associated with hemolysis, but not in usual dosage range); significant hepatic dysfunction; myasthenia gravis, significant neurologic disease; long-term therapy in children (listed in Physicians Desk Reference as contraindication for hydroxychloroquine; main concern is overdose/toxicity; chronic toxicity risk, however, thought to be no greater than in adults); neither drug available as a syr; crush tab and mask bitter taste in jam, applesauce, or other soft food

Description

Adult Dose Pediatric Dose

Contraindications

Reducen la inflamacin Inhibe la quimiotaxis de neutrfilos

ANTI INFLAMATORIOS NO ESTEROIDES

Drug Name Description Indomethacin (Indocin) Only NSAID reported effective in urticarial vasculitis. Rapidly absorbed; metabolism occurs in liver by demethylation, deacetylation, and glucuronide conjugation; inhibits prostaglandin synthesis. 100-150 mg/d PO in divided doses 1-2 mg/kg/d PO in 2-4 divided doses; not to exceed 150200 mg/d Documented hypersensitivity; avoid in GI bleeding or renal insufficiency

Adult Dose Pediatric Dose Contraindications

Reducen la inflamacin Inhibe la sntesis de prostaglandinas

AGENTES CITOTXICOS
Drug Name Azathioprine (Imuran) Purine precursor that affects formation of adenine and guanine. Results in impaired DNA synthesis in immunocompetent cells such as lymphocytes, which are dividing rapidly during inflammatory process. Has slow onset of action; rarely used as monotherapy. 1 mg/kg/d qd/bid (empiric) or based on TPMT level (see Precautions); increase dose by 0.5 mg/kg/d after 6-8 wk if necessary; increase q4wk; 2 mg/kg/d maximum dose for most dermatologic purposes Not established.

Description

Adult Dose Pediatric Dose

Interfiere en sntesis de DNA de cl inmunocompetentes Ahorrador de corticoides

GLUCOCORTICOIDES
Drug Name Prednisone (Deltasone) Although is most effective, adverse effect profiles preclude it from use as a first-line agent. Consider only after failure of antihistamines, indomethacin, colchicine, dapsone, or hydroxychloroquine. Effect on urticarial vasculitis likely is mediated by its anti-inflammatory effect. This class of medications decreases capillary permeability and inhibits the mitotic rate of lymphocytes. 0.5-1.5 mg/kg/d PO initial; taper as disease responds; if chronic use required, qod administration is safer 0.5-2 mg/kg/d PO in divided dose bid to qid Documented hypersensitivity; viral infection, peptic ulcer disease, hepatic dysfunction, connective-tissue infections, and fungal or tubercular skin infections; GI disease

Description

Adult Dose Pediatric Dose Contraindications

Efecto anti inflamatorio

Urticaria Vasculitis
Pronstico Es una patologa de curso crnico, por lo tanto es importante la educacin del paciente. Una minora de pacientes presentan afectacin sistmica, y requieren un abordaje multidisciplinario.

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