Púrpura de Schönlein-Henoch Buloso. Caso clínico / Bullous Henoch-Schönlein purpura. Case report

Resumen: Introducción: El púrpura de Schonlein-Henoch (PSH) O Vasculitis IgA es la vasculitis sistémica más frecuente de la edad pediátrica. Se manifiesta clínicamente como púrpura palpable, artralgias, dolor abdominal y compromiso renal. El púrpura palpable buloso a diferencia de lo que ocurre en la edad adulta, es muy infrecuente en la infancia. Objetivo: Reportar una forma infrecuente de presentación cutánea del PSH en niños. Caso clínico: Niña de 14 años con historia de 2 semanas con ampollas dolorosas múltiples y confluentes en ambas extremidades inferiores asociado a artralgias. A la histo-patología destacan vesículas intracórneas, epidermis con acantosis y espongiosis e infiltrado dérmico perivascular. Inmunofluorescencia directa (+) para IgA. Se plantea el diagnóstico de PSH ampollar y se realiza tratamiento inicial con corticoides intravenosos. A los tres días del traslape a corticoides orales aparecen nuevas lesiones equimóticas en ambas piernas. Se decide asociar azatriopina e iniciar descenso de corticoides, obteniéndose buena respuesta. Conclusión: Si bien la formación de bulas en el PSH no agrega morbilidad, suele ser un fenómeno alarmante que requiere realizar diagnóstico di ferencial con otras patologías. El uso de corticoides estaría indicado en estos casos ya que disminuiría la producción de las metaloproteinsas responsables de la formación de las bulas.

Abstract: Henoch-Schönlein purpura (HSP) or IgA Vasculitis is the most common childhood vasculitis. The classic tetrad of signs and symptoms include palpable purpura, arthralgia, abdominal pain and renal disease. The occurrence of hemorrhagic bullae in children with HSP is rarely encountered. Objec tive: To report an unusual cutaneous manifestation of HSP in children. Case report: A 14-year-old girl complained about a 2-week painful bullous rash in both lower extremities and multiple arthral gias. There was no history of abdominal pain or urinary symptoms. In both lower extremities, there were numerous palpable purpura and hemmorrhagic bullae. In light of clinical findings, laboratory tests and skin biopsy are requested. The histopathology described intraepidermal blisters, acanthosis, spongiosis and perivascular dermal infiltrate. Direct immunofluorescence (IFD) (+) for IgA. The diagnosis of bullous HSP was made and treatment with endovenous corticosteroids was initiated. Three days after overlapping to oral corticosteroids, new ecchymotic lesions appeared in both legs. Due to the persistence of cutaneous involvement and negative control tests, azathioprine was associa ted obtaining a good response. Conclusion: Although bullous lesions in HSP does not add morbidity, it is often an alarming phenomenon with multiple differential diagnoses. The anti-inflamatory effect of corticoids is likely to be beneficial in the treatment of patients with severe cutaneous involvement through inhibition of proinflammatory transcription factors and decreasing the production of the metalloproteinases.

Presentación atípica de púrpura de Schönlein-Henoch, a raíz de un caso / Atypical presentation of Henoch-Schönlein purpura. Regarding a clinical case

El Púrpura de Schonlein Henoch (PSH) corresponde a la vasculitis sistémica más común en la edad pediátrica, presentándose el 90% de los casos antes de los 10 años. Gran parte de los casos son precedidos por una infección respiratoria alta, sugiriendo un potencial gatillante infeccioso, sin embargo la causa subyacente aún se desconoce. El depósito de complejos inmunes IgA en distintas localizaciones lleva a las manifestaciones clínicas órgano especificas: púrpura palpable, artralgias, daño renal, dolor abdominal y otras formas de compromiso gastrointestinal, entre otras. La presencia de lesiones cutáneas constituye uno de los pilares diagnósticos de esta patología y la ausencia de éstas orienta el estudio a otros diagnósticos diferenciales. En cuanto al tratamiento, la mayoría de los casos cursan con buena evolución y resolución espontánea de los síntomas, reservándose el uso de glucocorticoides sistémicos para casos severos, obedeciendo al criterio del médico tratante al no existir guías de manejo específicas. Acontinuación se presentará el caso de una niña de 3 años con diagnóstico de PSH cuya manifestación inicial fue el compromiso gastrointestinal, presentando dolor abdominal y hemorragia digestiva baja previa a la aparición del rash cutáneo.

Henoch Schönlein purpura is a systemic vasculitis most commonly found in children, with 90% of cases detected in the under 10´s. Many cases are preceded by an upper respiratory tract infection which suggests a potential infectious trigger, however the underlying cause is not yet known. Deposition of IgAimmune complexes in different locations gives rise to the organ specific clinical signs: palpable purpura, arthralgias, kidney damage, abdominal pain and other forms of gastrointestinal involvement amongst others. The presence of cutaneous lesions is one of the diagnostic pillars of this pathology and its absence suggests other differential diagnoses. As regards treatment, most cases have a favorable clinical course with spontaneous resolution of symptoms, corticoid use being reserved for severe cases at the physician's discretion as there are no clinical guidelines. We present the case of a 3 year old girl with Henoch Schönlein purpura who originally presented with a gastrointestinal disorder with abdominal pain and lower gastrointestinal bleeding prior to the appearance of the rash.

Púrpura de Schönlein-Henoch en adulto HIV positivo adicto a cocaína y ANCA-p positivo / Henoch-Schönlein purpura in a cocaine consumer man with HIV infection and ANCA-p positivity

La púrpura de Schönlein-Henoch (PSH) es una vasculitis de pequeños vasos con depósito de inmunocomplejos de IgA. La presentación en adultos es rara y grave. Los casos comunicados de PSH en pacientes infectados por HIV son escasos. Los anticuerpos anticitoplasma de neutrófilos (ANCA), detectados por inmunofluorescencia indirecta (IFI), se encuentran comúnmente en otras vasculitis sistémicas primarias pero rara vez en PSH y es aún más inusual el patrón perinuclear (ANCA-p). Por otra parte, la positividad de los ANCA puede ser detectada en una serie de condiciones patológicas distintas de las vasculitis de pequeños vasos en asociación a procesos infecciosos, incluyendo el HIV, o a consumo de cocaína, y en especial el patrón de ANCA-p vinculado a medicamentos o enfermedades autoinmunes e inflamatorias intestinales. Se presenta el caso de un varón de 35 años con hábitos tóxicos (cocaína, marihuana) que consultó por dolor abdominal, hematoquecia y lesiones purpúricas en extremidades inferiores, que agregó fiebre, artralgias y progresión de lesiones purpúricas asociadas a síndrome nefrítico y ANCA-p (+). Durante la internación se detectó infección por HIV. La biopsia renal evidenció una nefropatía a IgA que tuvo una respuesta favorable al tratamiento corticoideo y antiproteinúrico. La comunicación del caso obedece a la rareza de la presentación y a la complejidad para establecer el diagnóstico y la terapéutica. Aún resta dilucidar el rol de los ANCA en la etiopatogenia y el manejo de la PSH del adulto.

The Henoch-Schönlein purpura (HSP) is a small vessel vasculitis with IgA immune complex deposition. The presentation in adults is rare and severe. Reported cases of HSP in patients infected with HIV are scarce. Neutrophil cytoplasmic antibodies (ANCA) are commonly found in other systemic vasculitis, but rarely in HSP and even more unusual the perinuclear pattern. Beside small vessel vasculitis, positivity of ANCA can be detected in a number of different pathological conditions in association with infectious processes, including HIV, or cocaine use, and especially the pattern of ANCA-p, associated with drugs, inflammatory bowel or autoimmune diseases. We report the case of a 35 years old man with toxic habits (cocaine, marijuana) who consulted for abdominal pain, hematochezia and purpura on lower extremities, and later fever, joint pain and progression of purpura associated with nephritic syndrome and ANCA-p (+). During hospitalization HIV infection was detected. Renal biopsy showed IgA nephropathy with favorable response to corticosteroid and antiproteinuric treatment. The communication of the case is due to the rarity of the presentation and therapeutic diagnostic challenge. It remains to elucidate the role of ANCA in the pathogenesis and management of adult PSH.

The clinical significance of hs-CRP and myocardial enzymes, cTn Ⅰ changes in allergic purpura patients with the myocardial injury / 中国基层医药

Objective To investigate the clinical significance of the changes of hypersensitive C-reactive protein (hs-CRP) and myocardial enzyme spectrum,cardiac troponin Ⅰ (cTn Ⅰ) in patients with Henoch-Schnlein purpura.Methods 100 patients with Henoch-Schnlein purpura were selected.50 patients only skin involved were selected as the simple group,50 patients accompanied by other organs involved were selected as mixed group.50 healthy people in the same period were selected as the control group.The serum hs-CRP,myocardial enzyme spectrum and cTn Ⅰ were tested and compared.Results The hs-CRP,serum myocardial enzyme spectrum and cTn Ⅰ levels of mixed and simple group were significantly higher than the control group(all P ＜ 0.05) ; Serum CK-MB,cTn Ⅰ and hs-CRP levels of the mixed group were significantly higher than those of simple group (all P ＜ 0.05).The abnormalities number of CK-MB and cTn Ⅰ respectively accounted for 33％ (33/100) and 32％ (32/100) ;The constituent ratio of the number of abnormal serum CK-MB and cTn Ⅰ of the mixed group was significantly higher than the simple group (x2 =4.047,3.908,all P ＜ 0.05) ;The Pearson correlation analysis showed that the relationship between the serum hs-CRP and CK-MB,cTn Ⅰ levels was linear,which were positively correlated (r =0.872,0.801,all P ＜ 0.05).Conclusion Patients with allergic purpura should early detect the serum levels of hs-CRP,myocardial enzyme spectrum and cTn Ⅰ,and treat the myocardial injury in time.

Henoch-Schonlein purpura: ultrasonography of scrotal and penile involvement

Testicular or scrotal involvement has been reported in children with Henoch-Schonlein purpura (HSP), but there are very few reports on penile involvement. We report the initial and follow-up ultrasonographic findings of scrotal and penile involvement of HSP in a 5-year-old boy. On ultrasonography, scrotal soft tissue thickening and epididymal swelling with increased vascularity were noted, and on the penis, a focal mass-like lesion appeared on the dorsal surface of the distal penis, having a hypoechoic mass-like appearance without visible vascular flow on a Doppler study. After 2 days of treatment, follow-up ultrasonography showed normal scrotum and penis with a resolved soft tissue mass-like lesion. Therefore, we think that HSP ultrasonographic findings involving the scrotum and penis might help to diagnose scrotal and penile involvement in a case of HSP and to avoid unnecessary medication and/or surgical procedures.

A Case of Elderly-Onset Crescentic Henoch-Schonlein Purpura Nephritis with Hypocomplementemia and Positive MPO-ANCA

Henoch-Schonlein purpura (HSP) is common in childhood and often self-limiting. There have been limited studies on elderly-onset HSP nephritis (HSPN). A 76-yr-old man was transferred to our hospital with a 1-month history of oliguria, abdominal pain, edema and palpable purpura in the legs. Three months ago, he was admitted to another hospital with jaundice, and consequently diagnosed with early common bile duct cancer. The patient underwent a Whipple's operation. Antibiotics were administrated because of leakage in the suture from the surgery. However, he showed progressive renal failure with edema and purpura in the legs. Laboratory investigations showed serum creatinine 6.4 mg/dL, 24-hr urine protein 8,141 mg/day, myeloperoxidase anti-neutrophil cytoplasmic antibodies (MPO-ANCA) 1:40 and C3 below 64.89 mg/dL. Renal biopsy showed crescentic glomerulonephritis, as well as mesangial and extracapillary Ig A deposition. We started steroid therapy and hemodialysis, but he progressed to end-stage renal failure and he has been under maintenance hemodialysis. We describe elderly onset HSPN with MPO-ANCA can be crescentic glomerulonephritis rapidly progressed to end stage renal failure.

Serum levels of insulin-like growth factor-1 and insulin-like growth factor-binding potein-3 in Henoch-Schonlein purpura / 中华风湿病学杂志

Objecfive To investigate the role of serum Insulin-like growth factor(IGF)-1,insulinlike growth factor-binding potein(IGFBP)-3 in children with Henoch-Schonlein purpura(HSP).Methods The serum concentration of IGF-1,1GFBP-3 was measured by enzyme-linked immunosorbent assay(ELISA)method in 45 acute SHP patients,40 recoverv patients and 30 healthy controls.Results The serum levels of IGF-1 [(452±183)μg/L],IGFBP-3 [(13 897±3124)μg/L] and C-reactive protein(CRP)[(20±8)mg/L]in acute phase were significantly higher than those in healthy controls(P0.05).The serum levels of IGF-1[(621±253)μg/L] and IGFBP-3[(18 763±3173)μg/L] were higher in the renal damage group than in the non-renal damage group(P0.05).whereas the serum level of CRP was not significantly different(P>0.05).The serum levels of IGF-1,IGFBP-3 showed positive correlation with the level of CRP(r=0.624,0.672,P<0.01).Conclusion The IGF-1 and IGFBP-3 may play an important role in the pathological mechanism of HSP.The level of IGF-1 may be used as an indicator for HSP disease activity and progression.IGF-1 mav have a close relation with the damageof renaJ system in HSP.

The role of activation of toll-like receptors in immunological pathogenesis of Henoch-Schonlein purpura / 中华风湿病学杂志

ObjectiveTo investigate the role of signal transduction of TLRs in the Henoch-Schonlein purpura (HSP). Methods Reverse-transcription PCR (RT-PCR) and real-time PCR were used to evaluate the levels of TLRs(1～10), MyD88, TRAF6, TRIF, IFN-α, IFN-β, IL-6, IL-1β, TNF-α, IP-10, RANTES,iNOS, Blys/April mRNA expression in peripheral blood mononuclear cells and their expression levels were compared using t test., while the concentration of plasma cytokines such as Blys、IFN-α、IFN-β、IL-6、IL-1、TNF-α was measured by enzyme-linked immunosorbent assay(ELISA).Expression levels of those genes were compared using t test. Results①Compared with the control group, the expression levels of TLR1, TLR2,TLR6, TLR5, TLR3, TLR7, TLR9 mRNA were up-regulated significantly(P＜0.01), while no difference of TLR4 was detected (P＞0.05).②Transcription levels of MyDg8(2.47±1.06) vs(0.73±0.22), TRAF6 (2.54±0.72)×10-3vs(0.70±0.20)×10-3, TRIF(3.18±0.86)×10-3vs(0.93±0.35)×10-3 were significantly up-regulated in acute phase of HSP (P＜0.01).③The levels of IFN-α and IFN-β protein and mRNA were remarkable increased (P＜0.01).④ The expression of cytokine/chemotactic factor such as IL-6, IL-1β, IP-10, RANTES,iNOS was higher than that of the control group(p＜0.01), while TNF-αdid not change in children with HSP (P＞0.05). ⑤ It was detected that the expression of Blys/April was higher than that of the control group(P＜0.01). ConclusionExpressions of TLR1, TLR2, TLR6, TIR5, TLR3, TLR7, TLR9, MyD88, TRAF6, and TRIF are up-regulated during acute phase of HSP, suggesting that aberrant activation of TLRs triggered by microbes may be one of the initiating factors of immune aberrance in HSP. Over expression of cytokine/chemotactic factor or Blys/April owning to the aberrant activation of TLRs, may be correlated with immunological pathogenesis of HSP.

Study of risk factors in renal involvement in children Henoch-Schonlein purpura / 中华疾病控制杂志

Objective To analyze children Henoch-Schonlein purpura of nephral involvement factors.Methods 212 cases of children hospitalized with HSP from Jan 1,2007 to Dec 31,2007,were divided into purpura nephritis(HSPN) and non-nephritis groups.Such factors as gender,age,clinical and laboratory tests were dealt with by single-and multi-variable analysis.Results ①96 cases(45.28%) in HSP were with kidney damage,92.71% HSPN children with abnormal urine test within 3 months.②Symptoms of severe rash,skin rash repeated in three months ≥3 times,persistent severe abdominal pain and severe joint involvement were closely related to the occurrence of HSPN.Logistic regression analysis had statistical significance(P

Clinical analysis of Fufangzigantang in treatmem of henoch-schonlein purpura / 中国基层医药

Objective To compare the efficacy and safety of Fufangrigantang,prednisone and chlorphena-mine in the treatment of henoch-schonlein purpura. Methods 96 cases were randomly divided into two groups, con-trol group 40 cases ,taking prednisone, chlorpheniramine ;56 cases of the treatment group taking compound decoction purple,were treated for two weeks. Results In the treatment group directive rate in was 96.4%. Effective rate in the control group was 72.5% (χ2=11.41, P<0.01). Conclusion To use Fufangzigantang with other conventional drugs together is better than use prednison and chlorphenamine in the treatment of allergic purpura.

Compromiso testicular en púrpura de Schõnlein Henoch, utilidad de la ecotomografía doppler / Testicular involvement in Henoch-Schõnlein purpura: the useful of doppler sonography

El púrpura de Schõnlein Henoch (PSH) es la vasculitis más frecuente de la infancia; afecta principalmente la piel, articulaciones y sistema gastrointestinal. El compromiso de los testículos se observa en el 15 por ciento de los pacientes, pero es raro como forma de presentación (1-2 por ciento). El presente caso clínico describe un niño de 4 años de edad sin antecedentes mórbidos que consultó por dolor, eritema, aumento de volumen testicular derecho y púrpura palpable de extremidades inferiores, cuya ecografía testicular mostró testículos normales con flujo Doppler normal y aumento de grosor de escroto y cordón espermático ipsi lateral. Exámenes de laboratorio fueron normales. Se diagnosticó PSH con compromiso testicular y se trató exitosamente con corticoides orales por una semana. Es importante considerar esta patología como diagnóstico diferencial de testículo agudo, para evitar la exploración quirúrgica innecesaria. Las características ecográficas permiten confirmar la sospecha diagnóstica.

Henoch Schõnlein purpura is the most common vasculitis presenting in the childhood, with frequent skin, articular and gastrointestinal compromise. Testicular pain is rare as an onset symptom (1-2 percent), but 15 percent of the cases develop it during the illness course. This case report describes a previously healthy 4 year old boy, who was admitted in theemergency department with a painful, erythematous right scrotal swelling, associated with palpable purpura on both inferior extremities. Testicular Color Doppler US imagingout ruled testicular alterations, but the ipsilateral scrotum and spermatic cord were enlarged. Blood was drawn for serologic and hematologic testing with normal results. Shõnlein Henoch purpura with testicular involvement was diagnosed and successfully treated with oral corticosteroids for one week. It is important to consider Schõnlein Henoch purpura among the differential diagnosis of acute testicle in order to avoid unnecessary surgical exploration. Testicular Doppler Ultrasound imaging is recommended to confirm the diagnosis.

Production of Chemokines in Kawasaki Disease, Henoch-Schonlein Purpura and Acute Febrile Illness

We compared the production of three chemokines; interferon-gamma-inducible protein-10 (IP-10), monocyte chemoattractant protein-1 (MCP-1) and growth-related onco-gene-alpha(Gro-alpha) that attracts monocytes or neutrophils, or both, in peripheral blood at acute stage of Kawasaki disease (n=29), Henoch-Schonlein purpura (n=15) and acute febrile illnesses (n=12). The production of the chemokines was assayed by ELISA. The plasma levels of IP-10 were markedly elevated in Kawasaki disease (538.6 +/-336.4 pg/mL) and acute febrile illnesses (417.1 +/-262.2 pg/mL) compared with in Henoch-Schonlein purpura (58.7 +/-95.7 pg/mL) (p<0.05). The MCP-1 levels were elevated in Kawasaki disease (443.0 +/-473.1 pg/mL) and acute febrile illnesses (328.6 +/-261.1 pg/mL) compared with in Henoch-Schonlein purpura (82.9 +/-79.0 pg/mL) (p<0.05). The Gro- levels were elevated only in acute febrile illnesses (134.3 +/-153.6 pg/mL) compared with in Kawasaki disease (31.8 +/-22.1 pg/mL) or Henoch-Schonlein purpura (29.4 +/-53.3 pg/mL) (p<0.05). According to these results, monocytes may play an important role in Kawasaki disease. In acute febrile illness-es, both monocytes and neutrophils may play an important role. By contrast, Henoch-Schonlein purpura may not be associated with the role of monocytes and neutrophils. Further studies using a larger number of cases are needed.