HistoryThis section has been translated automatically.
DefinitionThis section has been translated automatically.
Mostly acute, frequently infection-associated, leukocytoclastic IgA1-positive immune complex (multi-organ) vasculitis (small vessel vasculitis), which occurs mainly in small children and adolescents and is clinically characterized dermatologically by a (palpable) purpura. The disease is the most common syndromal vasculitis in children.
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Occurrence/EpidemiologyThis section has been translated automatically.
Incidence: 10-15/100.000 children/year.
EtiopathogenesisThis section has been translated automatically.
Often associated with streptococcal infections of the upper respiratory tract. Preferred occurrence during the cold season. Other infections, e.g. viral infections or vaccinations against viral diseases (meningitis C) are also blamed as triggers, even if the connections are not always clear.
In rare cases, the disease in adults can also be induced by alcohol.
A monclonal (MGUS) or polyclonal IGA gammopathy must be clarified(Umemura H et al.).
ManifestationThis section has been translated automatically.
Occurring mainly (about 75%) in children between the ages of 4 and 11, no gender preference. No preference for ethnic groups.
Rare occurrence also in adults (into old age). IgA-associated leukocytoclastic vasculitis tends to be severe and regular in adults, with systemic involvement; systemic manifestations (severe illness, arthritides, renal pain) may dominate the clinical picture. IgG- or IgM-associated vasculitides are more common in adults (note: a clear classification of the deposited immunoglobins is not always possible).
The disease occurs seasonally more frequently in the winter months.
LocalizationThis section has been translated automatically.
Symmetrical infestation; mainly on the extensor sides of the legs (the lower legs are preferred in stasis) and on the buttocks.
Clinical featuresThis section has been translated automatically.
The clinical picture corresponds to an acute or chronic inflammatory systemic disease with varying organ involvement. At the beginning, during a prodromal phase lasting 14-21 days, vague general symptoms often develop with fever, uncharacteristic rheumatic complaints with arthralgias, arthritides and myalgias. Less commonly, myositis is present.
Skin: The characteristic clinical dermatologic picture (leading symptom) is characterized by hemorrhagic exanthema (palpable round-oval or retiform purpura), with emphasis on the lower extremity and buttocks, and with (ascending pattern) less frequent involvement of the upper extremity, face, and trunk.
Efflorescence type: Initially, 0.1 cm to 5.0 cm bright red spots develop. Later, maculo-papular, deep red, or red-blue to blue-black papules and plaques develop (palpable purpura). With increasing duration, a brown-yellow discoloration of the exanthema begins. Regression after 14-21 days. Total duration 3-16 weeks. In severe forms of the disease: Lentil- to coin-sized, pink- to blue-red, later haemorrhagic, itchy, possibly urticarial spots. Transition to blue-red papules and plaques, vesicles, haemorrhagic blisters, pustules and possibly ulcerations. 5-10% of patients develop a chronic persistent disease with a relapsing course.
- Arthritides (75% of patients); painful swellings, especially of the ankle joints (my child suddenly can no longer walk!).
- GI manifestations (50-75%): colicky abdominal pain, vomiting, gastrointestinal bleeding (hemorrhagic small vessel vasculitis of the intestinal mucosa).
- Kidneys: glomerulonephritis (30-90%) with micro/macrohematuria indistinguishable from IgA nephritis (histologically: mesangioproliferative glomerulonephritis) which may be complicative as Rapid progressive glomerulonephritis.
- CNS: headache, behavioural disturbances (10-30%).
LaboratoryThis section has been translated automatically.
Acute-phase reactions (high BSG, CRP, leuko- and thrombocytosis) go parallel with the acuteity of vasculitis. There may be circulating immune complexes (lowered complement: CH50,C3, Ced,C4), pathological urine sediment or blood in the stool. Activity parameters are the soluble sIL-2 and factor VIII-associated antigen (extent of endothelial damage; see factor VIII below).
There is no specific, diagnostically conclusive parameter from a laboratory point of view. In addition, immunological and molecular biological techniques should be used to exclude hepatitis C/B. Autoantibodies such as ANCA and ANA are associated with both activity and diagnosis.
HistologyThis section has been translated automatically.
Edema of the papillary dermis appears in early stages. The epidermis is usually unchanged; occasionally there is exocytosis of neutrophil granulocytes, pustular formation and necrosis. Mostly present is a moderately dense, superficial, perivascularly oriented inflammatory infiltrate of lymphocytes, histiocytes, neutrophil granulocytes and nuclear debris (leukocytoclasia, nuclear dust). Endothelial cells are epitheloid swollen and protrude into the lumen. Fibrin is found in the vessel wall of post-capillary venules. Different densities of perivascular accentuated erythrocyte extravasations can be detected. The exudation can lead to subepidermal blistering and consecutive epithelial necrosis, recognizable by spongiosis and fading keratinocytes.
Direct ImmunofluorescenceThis section has been translated automatically.
Granular deposits of IgA in the vessel walls. The IgA deposits are considered to be of major pathogenetic importance for the clinical picture (see also IgA nephropathy)
DiagnosisThis section has been translated automatically.
The diagnosis is based primarily on the typical history/clinical findings(palpable purpura, abdominal and joint pain; age by definition below 21 years, often preceded by an upper respiratory tract infection) and, if necessary, a histological examination of affected skin areas.
Laboratory findings may support the diagnosis, but are non-specific. In addition to an increased erythrocyte sedimentation rate, these include the detection of circulating immune complexes and an increased total IgA. The coagulation parameters are usually unremarkable (DD of purpura!), although global tests of blood coagulation such as the bleeding time (which also covers the vascular component of haemostasis) and the so-called Rumple-Leede test can be pathological. Caution: The Rumple-Leede or capillary resistance test is not specific for angiopathies; abnormal findings can also be found in thrombocytopenias and thrombopathies. If necessary, an increase in antistreptolysin titers may indicate a triggering upper respiratory tract infection.
It is important to monitor visceral organ function, especially the kidneys:
- Renal involvement may be present (renal involvement in the form of glomerulonephritis with hematuriaand proteinuria is present in 20-50% of children).
- Arthritides (ankle > knee > elbow): skeletal scintigram if necessary.
- Intestinal involvement: about 2/3 of children have gastrointestinal complications with recurrent colicky abdominal pain. Abdominal ultrasonography and, if necessary, radiological clarification or endoscopic diagnosis are useful.
- Neurological symptoms: if necessary, cranial CT or cranio-MRI diagnostics are required.
General therapyThis section has been translated automatically.
External therapyThis section has been translated automatically.
Slight volatile episodes or localised forms of the disease do not generally require treatment. If necessary, glucocorticoid-containing topicals, possibly also under occlusive dressings, e.g. 0.25% prednicarbate (e.g. Dermatop ointment) or 0.1% mometasone (e.g. Ecural ointment) or 0.5-1.0% hydrocortisone cream R120 or 0.1% betamethasone ointment R028 or 0.1% triamcinolone ointment.
Internal therapyThis section has been translated automatically.
Childhood: If the disease occurs in childhood, the prognosis is good, rarely an immunosuppressive therapy is necessary. For prophylaxis of the nephrotic syndrome some authors recommend the early use of glucocorticoids, in severe cases e.g. prednisone 1-3 mg/kg bw/day. There are no objectifiable data on the clinical effect (duration of disease, recurrence) of glucocorticoids in PSH, but only personal experience reports (evidence level D/E).
There are anecdotal reports (see vasculitis below) of dapsone and colchicine (level of evidence E).
Large-area infestation: Medium to high-dose monotherapy with glucocorticoids (e.g. 80-100 mg prednisolone equivalent). Rapidly eliminate within 14-21 days.
Severe purpura Schönlein-Henoch with systemic involvement(especially kidney involvement) and without self-limiting course: Dual immunosuppressive therapy with glucocorticoids such as prednisolone (e.g. Predni H, Decortin H) 80-200 mg/day and cyclophosphamide as the first choice. With gradual dose reduction, the lowest possible maintenance dose of the glucocorticoid should be aimed at for approx. 3-4 weeks after the skin findings. The combination with cyclophosphamide for a faster reduction of the glucocorticoids is appropriate in the case of extensive findings which require longer therapy.
Progression/forecastThis section has been translated automatically.
With the exception of Purpura fulminans favorable prognosis. Recurrences are possible for years. Control of kidney function necessary. Chronic renal insufficiency is possible.
TablesThis section has been translated automatically.
ACR-1990 criteria for the classification of the Purpura Schönlein-Hennoch*
Patient age < 20 years
Gastrointestinal complaints (diffuse abdominal complaints) or signs of intestinal ischaemia (bloody diarrhoea)
Detection of granulocytes and their nuclear fragments (leukocytoclasia) in and around the vessel walls of arterioles or venules
Additionally: Granular deposits of IgA in and around the vessel walls
*the detection of 2 of the first 4 diagnostic criteria is diagnostic (specificity > 85%)
ACR-1990 criteria for the classification of cutaneous leukocytoclastic angiitis
Patient age > 16 years
Medication to be taken when first symptoms appear
Detection of granulocytes and their nuclear fragments (leukocytoclasia) in and around the vessel walls of arterioles or venules
Note(s)This section has been translated automatically.
- In infancy and childhood IgA-associated leukocytocolic vasculitis also manifests itself under the picture of " infantile, acute, hemorrhagic edema", especially in the facial region (Finkelstein's disease).
- Another typical IgA-associated course of the disease in infancy and toddlers is the cocardian purpura (Seidelmeier): see below infantile, acute hemorrhagic edema.
LiteratureThis section has been translated automatically.
- Chua IC et al (2004) Cutaneous IgA-associated vasculitis induced by alcohol. Br J Dermatol 151(Suppl 68): 85-86
- Egan CA et al (2000) Relapsing Henoch-Schonlein purpura associated with Pseudomonas aeruginosa pyelonephritis. J Am Acad Dermatol 42: 381-383
- Fervenza FC (2003) Henoch-Schonlein purpura nephritis. Int J Dermatol 42: 170-177
- Gonzalez-Gay MA et al (2005) Clinical approach to cutaneous vasculitis. Curr Opin Rheumatol 17: 56-61
- Gougerot H (1932) Septicémie chronique indetérminée caracterisée par de petits nodules dermiques ("dermatitis nodularis non necroticans"), éléments érythémato-papuleux, purpura. Bull Soc franç dermatol syphiligr (Paris) 39: 1192-1194.
- Heberden W (1802) Commentaries on the History and Cure of Diseases. In: De purpureis maculis, chapter 78, T. Payne (ed.), London.
- Heng MCY (1985) Henoch-Schönlein purpura. Br J Dermatol 112: 235-240.
- Enoch E (1868) On the relation of purpura and intestinal disorders. Berliner Klinische Wochenschrift 5: 517-519
- Enoch E (1874) On a peculiar form of purpura. Berliner klinische Wochenschrift 11: 641
- Hundeiker M et al (1977) Dermatologic forms of purpura as early and late allergic reactions. Akt Dermatol 3: 39-48
- Kawasaki Y et al (2003) Clinical and pathological features of children with Henoch-Schoenlein purpura nephritis: risk factors associated with poor prognosis. Clin Nephrol 60: 153-160
Linskey KR et al (2012) Immunoglobulin A--associated small-vessel vasculitis: a 10-year experience at the Massachusetts General Hospital. J Am Acad Dermatol. 2012 May;66(5):813-22
Schoenlein JL (1832) General and special pathology and therapy. Transcribed from his lectures by some of his auditors and published unauthorized. Würzburg, Etlinger
- Schönlein JL (1841) Peliosis rheumatica. In: General and special pathology and therapy. 5th edition, St. Gallen, 2 volumes, p. 41.
- Umemura H et al. (2018) Leukocytoclastic vasculitis associated with immunoglobulin A lambda monoclonal gammopathy of undetermined significance: A case report and review of previouslyreported
cases. J Dermatol 45:1009-1012.
Incoming links (30)Abdominal purpura; Acute hemorrhagic infantile edema; Amebiasis; Beautiful little enoch purpura; Betamethasone valerate cream 0.05-0.1% (w/o); Complement Component 2 Deficiency; Complement system; Dermatitis-arthritis syndromes; Enoch, edward heinrich; Eosinophilic granulomatosis with polyangiitis; ... Show all
Outgoing links (16)Acute hemorrhagic infantile edema; Anca; Antinuclear antibodies; Betamethasone valerate cream 0.05-0.1% (w/o); Compression therapy; Cyclophosphamide; Factor viii; Glucocorticosteroids; Hydrocortisone cream 0.5-2.0% (w/o); Iga nephropathy; ... Show all
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