Purpura beauty enoch D69.0

Authors: Prof. Dr. med. Peter Altmeyer, Konrad Heisterkamp

Co-Autor: Julian Baur

Our authors

Last updated on: 29.10.2020

Dieser Artikel auf Deutsch

Synonym(s)

anaphylactoids purpura; Athrombopenic purpura; Beautiful little enoch Purpura; Gougerot's disease; Gougerot's trisymptom; Gougerot symptom; Henoch-Schönlein Purpura; IgA vasculitis; Immunocomplex vasculitis; Leucocytoclastic vasculitis; Maladie tri(penta)-symptomatique Gougerot; peliosis rheumatica; Purpura Beauty; purpura rheumatica; Trisymptom Gougerot; Vasculitis allergica-hemorrhagic type

History
This section has been translated automatically.

Heberden, 1802; Schönlein, 1832; Henoch, 1868; Gougerot, 1932

Definition
This 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.

Occurrence/Epidemiology
This section has been translated automatically.

Incidence: 10-15/100.000 children/year.

Etiopathogenesis
This 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.).

Manifestation
This section has been translated automatically.

Mainly (about 75%) occurring in children between the ages of 4 and 11, no gender preference. No preference for ethnic groups.

Rare occurrence also in adults (up to old age). IgA-associated leukocytoclastic vasculitis tends to be severe and regular with systemic involvement in adulthood. IgG- or IgM-associated vasculitis is more frequent in adults (note: a clear assignment of the deposited immunoglobulins is not always possible).

The disease is seasonally more frequent in the winter months.

Localization
This 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 features
This 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, often vague general symptoms develop with fever, uncharacteristic rheumatic complaints with arthralgias, arthritides and myalgias. Myositides are more rarely present.

Skin: The characteristic clinical-dermatological picture (leading symptom) is characterized by a hemorrhagic exanthema (palpable round-oval or retiform purpura), with emphasis on the lower extremity and the buttocks, and in (ascending pattern) more rarely on the upper extremity, face and trunk.

Efflorescence type: Initially light red spots of 0.1 cm to 5.0 cm in size appear. Later maculo-papular, deep red, or red-blue, to blue-black papules and plaques (palpable purpura) develop. With further increasing duration a brown-yellow discoloration of the exanthema begins. Recovery after 14-21 days. Total duration 3-16 weeks. In severe cases: lenticular to coin-sized, pink to blue-red, later haemorrhagic, itchy, possibly urticaria-transforming spots. Transition to blue-red papules and plaques, blisters, haemorrhagic blisters, pustules and possibly ulcerations. 5-10% of patients develop a chronic persistent clinical picture with intermittent course.

Extracutaneous manifestations:

  • Arthritis (75% of patients); painful swelling, especially of the ankle joints (my child suddenly can't walk anymore!).
  • GI manifestations (50-75%): colicky abdominal pain, vomiting, gastrointestinal bleeding (small vascular haemorrhagic vasculitis of the intestinal mucosa).
  • Kidneys: glomerulonephritis (30-90%) with micro- /macro-haematuria which is indistinguishable from IgA nephritis (histologically: mesangioproliferative glomerulonephritis) and which can progress complicatively as rapid progressive glomerulonephritis.
  • CNS: headache, behavioural disorders (10-30%).

Laboratory
This 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.

Histology
This 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 Immunofluorescence
This 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)

Diagnosis
This section has been translated automatically.

The diagnosis is mainly based on the typical anamnesis/clinic(palpable purpura, abdominal and joint pain; age by definition below 21 years, often preceding upper respiratory tract infection) and, if necessary, a histological examination of affected skin areas.

Laboratory findings may support the diagnosis, but are not specific. In addition to an increased blood sedimentation rate, these include the detection of circulating immune complexes and an increased total IgA. The coagulation parameters are usually inconspicuous (DD of the purpura!), although global tests of blood coagulation such as bleeding time (which also records the vascular component of hemostasis) and the so-called rumple-led test can also be pathological. Caution: The Rumple-Leede or capillary resistance test is not specific for angiopathies, conspicuous findings are also found in thrombocytopenia and thrombocytopathy. An increase in antistreptolysin titers may indicate a triggering infection of the upper respiratory tract.

It is important to monitor visceral organ functions, especially the kidneys:

  • Kidney involvement may be present (renal involvement in the form of glomerulonephritis with haematuria and proteinuria occurs in 20-50% of children).
  • Arthritides (hocks > knee > elbow): skeletal scintigram may be present.
  • Intestinal involvement: about 2/3 of the children have gastrointestinal complications with recurrent colicky abdominal pain. Abdominal sonography and, if necessary, radiological clarification or endoscopic diagnostics are appropriate.
  • Neurological symptoms: if necessary, cranial CT or cranio-MRI diagnostics are necessary.

General therapy
This section has been translated automatically.

Physical protection or bed rest (reduction of the static effect) and, if necessary, an accompanying mild compression therapy of the lower extremity.

External therapy
This 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 therapy
This 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).

If streptococci are detected in the throat swab: benzylpenicillin or penicillin V.

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/forecast
This 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.

Tables
This section has been translated automatically.

ACR-1990 criteria for the classification of the Purpura Schönlein-Hennoch*

Patient age < 20 years

palpable purpura

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

palpable purpura

Maculopapular exanthema

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.

Literature
This section has been translated automatically.

  1. Chua IC et al (2004) Cutaneous IgA-associated vasculitis induced by alcohol. Br J Dermatol 151(Suppl 68): 85-86
  2. Egan CA et al (2000) Relapsing Henoch-Schonlein purpura associated with Pseudomonas aeruginosa pyelonephritis. J Am Acad Dermatol 42: 381-383
  3. Fervenza FC (2003) Enoch-Schonlein purpura nephritis. Int J Dermatol 42: 170-177
  4. Gonzalez-Gay MA et al (2005) Clinical approach to cutaneous vasculitis. Curr Opin Rheumatol 17: 56-61
  5. Gougerot H (1932) Septicémie chronique indérminée caracterisée par de petits nodules dermiques ("dermatitis nodularis non necroticans"), éléments érythémato-papuleux, purpura. Bull Soc franç dermatol syphiligr (Paris) 39: 1192-1194
  6. Heberden W (1802) Commentaries on the History and Cure of Diseases. In: De purpureis maculis, chapter 78, T. Payne (ed.), London
  7. Heng MCY (1985) Henoch-Schönlein Purpura. Br J Dermatol 112: 235-240
  8. Enoch E (1868) On the connection between purpura and intestinal disorders. Berlin clinical weekly 5: 517-519
  9. Enoch E (1874) About a peculiar form of purpura. Berlin clinical weekly 11: 641
  10. Hundeiker M et al (1977) Dermatological purple forms as early and late allergic reactions. Act Dermatol 3: 39-48
  11. 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
  12. Schönlein JL (1832) General and special pathology and therapy. Written down after his lectures by some of his listeners and published without authorization. Würzburg, Etlinger
  13. Schönlein JL (1841) Peliosis rheumatica. In: General and special pathology and therapy. 5th edition, St. Gallen, 2 volumes, p. 41
  14. 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.

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

Authors

Last updated on: 29.10.2020