Granulomatous allergic vasculitis – symptoms, treatment, prognosis

Syn .: Granulomatosis allergica Churg-Strauss, Churg-Strauss syndrome, Churg-Strauss granuloma, allergic granuloma, neutrophilic and palisomatous granulomatous dermatitis, interstitial granulomatous dermatitis with arthritis and rheumatoid papules, allergic granulomatous arteritis.

Def .: Granulomatous necrotizing vasculitis within internal organs and skin, accompanied by eosinophilia and asthma.

Epid .: A rare disease, women suffer more often, usually in middle age.

Etiol .: Cause unknown. The syndrome has been reported with the use of leukotriene receptor antagonists (zafirlukast).

Loc .: Multiorgan: vasculitis type changes

(allergic angiitis) can include the respiratory system, gastrointestinal tract, heart, nervous system, kidneys, skin.

Clin.:

* Respiratory: rhinitis (hay fever), asthma, pulmonary inflammation and granulomas, and eosinophilic infiltrates leading to adverse lung disease.

* Digestive tract: lung eosinophilic infiltrates may occupy the gastrointestinal tract.

* Heart: infiltrative changes in the coronary vessels and the heart muscle.

* Kidneys: kidney involvement is rare and the disease is milder.

* Skin: skin lesions are present in about 10% of patients; can be of various forms:

– petechiae,

– erythema similar to erythema multiforme,

– painful lumps, most often on distal parts of the limbs or on the scalp,

– macular papules, they may become crusted,

– ulcers

– subcutaneous, disintegrating tumors,

– in patients with rheumatoid arthritis, the possibility of cutaneous granulomas similar to granuloma annulare or rheumatoid nodule (nodus rheumaticus).

Hist .: The histopathological picture consists of:

* In erythematous lesions: necrotizing vasculitis with eosinophils, without granulomatous reaction.

* In nodular changes:

– basophilic necrosis surrounded by a palisade-like granulation tissue (Churg-Strauss granuloma),

– infiltrates of lymphocytes, eosinophils, histiocytes and giant cells in the lower layers of the skin,

– leukocytoclastic vasculitis,

– diffuse mucin, in small amounts.

DL: Eosinophilia – also in lavage from the bronchial tree, accelerated ESR, elevated acute phase proteins.

DI: In about 70% of cases, positive antibodies against various antigens of leukocytes, mainly not myeloperoxidase, the so-called antibodies staining the periphery of the nucleus (perinuclear ANCA – pANCA), sometimes elevated levels of IgE.

DR: Asthma, other forms of necrotizing vasculitis.

Heal: Multidisciplinary treatment of organ disease. Skin changes:

– general treatment: corticosteroids, sulfones (dapsone), antimalarial drugs, retinoids, PUVA;

– local treatment: intralesional corticosteroids (triamcinolone acetate) or under an occlusive dressing.

Year: Chronic disease. The prognosis is worsened by the occurrence of cardiac or pulmonary changes.

Lit.: [1] Keogh K.A., Specks V.: Churg-Strauss syndrome: update on clinical, laboratory and therapeutic aspects. Sarcoidosis Vasc Diff use Lung Dis 2006, 23(1); 3-12. [2] Keogh K.A., Specks V.: Churg-Strauss syndrome. Semin Respir Crit Car Med 2006, 27(2); 148-57.

Source: A. Kaszuba, Z. Adamski: “Lexicon of dermatology”; XNUMXst edition, Czelej Publishing House

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