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