Cutaneous extravascular necrotizing granuloma and lymphocytic lymphoma

Abstract

A patient had clinical findings of asthma, peripheral eosinophilia, multisystem abnormalities, and cutaneous extravascular granulomas, consistent with a diagnosis of allergic granulomatosis. In addition, a well-differentiated lymphocytic lymphoma was diagnosed on the basis of bone marrow findings, and a monoclonal gammopathy was demonstrated by serum immunoelectrophoresis. Clinically, the skin lesions appeared as erythematous papules or nodules on the extremities, and some of them showed central ulceration. Histologically, there were dermal extravascular necrotizing granulomas embedded in a granulomatous and inflammatory cell infiltrate. These lesions bore a striking resemblance to those described by Churg and Strauss in patients with allergic granulomatosis, although tissue eosinophilia was significantly less prominent.

Extravascular necrotizing palisaded granulomas as the presenting skin sign of systemic lupus erythematosus

“…In accordance with this hypothesis we demonstrated prominent deposits of IgM and C3 in dermal vessels and at the epidermal–dermal junction and histopathological features of leucocytoclastic vasculitis besides characteristic granulomas. Similar findings of basement membrane IgM and C3 fluorescence and superficial vessels positive in IgG and C3 have also been described in a case of extravascular necrotizing palisaded granulomas in a lupus patient by Finan and Winkelmann 1 . Immune complex generation and deposition similar to that of extravascular necrotizing palisaded granulomas may be seen in ‘ordinary’ leucocytoclastic vasculitis, urticarial vasculitis, erythema elevatum et diutinum and Sweet syndrome 16,17 .…”

“…They are characterized clinically by the occurrence of sometimes tender, erythematous to violaceous papulonodules usually symmetrically distributed on the elbows or distal upper extremities and fingers, and are histologically defined by the presence of palisading macrophages surrounding a central necrosis composed of basophilic fibrin, degenerative collagen, neutrophils and neutrophilic debris 1 . Several mainly histopathological retrospective studies have observed these granulomas in patients suffering from Churg–Strauss disease, Wegener's granulomatosis, 2 rheumatoid arthritis, subacute bacterial endocarditis, 3 lymphoproliferative disease, 4–6 infectious hepatitis, 3 inflammatory bowel disease, 7 Takayasu arteritis 8,9 and systemic lupus erythematosus (SLE) 1,7 , 10 …”

Clinical utility of ANCA tests for the dermatologist

“…Histopathologic correlates include vasculitis (LCV or granulomatous), acute and /or neutrophilic inflammation or palisaded granuloma. This latter entity has been referred to as the Churg-Strauss granuloma or extravascular granuloma 75 (Figs 10 & 11). Once again, this entity is not specific for WG and can be seen in a variety of other (Table 3).…”

Collagenolytic (necrobiotic) granulomas: part II – the ‘red’ granulomas

“…65,66 Although the Churg-Strauss granuloma is found most frequently on patients with CSS, it can also occur in patients with autoimmune and lymphoproliferative disorders and is therefore not pathognomonic of CSS. 67,68 The third phase of CSS development can include a neuropathy, which occurs in 66% of patients. The most common symptom is usually a mononeuritis monoplex, which can progress to a symmetrical sensori-motor peripheral neuropathy.…”