The histological and pathogenetic spectrum of cutaneous disease in monoclonal gammopathies

Abstract

The dermatological disorders associated with monoclonal gammopathies are clinically heterogeneous and may be divided into four groups with distinctive pathogenetic mechanisms (a) specific (infiltrative) disorders including primary and secondary cutaneous plasmacytoma and cutaneous lymphoplasmacytic infiltration of Waldenström's disease (b) skin disorders because of the deposition of monoclonal immunoglobulin (M protein), including amyloidoisis macroglobulinemia cutis, light chain deposition of Randall's disease, follicular spicules of the nose, and cryoglobulinemia (c) skin disorders associated with monoclonal gammopathies, including highly associated (>50%), weakly associated (<50%) or anecdotal and (d) miscellaneous (non specific). In most cases, histopathology is crucial to confirm or to diagnose those skin conditions and is also very useful to understand their pathogenetic mechanisms.

IgA pemphigus associated with monoclonal gammopathy completely resolved after achievement of complete remission of multiple myeloma with bortezomib, cyclophosphamide and dexamethasone regimen

“…As in some other autoimmune diseases, treatment responses to rituximab have been reported. However, none of the above treatment strategies is universally applicable [12][13][14][15][16][17][18].…”

“…Dermatologic manifestations of monoclonal gammopathies and disorders associated with monoclonal gammopathies[12,14] POEMS syndrome: polyneuropathy, organomegaly, endocrinopathy/edema, M-protein, skin changes.…”

Manifestations dermatologiques des gammapathies monoclonales : apport de l’histopathologie cutanée

“…• infiltration cutanée directe par la population monoclonale plasmocytaire ou lymphoplasmocytaire ; • induction de lésions secondaires par les dépôts d'immunoglobulines ; • lésions cutanées induites par l'activité biologique des immunoglobulines monoclonales [4].…”

M protein deposition in the skin: a rare manifestation of Waldenström macroglobulinemia

“…The third group comprises skin disorders associated with WM to varying degrees, which can be ascribed partly to quantitative or immunologic effects of immunoglobulin. The last group contains unspecific reactions, such as purpura and pruritus [2,6,7].…”

“…Ultrastructurally the depositions consist of mostly amorphous to granular, and in some cases also microfibrillar electron-dense material. Eruption of these lesions is not related to the stage of disease, or to serum IgM levels, but alternate periods of flattening and regrowth seem to relate to the efficacy of treatment [7][8][9][10].…”