Subcorneal Pustular Dermatosis Associated with IgA Myeloma and Intraepidermal IgA Deposits

Abstract

We report a 59-year-old woman who had subcorneal pustular dermatosis and IgA-κ myeloma. Immunofluorescence tests showed intercellular IgA-κ deposits in the upper portion of the lesional epidermis and circulating IgA-κ anti-intercellular autoantibodies in a titer of 1:40. A combination chemotherapy for myeloma induced dramatic improvement of the skin lesions in accordance with a marked decrease in serum IgA levels as well as the disappearance of circulating anti-intercellular IgA autoantibodies, suggesting a pathogenetic link between skin lesions and IgA-κ paraprotein produced by myeloma cells.

Antidesmocollin 1 autoantibody negative subcorneal pustular dermatosis-type IgA pemphigus associated with multiple myeloma

“…Those IgA pemphigus cases associated with anti‐Dsg1 or anti‐Dsg3 antibodies show IgA deposits in cell surfaces of only the uppermost or lower epidermis, respectively. The staining pattern of our patient and that described in the report by Takata et al . may be similar to that observed in Dsc1‐related IgA pemphigus.…”

“…Five cases of SPD‐type IgA pemphigus associated with MM have been reported . Molecular studies were carried out in one of these cases, which showed negative results for anti‐Dsc1–3 IgA/IgG antibodies, as we found. Additional immunological and molecular studies in our patient ruled out IgA/IgG autoantibodies against other epidermal antigens.…”

“…It is not known why only some of these patients will develop an associated haematological malignancy . Five cases of SPD‐type IgA pemphigus associated with MM have been reported . Molecular studies were carried out in one of these cases, which showed negative results for anti‐Dsc1–3 IgA/IgG antibodies, as we found.…”

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].…”

Dermatoses neutrofílicas: parte II

“…Among them we highlight IgA and IgG monoclonal gammopathy, as well as myeloproliferative disorders, especially multiple myeloma. 7,8,9 The onset of these diseases may occur after years, which justifies the prolonged follow-up of these patients.…”