Waldenström Macroglobulinemia–Induced Bullous Dermatosis

Cutaneous Waldenström's macroglobulinemia with “deck-chair” sign treated with cyclophosphamide

“…The first category includes both bullous dermatosis with subepidermal blisters and the presence of IgM and kappa light chains in direct immunofluorescence 6 and IgM storage lesions 7 characterized by flesh-colored or translucid papules, predominantly on extensor skin surfaces, buttocks, and the trunk, with the presence of an eosinophilic periodic acidSchiffepositive material occupying the dermis, but no staining for amyloid substance.…”

Gangrenous, Hemorrhagic, Bullous Cellulitis Associated with Pseudomonas aeruginosa in a Patient with Waldenström's Macroglobulinemia

“…We believe that the underlying Waldenströ m's macroglobulinemia contributed to the pathophysiology of the development of the rare skin manifestations of the infection seen in our patient because there have been other reports in the literature of patients with Waldenströ m's macroglobulinemia and related disorders who had hemorrhagic, bullous lesions [9][10][11][12]. Our patient did not have any previous skin manifestations of Waldenströ m's macroglobulinemia or hypergammaglobulinemic purpura.…”

“…There have been published cases of patients, with Waldenströ m's macroglobulinemia and hyperglobulinemia, who developed various types of skin lesions including purpura and hemorrhagic bullae [9][10][11][12] and yet there was no description in these case reports of such devastating skin lesions observed in our patient. A possible underlying mechanism of the initial development of the lesions in our case may be the dermal deposition of IgM globulins and destruction of the skin continuity [9].…”

“…A possible underlying mechanism of the initial development of the lesions in our case may be the dermal deposition of IgM globulins and destruction of the skin continuity [9]. We hypothesize that such a skin breach was secondarily superinfected with P. aeruginosa.…”

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

“…La maladie est caractérisée par des dépôts d'immunoglobulines IgM kappa à la jonction dermoépidermique en immunohistochimie et en immunofluorescence directe. Ces dépôts pourraient jouer un rôle dans la formation des lésions bulleuses mais le mécanisme physiopathologique reste inconnu [31][32][33][34][35], même si l'on peut penser qu'un mécanisme proche de celui des dermatoses bulleuses auto-immunes de jonction puisse être impliqué.…”