A Case of Acquired Autoimmune Bullous Disease Associated with IgM Macroglobulinaemia

Morita, Eishin; Horiuchi, Kenji; Yamamoto, Shoso; Hashimoto, Takashi

doi:10.1111/j.1346-8138.1999.tb02070.x

Cited by 28 publications

(24 citation statements)

References 17 publications

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“…A rare complication because of the deposition of the paraprotein in Waldenstrom macroglobulinemia is subepidermal bullous dermatosis, associated with IgM gammopathy or IgM bullous disease . In this disorder, circulating IgM antibodies directed at the basement membrane zone have been found to localize to the lamina lucida and subepidermal basement membrane zone.…”

Section: Discussionmentioning

confidence: 99%

Cutaneous macroglobulinosis: a case series

Camp

Magro

2012

J Cutan Pathol

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Cutaneous macroglobulinosis is a rare skin manifestation of Waldenstrom macroglobulinemia. It is characterized by the deposition of eosinophilic, immunoglobulin‐derived material in the dermis. It typically presents as pink or skin‐colored papules favoring the extensor surfaces of the extremities. There are 11 reported cases of cutaneous macroglobulinosis in the literature. In our consultative dermatopathology practice we encountered three additional cases. In Case 1, a 41‐year‐old female with a monoclonal immunoglobulin M (IgM)‐kappa gammopathy developed skin‐colored papules on her extensor extremities, forehead and back. Histopathologic and immunohistochemical analyses revealed periodic acid‐Schiff (PAS)‐positive pink material in the dermis that stained with IgM. In Case 2, an 83‐year‐old female with a monoclonal IgM‐lambda paraproteinemia developed non‐blanching papules and plaques on the lower extremities and cheeks. Skin biopsies showed a striking occlusion of the vessels with a PAS‐positive eosinophilic precipitate, which was also found in the extravascular spaces and stained with IgM using direct immunofluorescence (DIF) and immunohistochemical stains. In Case 3, an 80‐year‐old male with Waldenstrom macroglobulinemia developed ulcerated papules and nodules on the lower extremities. The biopsy findings were similar to those of Case 2. We present a series of three patients with cutaneous macroglobulinosis and explore variations in the clinical and histopathological findings of this uncommon entity.

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Section: Discussionmentioning

confidence: 99%

Cutaneous macroglobulinosis: a case series

Camp

Magro

2012

J Cutan Pathol

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“…However, in all previous cases of autoimmune bullous diseases with macroglobulinemia, IgM reactivity with the BMZ was detected ( Table 1). [3][4][5][6][7][8] In the only one case report, immunoblot analysis revealed the IgM antibodies to 290-kDa protein in dermal extract. 6 Therefore, it is still possible to speculate that IgM antibodies in this case may play a role in blister formation.…”

Section: Discussionmentioning

confidence: 99%

Possible paraneoplastic syndrome case of bullous pemphigoid with immunoglobulin G anti‐BP180 C‐terminal domain antibodies associated with psoriasis and primary macroglobulinemia

Maki

Demitsu

Umemoto

et al. 2015

The Journal of Dermatology

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A 61-year-old Japanese man developed bullous skin lesions during topical therapy for psoriasis vulgaris. Physical examination demonstrated numerous tense bullae and scaly erythemas on the trunk and extremities. Histopathology of the skin biopsy demonstrated subepidermal bullae and lymphocytic infiltration with eosinophils in the dermis. Direct immunofluorescence revealed linear deposits of immunoglobulin (Ig)G, IgA and C3 along the basement membrane zone. Indirect immunofluorescence of 1 mol/L NaCl-split skin showed IgG reactivity with both epidermal and the dermal sides. IgM reactivity with both the epidermal and dermal sides was also detected. Enzyme-linked immunosorbent assays showed negative results for both BP180 and BP230. Immunoelectrophoresis of serum and bone marrow aspiration revealed underlying primary macroglobulinemia with M-proteinemia of IgM-κ type. Immunoblot analysis revealed IgG, but not IgM, antibodies to recombinant protein of BP180 C-terminal domain. We diagnosed the present case as bullous pemphigoid with IgG anti-BP180 C-terminal domain autoantibodies associated with primary macroglobulinemia and psoriasis vulgaris. Systemic administration of prednisolone 30 mg/day resulted in dramatic improvement of both bullous and psoriatic skin lesions. When the bullous and psoriatic lesions relapsed, DRC chemotherapy (dexamethasone, rituximab and cyclophosphamide) for macroglobulinemia was performed. Then, the psoriatic lesions improved and the bullous lesions disappeared. We suggested that the present case may be paraneoplastic syndrome of bullous pemphigoid associated with primary macroglobulinemia and psoriasis vulgaris.

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“…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.…”

Section: Dermatose Bulleuse à Igmunclassified