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• In 2015, an 84-year-old Caucasian woman developed a

pruriginous eruption on the arms, legs and trunk, in the

absence of mucosal involvement or constitutive symptoms.

• Skin biopsy identified a neutrophilic and eosinophilic

infiltrate, with a moderate aggregation of lymphocytes

around the small blood vessels.

• Direct immunofluorescence showed a positive staining of

basal epidermal cells with IgG, IgM and C3 deposits, while

indirect immunofluorescence remained negative.

• Serum BP180 ELISA was positive (33.05 RU/mL, normal

range <20), and immunoelectrophoresis showed an IgA

Kappa paraprotein (8g/L).

• The diagnosis of bullous dermatosis was retained in the

context of MGCS.

• Treatment with topical corticosteroids was initiated without

success, immunosuppressive therapy consisting of steroids

and later on ciclosporin also failed to be effective.

• In November 2019, in a context of fatigue and weight loss,

the hematological situation evolved to a SD stage II, ISS 3

IgA kappa MM that required a systemic therapy with the

VRD lite regimen.

• Very good partial response was achieved after three cycles,

with rapid and complete resolution of the skin lesions.

Patients with uncommon bullous eruption should be investigated for the development of an

underlying plasma cell disorder, since monoclonal proteins may possess an antibody activity

against basal membrane antigens, causing bullae formation.

Plasma cell dyscrasias have been associated with a variety of skin manifestations in the context of monoclonal gammopathy of

clinical significance (MGCS). So far, only three cases of acquired bullous dermatosis have been reported in the literature in

association with IgA multiple myeloma (MM).

Bullous dermatosis as first manifestation of IgA multiple myeloma.

Dufour I1, Verstraete G1, Raedemaeker J1, Andreozzi F1, Sogorb A1, Bamps L1,

Chabert S1, Bailly S1, Herman A2, Tennstedt D2, Marot L2, Dachelet C3, Camboni A3,

Lecouvet F4, Defour JP5, Vekemans MC1

Department of Hematology1, Dermatology2, Pathology3, Radiology4, Clinical Biology5

Cliniques universitaires Saint-Luc, UCL, Brussels, Belgium

BACKGROUND

CASE PRESENTATION

Figures 1 et 2 - Diffuse pruriginous skin eruption predominantly developed

on arms and legs.

CONCLUSION

1 2

Figure 3 - Skin biopsy showing a neutrophilic and eosinophilic infiltrate,

with a moderate aggregation of lymphocytes around the small blood vessels.

3

0

5

10

15

20

25

30

35

M-p

rote

in (

g/L

)

Figure 4 – M-protein evolution under treatment. IgA kappa

dropped from 29 g/L to 4.3g/L, after three cycles of therapy.