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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.