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Immunobullous dermatosis associated with Waldenström macroglobulinaemia treated with rituximab.
[waldenström macroglobulinemia]
Waldenström
macroglobulinaemia
(
WM
)
is
a
chronic
lymphoproliferative
disorder
characterized
by
the
presence
of
a
monoclonal
IgM
paraprotein
.
Specific
cutaneous
features
of
WM
include
neoplastic
cell
infiltrates
,
IgM
storage
papules
and
IgM
bullous
dermatosis
.
We
report
a
patient
with
subepidermal
bullous
disease
associated
with
WM
.
Immunofluorescence
identified
IgM
deposition
along
the
basement
membrane
zone
(
BMZ
)
with
circulating
anti-
BMZ
IgM
antibodies
reacting
with
the
dermal
side
of
salt
-
split
skin
.
The
autoantibodies
did
not
react
with
type
VII
collagen
or
laminin
332
.
Following
failed
treatment
with
doxycycline
,
prednisolone
,
intravenous
immunoglobulin
and
dapsone
,
the
patient
was
successfully
treated
with
a
modified
RCVP
regimen
(
rituximab
,
cyclophosphamide
and
prednisolone
)
.
To
our
knowledge
,
this
is
the
first
reported
case
of
IgM
bullous
disease
of
WM
treated
with
rituximab
.
Diseases
Validation
Diseases presenting
"first reported case"
symptom
achondroplasia
acute rheumatic fever
alexander disease
allergic bronchopulmonary aspergillosis
aniridia
cutaneous mastocytosis
dedifferentiated liposarcoma
epidermolysis bullosa simplex
fabry disease
focal myositis
harlequin ichthyosis
heparin-induced thrombocytopenia
kabuki syndrome
malignant atrophic papulosis
megacystis-microcolon-intestinal hypoperistalsis syndrome
pleomorphic liposarcoma
primary hyperoxaluria type 1
thoracic outlet syndrome
waldenström macroglobulinemia
well-differentiated liposarcoma
werner syndrome
wolf-hirschhorn syndrome
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