Back issues No.4 - 2010 / Correspondence  

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Bullous pemphigoid associated with acquired hemophilia
Ching-Yin Chen, Ya-Hui Chen, Ji-Chen Ho, Chieh-Shan Wu
DERMATOLOGICA SINICA 28 (2010) 173–176

A 24-year-old man had bullous pemphigoid confirmed by
histopathology, direct immunofluorescence, and a salt-split
skin immunofluorescence examination in another hospital.
He was treated with prednisolone 15 mg per day and was
stable for 2 years. The patient was then admitted to our hospital
for simple fasciotomy of an extensive and painful intramuscular
hematoma over the right thigh with impending
compartment syndrome from a machine injury over the right
wrist and right thigh. Extensive bruising combined with swelling
and tenderness over the right radial part, the right thigh
and some scattered bean-sized dark-red purpura over the
back and abdomen were revealed during a physical examination
(Figure 1). The patient’s history showed that there was
no previous personal or family history of bleeding tendency.
Preoperative clotting tests showed prolongation of
an activated partial thromboplastin time of 43.5 seconds and
a normal prothrombin time. Postoperatively, there was extensive
wound bleeding that required massive blood transfusion.
The factor VIII inhibitor level was 256 Bethesda units/mL
(normal: < 1 Bethesda units/mL) and the coagulant activity
of factor VIII was reduced to 8% (normal: 60–150%). The
skin lesions progressed to a diffuse nonblanchable confluent
dusky-red ecchymosis over an erythematous-edematous base
on the trunk and extremities (Figure 2). Under the impression
of acquired hemophilia type A, the patient was treated with
recombinant human factor VII (NovoSeven; Novo Nordisk
Inc., Bagsvoerd, Denmark) and immunosuppressive therapy
was subsequently administered to inhibit antibody production.
After replacement therapy with NovoSeven for 24 days, the
wound gradually stopped bleeding. Plasmapheresis (once daily for 1 week), pulse therapy with methylprednisolone
(1000 mg/day for 3 days) and rituximab (100 mg/week for
2 weeks) were initially used for correcting the coagulopathy.
Prednisolone (1 mg/kg/day) and cyclophosphamide (100 mg/
day) were prescribed as maintenance therapy. The coagulatory
disorder improved after 2 months of treatment associated
with reduction of factor VIII inhibitor to 35 Bethesda
units/mL with concomitant improvement of ecchymosis
(Figure 3). A subsequent reduction in the factor VIII inhibitor
level was observed and it was 3 Bethesda units/mL after
3 months of follow-up.

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