Wegeners granulomatosis of Face

1. Locally Advanced Wegener's Granulomatosis of Face Muhammad Saaiq
Ann. Pak. Inst. Med. Sci. 2010; 7(1): 67-69 67
Case Report
Locally Advanced Wegener's
Granulomatosis of Face Culminating
in Death
A 25 years old pregnant lady presented with an extensive destructive
lesion involving nose, cheeks, upper lip, and lower eyelids. Histology of the
lesion showed necrotizing granulomatous inflammation with evidence of
vasculitis. The patient was antineutrophilic cytoplasmic antibody (c-ANCA)
positive. There was no evidence of any systemic involvement. Following
delivery, combination therapy with systemic steroid and
cyclophosphamide was instituted, however the patient kept on
deteriorating until death.
KeyWords: Wegener granulomatosis. Necrotizing vasculitis.
Antineutrophilic cytoplasmic antibody.
Muhammad Saaiq
Address for Correspondence:
Dr Muhammad Saaiq,
Department of Plastic Surgery,
Pakistan Institute of Medical
Sciences (PIMS), Islamabad.
muhammadsaaiq5@gmail.com
Introduction
Wegener granulomatosis (WG) is a clinicopathologic
entity, characterized in its classical form by the
Wegener’s triad of necrotizing granulomatous lesions of
the upper or lower respiratory tract, generalized
necrotizing vasculitis involving both arteries and veins,
and focal necrotizing glomerulonephritis. Although in its
classical form, WG is typically a multisystem disease
with protean manifestations, presentation of the disease
with limited organ involvement has long been
recognized. Regional WG refers to the sinopulmonary
disease in the absence of renal disease.
1-3
The diagnosis is based on clinical assessment and
histopathology. On histology, the hallmark lesion is
necrotizing vasculitis of small arteries and veins together
with granuloma formation that can be either
intravascular or extravascular. Lung involvement if
present is typically bilateral nodular cavitary infiltrates,
which, on biopsy findings, demonstrate necrotizing
granulomatous vasculitis. The renal biopsy lesion is that
of a pauci-immune necrotizing and crescentic
glomerulonephritis. A positive c-ANCA test is of value in
confirming the diagnosis, but these autoantibodies may
be absent, particularly in the limited variety of the
disease. Therefore a positive c-ANCA test is adjunctive
evidence for the diagnosis, a negative result must be
viewed in the context of the patient's clinical picture and
disease activity. 1,4, 5
We report our experience with a young pregnant
lady who presented with locally advanced WG. Regional
WG with predominant involvement of the facial
structures is rare and that too in the context of
pregnancy is even more rarer. This rarity prompts us to
report our case.
Case Report
A 25 years old lady initially presented with several
months history of illness with insidious onset of a lesion,
initially involving the surface of the nose. The lesion
progressively spread to involve the adjacent areas of
mid face, cheeks, upper lip, and lower eyelids. The lady
was having pregnancy of third trimester with a viable
fetus. On clinical examination, an ill defined lesion with
overlying plaques was visible on mid face. (Figure I)
There was induration of the surrounding subcutaneous
tissues. The regional lymph nodes were not enlarged.
There was no other systemic involvement.
Histology of the lesion showed necrotizing
granulomatous inflammation with evidence of vasculitis.
No fungal infection was seen. c-ANCA was also
positive. Basic essential investigations including Blood
complete examination, serum urea, creatinine,
electrolytes, blood sugar, liver function tests were all
within normal limits. X-ray chest was normal and did not
reveal any pathology. Ultrasound abdomen was also
unremarkable.

2. Locally Advanced Wegener's Granulomatosis of Face Muhammad Saaiq
Ann. Pak. Inst. Med. Sci. 2010; 7(1): 67-69 68
Figure. I: First time presentation with ill defined
lesion on mid face, with overlying plaques.
Consultation of Medicine and Gynae colleagues was
sought. The patient and her husband were explained the
benefits and risks of combination therapy with systemic
steroids and cyclophosphamide particularly in the
context of the viable fetus. They did not consent for the
institution of the proposed therapy and preferred to
continue pregnancy. She availed consultation at Mother
Child Health Centre, where she had antenatal booking
and subsequently delivered vaginally. During the last
one and half month of pregnancy, her condition of the
facial lesions deteriorated precipitously. One week after
delivery she presented to us again. ( Figure II )
Figure. II: Second time presentation following
vaginal delivery of viable baby.
This time she had extensive destruction of her facial
structures. ENT examination revealed destruction of
nasal walls. She was admitted for indoor management.
In collaboration with our colleagues from oncology and
medicine, Systemic steroid ( Prednisolone 1 mg/kg body
weight/ day ) and cyclophosphamide ( 2 mg/kg body
weight/ month ) therapy was instituted. Patient could
not survive to receive the next cycle of
cyclophosphamide.
Discussion
Our patient had a rare presentation of the
disease with regional WG, predominantly involving the
facial structures. We could not find any published report
of the disease in the context of pregnancy. Generally
speaking, WG is a very rare disease. In the US its
prevalence is estimated to be 3 cases per 100,000
persons while in the United Kingdom 5-year incidence
rate is reported to be 1.3 cases per 100,000 persons.
Essentially, the condition occurs equally in adult males
and females.
1
Since its original description by Wegener in
1939, the condition named after him has been further
elucidated. Wegener initially described a group of
patients with necrotizing granulomatous arteritis
involving the upper and lower respiratory tracts, sinuses,
middle ear, and nasopharynx. The mean survival of
adults with untreated WG was only 5 months, with 82%
of patients dying within the first year and 90% of patients
dying within the second year. Despite improvement with
the use of corticosteroids, the mean survival time was
increased only to 12.5 months. With the advent of
cytotoxic therapy, patient survival has markedly
improved.
6, 7
Our patient had pregnancy of third trimester with
a viable fetus. The cytotoxic agents such as
cyclophosphamide methotrexate etc have well
established D-fetal risks in humans. 1
Systemic steroids
such as methylprednisolone and prednisolone have well
established C-fetal risks in animals but such risks are
not yet studied in human subjects and hence may be
used if benefits outweigh risks to fetus.
1
We counseled
our patient and her husband regarding the benefits and
risks of combination therapy with systemic steroids and
cyclophosphamide in the context of the viable fetus.
They did not consent for the institution of the proposed
therapy and preferred to continue pregnancy.
A study comparing the classical and limited
varieties of the disease found that the groups shared
many features, particularly their requirement for
immunosuppressive therapy, since WG causes major
tissue destruction regardless of whether it is a localized
or a widespread process. At the immunopathological
level, the two groups appear to be part of a single
disease spectrum. Importantly, the non-renal WG group
may change the pattern of their disease to involve the
kidney. 3

3. Locally Advanced Wegener's Granulomatosis of Face Muhammad Saaiq
Ann. Pak. Inst. Med. Sci. 2010; 7(1): 67-69 69
In 1983, Fauci et al
8
reported a 93% complete
remission rate in 85 patients treated with prednisone
and oral cyclophosphamide. Despite all improvement in
management, the disease and its treatment related
morbidity is still profound. The best treatment options
still continue to be debated.
9
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