CLINICAL HISTORY:

Signs and symptoms:  Dyspnea, cough, hemoptysis, congestion, hematuria, painful rash of legs, perinasal area, and perioral area with ulceration, difficulty walking, and weakness.

Previous Treatment:  Previous course of PO prednisone approximately two months earlier due to unspecified rash of the lower extremities.

Other information:  Past medical history of MI, tobacco use, HTN, CAD, sedentary lifestyle.

PHYSICAL EXAM:

Temp - 98˚F
BP – 90/30 arterial line
HR – Low 100’s sinus tach
RR – 20
O2 Sat – 96% non rebreather
Examination of the skin revealed palpable purpura of the bilateral lower extremities. There were several erythematous nodules with shallow ulcerations of the perinasal/oral areas.

LABORATORY TESTS:

H/H – 8.4/28
WBC - 8
U/A – positive for RBC’s, protein, sediment
BUN – 50
Creat – 2.2
CXR – CHF with patchy infiltrates
ESR and CRP - elevated
C-ANCA - positive

DERMATOHISTOPATHOLOGY:

Non-specific perivascular inflammation of small arteries and veins with necrosis and fibrin deposition of vessel walls, red blood cell extravasation, thrombi and nuclear dust. A granulomatous inflammation in the blood vessels and surrounding dermis was also noted. No atypical lymphocytes noted.

DIFFERENTIAL DIAGNOSIS:

1.   Wegener's Granulomatosis
2.   PAN
3.   Lymphomatoid Granulomatosis
4.   Churg-Strauss
5.   SLE

SCROLL DOWN FOR ANSWER AND DISCUSSION.

CORRECT DIAGNOSIS:

Wegener's Granulomatosis

DISCUSSION:

Wegener's Granulomatosis is characterized by necrotizing granulomas of upper and lower respiratory tract,
necrotizing angiitis of medium sized blood vessels,
and focal necrotizing glomerulitis. Signs and symptoms include but are not limited to rhinorrhea, sinusitis, nasal and mucosal ulcerations, fever, weight loss, malaise, and strawberry gums.

There is a 1.3:1 male to female ratio and a mean age of 40 to 50. Obstruction of nose is common and pulmonary nodules are found in 40% – 70% of patients. Ulceration and hemorrhage of pulmonary nodules is possible. Granulomas of ear and mouth can be seen in some patients and ulceration of tongue and perforating ulcers of the palate are possible.

Other findings include:
Tracheal stenosis
Cutaneous findings occur in 45% of pts
Nodules often appear in crops
Nodules are firm and slightly tender, flesh colored and later ulcerate
Necrotizing angiitis of the skin can present as petechial, purpuric, or pustular eruption
Temporal arteritis–like symptoms
Livedo reticularis is rareFocal necrotizing glomerulitis 70 - 85% of pts
Renal failure – common cause of death
WG without renal involvement has good prognosis
Lung involvement – 90%
Arthralgia – 66%
Eyes – 58%
CNS – 22%
Cardiac – 12%

Radiographic findings include:
CXR – negative in up to 20%
Nodules, airspace opacities, atelectasis, reticular interstitial opacities, adenopathy are common
Tracheal-bronchial abnormalities are rare
CT – nodules and airspace consolidation are most common
Nodules vary in size from 5mm to 10cm
Airspace disease can be bilateral and diffuse due to pulmonary hemorrhage, scattered parenchymal disease, or localized with ill defined margin and central cavitation
Interstitial abnormalities include interlobular septal thickening, parenchymal bands, and bronchial wall thickening

Histopathological findings include Leucocytoclastic vasculitis with or without granulomatous inflammation

Diagnosis:
C-ANCA is often positive but not specific for WG
Lung or kidney biopsy – lung biopsy is preferred
Clinical Criteria for diagnosis include nasal or oral inflammation, abnormal CXR, urinary sediment, and suggestive biopsy.
2 of 4 criteria is diagnostic.
88% sensitive and 92% specific for diagnosis.

TREATMENT:

Our patient was initially started on IV solumedrol and was then transitioned to cyclophosphamide.

Treatment modalities include:
Cyclophosphamide – 2mg/kg/day or pulsed 15mg/kg every second week
Prednisone
Azathioprine
Cyclosporine
MTX
Bactrim

REFERENCES:

1.Bolognia JL, Jorizzo JL, Rapini RP, et. al. (2003) Dermatology. Spain: Mosby
2.Arnold, Odom, James. (2000) Andrews Diseases of the Skin. Philadelphia, PA. Elsevier.
3.Braunwald E, Fauci AS, Kasper DL, et al. (2001) Harrison's Principles of Internal Medicine. New York. McGraw-Hill.
4. www.emedicine.com

Additional Comment:

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