Necrotizing Sarcoid Granulomatosis: A Disease Not to be Forgotten
Table 1
Clinical characteristic of the two patients with necrotizing sarcoid granulomatosis. SACE: serum angiotensin-converting enzyme; ACE: angiotensin-converting enzyme; ADA: adenosine deaminase; PCR: polymerase chain reaction; BAL: bronchoalveolar lavage; AFB: acid-fastness; CT: tomography scan; EEG: electroencephalography; NMR: nuclear magnetic resonance; and FNAB: fine needle aspiration biopsy.
Case 1
Case 2
Sex and age
24-year-old female. 37 weeks pregnant
31-year-old male
Family history
Father diagnosed with discoid lupus
Brother diagnosed with sarcoidosis (pulmonary and cutaneous involvement)
Presentation
Left supraclavicular lymphadenopathy
Peripheral vertigo, weakness in right lower limb, instability, and sphincter incontinence
Physical examination
Approx. 4 × 4 cm supraclavicular tumour attached to deep planes
Bradipsychia. Right horizontal nystagmus. Paresis 4+/5 left upper limb and lower limbs. Left extensor cutaneous plantar reflex. Unstable romberg
Laboratory
No lymphopoenia. T CD4/CD8 lymphocyte ratio: 1.43. Normal SACE. Calcium/phosphorus metabolism: normal. 24 h urine calciuria slightly higher than normal (264 mg/dL). Positive Mantoux.
Discrete lymphopoenia. T CD4/CD8 lymphocyte ratio: 0.97. High SACE. Calcium/phosphorus metabolism: normal. Calciuria in urine at 24 h: normal. Positive Mantoux. Lumbar puncture: High ACE and ADA. Cultures (including fungi) and indian ink: negative. Sputum culture and mycobacterial PCR: negative. BAL and sputum samples: negative for AFB
Imaging tests
Cervical CT scan, lymphadenitis that does not suggest pyogenic origin. Chest x-ray: normal.
EEG: delta activity, more frequent on the right side. Brain NMR: meningeal uptake that extends to the cervical area. Chest CT scan: mediastinal nodules and bronchoalveolar infiltrates in both bases
Anatomical pathology
FNAB supraclavicular adenopathy: necrosis and granulomas. PCR mycobacterium tuberculosis: negative. Ganglion exeresis: chronic lymphadenitis with sarcoid granulomas (Figure 1)
Open lung biopsy: necrotizing granulomatous infiltrates. PCR mycobacterium tuberculosis: negative
