RADIOLOGIC
IMAGES IN DERMATOLOGY
Sarcoidosis. Part II: Extrapulmonary and systemic manifestations Scott D. Roberts, MD,a Ginat W. Mirowski, DMD, MD,b,c David Wilkes, MD,a Paul Y. Kwo, MD,d and Kenneth S. Knox, MDa Indianapolis, Indiana
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arcoidosis is a systemic disease of unknown etiology characterized by noncaseating granulomatous inflammation. Sarcoidosis was first described by Jonathan Hutchinson in 1869. Although known to affect any organ system, the chest, the eyes, and the skin are most commonly affected. Differences in incidence, severity, and pattern of organ involvement are based on ethnic background. The clinical course is progressive, though remissions and relapses do occur.
Systemic manifestations Constitutional symptoms (20%-35%) Cytokine mediated Fever more common early in disease Weight loss, myalgias, and night sweats Lung (> 90%) Cough, dyspnea most common Chest pain can mimic cardiac angina Pulmonary functions with decreased diffusing capacity, mild obstruction early; restrictive pattern in advanced disease Ocular (20%-80%) Anterior/posterior/pan uveitis (30%-65%) Conjunctival involvement (15%) Lacrimal gland involvement (\10%) may be unilateral or bilateral Salivary gland (6%-10%) Commonly seen when lacrimal glands are involved (Mikulicz’s syndrome) From the Pulmonary Divisiona and the Departments of Dermatologyb and Gastroenterology/Hepatology,d at Indiana University School of Medicine, and the Department of Oral Pathology, Medicine, and Radiology at Indiana University School of Dentistry.c Funding sources: None. Conflicts of interest: None identified. Reprint requests: Ginat W. Mirowski, DMD, MD, Department of Dermatology, 550 N. University Blvd., Suite 3240, Indianapolis, IN 46202-6267. E-mail:
[email protected]. J Am Acad Dermatol 2004;51:628-30. 0190-9622/$30.00 ª 2004 by the American Academy of Dermatology, Inc. doi:10.1016/j.jaad.2004.04.006
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Parotid swelling may be associated with uveitis and fever (Uveoparotid fever or Heerfordt’s syndrome). Usually lasts 2-6 months and is associated with facial nerve palsy Neurologic (2%-7%) Central and peripheral involvement. Cranial nerve palsies (Bell’s palsy, facial, or optic nerve palsies) Granulomatous basal meningitis Hypothalamic and pituitary masses Progressive multifocal leukoencephalopathy Peripheral neuropathy Cardiac (5%-20%) Conduction abnormalities Cardiomyopathy Sudden death Liver (20%) Infiltrative disease in the liver suggested by elevated serum alkaline phosphatase, hypercholesterolemia Granulomas seen adjacent to portal triads Cirrhosis Portal hypertension Spleen (50%-80%) Often asymptomatic Splenomegaly Thrombocytopenia rare Kidney (10%-20%) Renal granulomas, renal insufficiency rare Nephrolithiasis Hypercalcemia Musculoskeletal (20%-35% ) Acute polyarthritis (10%-40%) Most common in African Americans and females Muscle granulomas and proximal myopathy
Radiologic pearls (Figs 1-6) Abdomen Spleen can appear as homogenous organomegaly or multiple, asymptomatic, low-attenuation nodules (Fig 1)
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Fig 1. A, Abdominal CT scan in patient with stage 1 sarcoid illustrating multiple lowattenuation lesions in the spleen (white arrow). B, Follow up CT after 4 months of steroid therapy with near complete resolution of the splenic lesions.
Fig 2. Hand radiograph demonstrating multiple cystic bone lesions and associated soft tissue swelling (white arrows) in a patient with chronic, untreated sarcoid presenting with lupus pernio and bilateral hand pain and swelling.
Fig 3. Thallium201 myocardial scan done in a patient with stage 2 sarcoidosis experiencing frequent palpitations. A, Image at rest shows diminished perfusion in the septum at rest (white circle). B, After exertion, septal perfusion improves (‘‘reverse distribution’’).
Fig 4. Head MRI in a 38-year-old African American male with Stage 1 sarcoidosis presenting with headache and confusion. Cerebral spinal fluid sampling revealed elevated lymphocytes, protein, and angiotensin converting enzyme (ACE) level. A, Head MRI showed basilar enhancement in T1 weighted images after contrast (white arrow). B, There is a very subtle area of associated parenchymal edema on the flare images (white arrow).
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Fig 5. A, MRI in a person with Stage 1 sarcoidosis and progressive diplopia and headache shows a large left retro-orbital mass (white arrow). B, Follow-up after 5 months of therapy shows complete resolution of the mass.
Splenomegaly suggests portal hypertension due hepatic sarcoid Hepatomegaly or multiple, low attenuation nodules (mimics malignancy) Abdominal lymphadenopathy Musculoskeletal Most commonly involves small bones of the hands or feet (phalanges) Small, lytic ‘‘punched out’’ cortical lesions are typical (Fig 2) Osteopenia is common Cardiac Cardiomegaly, pulmonary edema on plain radiograph Thallium201 imaging with segmental areas of decreased uptake which decrease with exercise (‘‘reverse distribution’’) (Fig 3) Neurologic Computerized tomography (CT) with contrast Multiple, focal, hyperdense enhancing lesions with edema
Magnetic resonance imaging (MRI) with gadolinium enhancement (superior to CT) Nonenhancing periventricular white matter lesions Regions of meningeal enhancement, typically basilar (Fig 4) mass lesion (Fig 5)
BIBLIOGRAPHY Baughman RP, Lower EE, du Bois RM. Sarcoidosis. Lancet 2003;361: 1111-8. English JC 3rd, Patel PJ, Greer KE. Sarcoidosis J Am Acad Dermatol 2001;44:725-43. Lynch JP, Sharma O, Baughman RP. Extrapulmonary sarcoidosis. Semin Respir Infect 1998;13:229-54. Man˜a´ J. Magnetic resonance imaging and nuclear imaging in sarcoidosis. Curr Opin Pulm Med 2002;8:457-63. Scott GC, Berman JM, Higgins JL, Jr. CT patterns of nodular hepatic and splenic sarcoidosis: a review of the literature. J Comp Assist Tomog 1997;21:369-72. Wilcox A, Bharadwaj P, Sharma OP. Bone sarcoidosis. Curr Opin Rheumatol 2000;12:321-30.
