eMedicine Specialties > Dermatology > Reactive & Inflammatory Dermatoses
Sarcoidosis: Treatment & Medication
Updated: Sep 8, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
The need for medical therapy varies based on the symptoms and the organ systems involved in each patient.
- Oral corticosteroids are usually the treatment of choice for patients with neurologic, cardiac, or ocular involvement not responding to topical corticosteroids; hypercalcemia; and symptomatic stage II and all stage III pulmonary disease. The usual dose is 30-40 mg of prednisone daily for 2-3 months, with a gradual taper over 1 year to 10-20 mg every other day. Patients with severe disease may need prednisone doses up to 1 mg/kg/d. As opposed to the more severe systemic involvement requiring oral therapy, Löfgren syndrome is usually an acute process, which is self-limited and resolves without immunosuppressive treatment in weeks. Symptomatic relief can be obtained using nonsteroidal anti-inflammatory drugs (NSAIDs).
- Limited, nondisfiguring cutaneous involvement may be treated with topical or intralesional corticosteroids. Intralesional injections of 2-10 mg/mL13 of triamcinolone acetonide can be used at monthly intervals. More chronic skin lesions, such as plaques or lupus pernio, require more aggressive therapy because they can lead to permanent scarring. If intralesional corticosteroids are not effective, other standard therapies include systemic corticosteroids, methotrexate, and,14,15,16 antimalarials (hydroxychloroquine17 and chloroquine) can be used.18
- Infliximab is a promising option for those with recalcitrant or disfiguring disease.13,19,20
- Other agents that have been used to treat cutaneous sarcoidosis include cyclosporine, chlorambucil,21 oral isotretinoin,22 allopurinol,23 minocycline,24 doxycycline, psoralen with UVA, infliximab,25 etanercept, adalimumab,26 thalidomide,27,28,29 leflunomide, pentoxifylline, and melatonin.30
- One case report describes remission of sarcoidosis after the patient was placed on an ACE inhibitor.31
- Radiation has also been used to treat treatment-resistant cutaneous lesions.32
- One report describes improvement in two cases using Photodynamic Therapy with methyl aminolevulinate (Metvix).
- One report describes improvement in 2 cases using photodynamic therapy with methyl aminolevulinate (Metvix).33
Surgical Care
Surgical excision of small lesions or excision of larger lesions with skin grafting can be attempted. The risk of lesion recurrence and hypertrophic and keloidal scarring does exist.
Laser surgery using carbon dioxide34 and pulsed dye laser has also been used in the treatment of disfiguring skin plaques and lupus pernio.13
Consultations
Early involvement of other clinicians, such as an ophthalmologist, an internist, and a pulmonologist, for monitoring other organ systems that may be involved is recommended.
Diet
No specific diet is recommended.
Activity
Activity is not restricted, unless the lungs are affected, in which case the patient's activity may be limited by shortness of breath.
Medication
The medications with the best evidence for treatment in cutaneous sarcoidosis all have C recommendation grade: corticosteroids (eg, topical, intralesional, oral), methotrexate, chloroquine, hydroxychloroquine, and infliximab. Case reports have described improvement with tetracyclines, isotretinoin, thalidomide, pentoxifylline, leflunomide, chlorambucil, melatonin, cyclosporin, allopurinol, and adalimumab.
Corticosteroids
These agents are effective in stopping symptoms of disease. They are usually not indicated in cutaneous disease unless the patient has chronic, disfiguring lesions (eg, plaques, lupus pernio).
Prednisone (Deltasone, Meticorten, Orasone, Sterapred)
Immunosuppressant for treatment of autoimmune disorders; may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Stabilizes lysosomal membranes and also suppresses lymphocyte and antibody production.
Adult
20-40 mg/d PO for 2-3 mo, followed by slow taper to 10 mg qod as maintenance for 1 y; single morning dose is safer for long-term use, but divided doses have more anti-inflammatory effect
Pediatric
Not established
Coadministration with estrogens may decrease clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics
Documented hypersensitivity; viral, fungal, connective tissue, or tubercular skin infections; peptic ulcer disease; hepatic dysfunction; GI disease
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Abrupt discontinuation of glucocorticoids may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur with glucocorticoid use
Cytotoxic agents
These agents can decrease the dose of corticosteroid needed to control the disease. These agents can also be used for patients refractory to or those who cannot tolerate the adverse effects of systemic corticosteroids.
Methotrexate (Folex PFS, Rheumatrex)
Antimetabolite that inhibits dihydrofolate reductase, thereby hindering DNA synthesis and cell reproduction. Subjective improvement is usually seen after 4-6 mo of therapy, and objective improvement is seen after 6 mo of therapy. Folic acid (1-2 mg/d) is recommended to decrease adverse effects.
Adult
7.5-25 mg PO qwk
Pediatric
Administer as in adults
Drugs that increase toxicity include NSAIDs, salicylates, ethanol, sulfonamides, probenecid, penicillins, cephalosporins, colchicine, barbiturates, phenytoin, dipyridamole, and retinoids; drugs that decrease effects include tetracycline and chloramphenicol
Documented hypersensitivity; alcoholism; hepatic insufficiency; documented immunodeficiency syndromes; preexisting blood dyscrasias (eg, bone marrow hypoplasia, leukopenia, thrombocytopenia, significant anemia); renal insufficiency
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Avoid using drug in patients with CrCl <20%; adjust dose of drug for renal impairment; monitor CBC counts monthly, and liver and renal function q1-3mo during therapy (monitor more frequently during initial dosing, dose adjustments, or when risk of elevated MTX levels, eg, dehydration); has toxic effects on hematologic, renal, GI, pulmonary, and neurologic systems; discontinue if significant decrease in blood counts occurs; aspirin, NSAIDs, or low-dose steroids may be administered concomitantly with MTX (possibility of increased toxicity with NSAIDs, including salicylates, has not been tested); adverse effects include mucositis, nausea, carcinogenicity, teratogenicity, pneumonitis, and pulmonary fibrosis; liver biopsy is recommended after a cumulative dose of 1.5 g
Antimalarials
These agents may have immunomodulatory effects.
Hydroxychloroquine (Plaquenil)
Inhibits chemotaxis of eosinophils, locomotion of neutrophils, and impairs complement-dependent antigen-antibody reactions.
Adult
200-400 PO mg/d
Pediatric
Not established
Serum levels increase with cimetidine; magnesium trisilicate may decrease absorption
Documented hypersensitivity; psoriasis; retinal and visual field changes attributable to 4-aminoquinolones
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Most common adverse effects are nausea and vomiting; other adverse effects include diarrhea, headache, pruritus, myopathy, neuropathy, agranulocytosis, aplastic anemia, hemolysis, and blue/black pigmentation of skin; monitor CBC count, creatinine level, and LFTs every 1-2 mo while on medication; retinal toxicity can occur (avoid in patients with a history of retinal disease); while on medication, patients need regular ophthalmologic examinations
Immunomodulatory agents
These agents regulate key factors of the immune system.
Thalidomide (Thalomid, Contergan)
Immunomodulatory agent that may suppress excessive production of TNF-alpha and may down-regulate selected cell-surface adhesion molecules involved in leukocyte migration.
Adult
50-300 mg/d PO qd with water, preferably hs and at least 1 h pc
<50 kg (110 lb): Start at low end of dose regimen
Pediatric
Not established
Darbepoetin alfa may cause increase in thrombogenic state in patients with myelodysplastic syndrome; dexamethasone may increase risk of developing toxic epidermal necrolysis; docetaxel may increase risk of venous thromboembolism; zoledronic acid may increase risk of renal dysfunction; may increase sedation of alcohol, barbiturates, chlorpromazine, and reserpine
Documented hypersensitivity; sexually active males not using latex condom (risk to fetus from semen of patients taking thalidomide unknown), pregnancy, women of childbearing potential not using 2 forms of contraception
Pregnancy
X - Contraindicated; benefit does not outweigh risk
Precautions
Perform pregnancy test 24 prior to initiating therapy (qwk during first month, followed by monthly tests in women with regular menstrual cycles or q2wk with irregular menstrual cycles); bradycardia may occur; use protective measures (eg, sunscreens, protective clothing) against exposure to sunlight or UV light (eg, tanning beds); prescribing physician must register with the "System for Thalomid Education and Prescribing Safety" (STEPS) program established by manufacturer; caution in persons with neuritis, seizure disorder, or cardiovascular disease; patients with neoplastic or various inflammatory conditions may have increased incidence of thrombotic events, including pulmonary embolism
Serious adverse effects may include sedation (avoid hazardous tasks, eg, operating a motor vehicle or dangerous machinery), leukopenia (may increase HIV viral load in HIV-seropositive patients), orthostatic hypertension, seizures, deep thrombophlebitis, fever, infections, peripheral neuropathy (caution with concomitant use of substances associated with peripheral neuropathy), drug eruption, including Stevens-Johnson syndrome/toxic epidermal necrolysis
Tumor necrosis factor inhibitors
These agents inhibit tumor necrosis factor activity.
Infliximab (Remicade)
Neutralizes cytokine TNF-alpha and inhibits it from binding to TNF-alpha receptor.
Adult
5 mg/kg as single IV infusion
Pediatric
Not established
May reduce effectiveness of live virus vaccines; hepatosplenic T-cell lymphomas have occurred in patients on concomitant treatment with infliximab and azathioprine or 6-mercaptopurine; concomitant immunosuppressive therapy increases risk of serious infections, including sepsis and pneumonia (some fatal); concomitant use of infliximab and anakinra increases risk for serious infection
Documented hypersensitivity; tuberculosis or other active significant infection; moderate-to-severe congestive heart failure
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
TNF-alpha modulates cellular immune responses; anti-TNF therapies, such as infliximab, may adversely affect normal immune responses and allow development of superinfections; more cases of lymphoma were observed in TNF-alpha blockers compared with controlled groups; may increase risk of reactivation of tuberculosis in patients with particular granulomatous infections
Not recommended in reactivation of hepatitis B virus in chronic carriers; consider risk and benefits in persons residing in areas of endemic histoplasmosis; evaluate patients for risk of tuberculosis (tuberculin skin test) prior to initiating therapy
Caution in congestive heart failure and worsening mild congestive heart failure (NYHA class I/II) and do not administer doses >5 mg/kg; autoantibody formation (may develop lupuslike syndrome); caution in hepatotoxicity; hepatotoxicity, including acute liver failure and autoimmune hepatitis resulting in fatalities, has been reported; drug eruption may occur, including serum sickness–like reactions (latter may occur upon reinstitution of infliximab therapy after extended period without therapy); caution in leukopenia, neutropenia, thrombocytopenic disorder, and pancytopenia; patients with preexisting or recent onset of CNS demyelinating or seizure disorder (potential for exacerbation); increased risk of nonmelanoma skin cancers in psoriasis patients; patients are at increased risk for infections, including progression to serious infections leading to hospitalization or death (infections have included bacterial sepsis, tuberculosis, invasive fungal and other opportunistic infections)
More on Sarcoidosis |
| Overview: Sarcoidosis |
| Differential Diagnoses & Workup: Sarcoidosis |
 Treatment & Medication: Sarcoidosis |
| Follow-up: Sarcoidosis |
| Multimedia: Sarcoidosis |
| References |
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Further Reading
Keywords
sarcoidosis, angiolupoid sarcoid, Besnier-Boeck-Schaumann disease, Boeck's sarcoid, Darier-Roussy disease, lupus pernio, multiple benign sarcoid of Boeck, Schaumann benign lymphogranulomatosis, subcutaneous sarcoid, uveoparotid fever
