Sarcoidosis At A Glance <ul><li>Presented by  </li></ul><ul><li>Dr.Al-Hussain M. Assiri , MBBS , ArBIM  </li></ul><ul><li>...
SARCOIDOSIS <ul><li>Sarco = flesh; Eidos = like; Osis = condition </li></ul><ul><li>Multisystem disorder of unknown origin...
<ul><ul><li>Diagnosis is one of exclusion : </li></ul></ul><ul><ul><ul><li>Requires 3 criteria  </li></ul></ul></ul><ul><u...
Epidemiology <ul><li>► Exact prevalence (estimated at 10 to 20 per 100,000 population) . </li></ul><ul><li>►   Annual inci...
<ul><li>Immunology of Sarcoidosis </li></ul>
Etiology <ul><ul><ul><li>Speculative  </li></ul></ul></ul><ul><ul><ul><ul><ul><li>Familial cases – positive association wi...
 
Clinical Manifestations <ul><li>Constitutional Symptoms </li></ul><ul><ul><ul><li>found in 33% </li></ul></ul></ul><ul><ul...
<ul><li>Lungs </li></ul><ul><ul><ul><li>Foud in >90% </li></ul></ul></ul><ul><ul><ul><li>dyspnea, dry cough, chest pain (i...
 
 
 
 
 
 
 
Sarcoid – Cutaneous manifestations <ul><li>25-37% </li></ul><ul><li>Often occuras at onset of disease. </li></ul><ul><li>D...
Specific Lesions <ul><li>Maculopapular sarcoid  </li></ul><ul><li>Nodular sarcoid </li></ul><ul><li>Plaque sarcoid  </li><...
 
 
                                                      < TD>
 
 
 
 
Non-specific Skin Lesions <ul><li>Erythema nodosum </li></ul><ul><ul><ul><li>Lofgren’s Syndrome: EN + bilateral hilar aden...
 
 
 
<ul><li>Ocular </li></ul><ul><ul><ul><li>Found in 22-50% </li></ul></ul></ul><ul><ul><ul><li>Granulomatous uveitis (anteri...
<ul><li>Heart </li></ul><ul><ul><ul><li>Found in 5% - conduction disturbances; MI; pericardial disease; sudden death & cor...
<ul><li>Kidneys </li></ul><ul><ul><ul><li>Found in 4-40% </li></ul></ul></ul><ul><ul><ul><li>From hypercalcemia/hypercalci...
<ul><li>Muscular </li></ul><ul><ul><ul><li>Progressive proximal muscle weakness; myopathic pattern EMG, increased CpK. </l...
<ul><li>Rheumatologic </li></ul><ul><ul><ul><li>25-39% joint involvement; 2 forms – acute and chronic (<6 months). </li></...
 
<ul><li>Basic Sarcoidosis Work-up </li></ul><ul><ul><ul><li>History/PE </li></ul></ul></ul><ul><ul><ul><li>CBC, BUN, Cr, L...
 
Laboratory Manifestations   <ul><li>CBC </li></ul><ul><ul><ul><li>Anemia 5% </li></ul></ul></ul><ul><ul><ul><li>Leukopenia...
<ul><ul><ul><li>Cutaneous anergy  66% </li></ul></ul></ul><ul><ul><ul><li>CD4:CD8  – normal 1.8:1; low-activity sarcoid 1....
 
Prognosis of Sarcoidosis <ul><li>Highly variable, with a tendency to wax and wane, either spontaneously, or in response to...
Adverse prognostic factors <ul><li>Lupus pernio  </li></ul><ul><li>Chronic uveitis  </li></ul><ul><li>Age at onset greater...
<ul><li>Sarcoidosis Syndromes: </li></ul><ul><ul><ul><li>Lofgren’s Syndrome </li></ul></ul></ul><ul><ul><ul><ul><ul><li>Hi...
Sarcoidosis in pregnancy <ul><li>Sarcoidosis does not affect pregnancy adversely, but the disease may worsen after parturi...
<ul><li>Treatment </li></ul><ul><li>►   60% experience spontaneous resolution (83% with acute).  </li></ul><ul><li>►   An ...
ATS Guidelines <ul><li>Prednisolone 20-40 mg daily Max, 60 mg </li></ul><ul><li>For 8-12 weeks then taper down to keep pat...
Rheumatology Guidelines <ul><ul><ul><li>Prednisone 0.5 mg/kg/day; single daily dose, tapered by 5 mg q2 weeks until at 0.1...
<ul><li>Dermatology Guidelines: </li></ul><ul><li>Systemic Sarcoidosis : </li></ul><ul><ul><ul><li>Prednisone 1 mg/kg/d x ...
 
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Sarcoidosis agreat mimic

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Sarcoidosis agreat mimic

  1. 1. Sarcoidosis At A Glance <ul><li>Presented by </li></ul><ul><li>Dr.Al-Hussain M. Assiri , MBBS , ArBIM </li></ul><ul><li>SSC-Med , JBIM </li></ul><ul><li>Specialist in internal medicine &Rheumatology (ACH) </li></ul>
  2. 2. SARCOIDOSIS <ul><li>Sarco = flesh; Eidos = like; Osis = condition </li></ul><ul><li>Multisystem disorder of unknown origin characterized by the accumulation of lymphocytes and mononuclear phagocytes that induce the formation of noncaseating epithelioid granulomas with secondary derangement of normal tissue or organ anatomy and function. </li></ul><ul><li>Acute sarcoid = less than 2 years. </li></ul><ul><li>Chronic sarcoid = lasting longer than 2 years. </li></ul>
  3. 3. <ul><ul><li>Diagnosis is one of exclusion : </li></ul></ul><ul><ul><ul><li>Requires 3 criteria </li></ul></ul></ul><ul><ul><ul><ul><li>Compatible clinical or radiologic picture, or both </li></ul></ul></ul></ul><ul><ul><ul><ul><li>2) histologic evidence of non-caseating granulomas </li></ul></ul></ul></ul><ul><ul><ul><ul><li>3) Negative special stains and cultures for acid-fast bacilli, fungi and bacteria. </li></ul></ul></ul></ul>
  4. 4. Epidemiology <ul><li>► Exact prevalence (estimated at 10 to 20 per 100,000 population) . </li></ul><ul><li>► Annual incidence of sarcoidosis are not known with certainty </li></ul><ul><li>► Incidence peaks in winter and early spring. </li></ul><ul><ul><ul><li>Women greater incidence. </li></ul></ul></ul><ul><ul><ul><li>African ethnicity greater incidence. </li></ul></ul></ul><ul><ul><ul><li>More common in non-smokers. </li></ul></ul></ul>
  5. 5. <ul><li>Immunology of Sarcoidosis </li></ul>
  6. 6. Etiology <ul><ul><ul><li>Speculative </li></ul></ul></ul><ul><ul><ul><ul><ul><li>Familial cases – positive association with HLA-1, -B8, -B13, -DR3 and –B27. </li></ul></ul></ul></ul></ul><ul><ul><ul><ul><ul><li>Rare autosomal dominant granulomatous syndrome (Blau Syndrome – 16p12-q21) – lacks pulmonary involvement. </li></ul></ul></ul></ul></ul><ul><ul><ul><ul><ul><li>High rate of detection of HHV-8 in sarcoid tissue. </li></ul></ul></ul></ul></ul><ul><ul><ul><ul><ul><li>Unlikely mycobacterial (gets better with steroids; no reduction with BCG; anti-tb meds ineffective). </li></ul></ul></ul></ul></ul><ul><ul><ul><ul><ul><li>Clay, talk, pine pollen, oxalosis, beryllium can cause similar picture. </li></ul></ul></ul></ul></ul>
  7. 8. Clinical Manifestations <ul><li>Constitutional Symptoms </li></ul><ul><ul><ul><li>found in 33% </li></ul></ul></ul><ul><ul><ul><li>fever, weight loss, fatigue </li></ul></ul></ul><ul><ul><ul><li>+/- night sweats </li></ul></ul></ul>
  8. 9. <ul><li>Lungs </li></ul><ul><ul><ul><li>Foud in >90% </li></ul></ul></ul><ul><ul><ul><li>dyspnea, dry cough, chest pain (intensified after alcohol), hemoptysis, asymptomatic. </li></ul></ul></ul><ul><ul><ul><li>4 Stages: </li></ul></ul></ul><ul><ul><ul><ul><ul><li>Stage 0: normal </li></ul></ul></ul></ul></ul><ul><ul><ul><ul><ul><li>Stage I: bilateral hilar lymphadenopathy with right paratracheal adenopathy without pulmonary infiltrates. </li></ul></ul></ul></ul></ul><ul><ul><ul><ul><ul><li>Stage II: bilateral hilar lymphadenopathy with pulmonary infiltrates. </li></ul></ul></ul></ul></ul><ul><ul><ul><ul><ul><li>Stage III: pulmonary infiltrates without hilar adenopathy. </li></ul></ul></ul></ul></ul><ul><ul><ul><ul><ul><li>Stage IV: end-stage fibrosis, bullae and honey-combing. </li></ul></ul></ul></ul></ul>
  9. 17. Sarcoid – Cutaneous manifestations <ul><li>25-37% </li></ul><ul><li>Often occuras at onset of disease. </li></ul><ul><li>Does not show correlation with extent of disease in general. </li></ul><ul><li>Skin lesions – more likely to have lymphadenopathy & hepatosplenomegaly than if no skin lesions. </li></ul><ul><li>May portend prognosis. </li></ul><ul><li>Classified as: </li></ul><ul><ul><ul><li>Specific (lesions contain granulomas) </li></ul></ul></ul><ul><ul><ul><li>Non-specific (lesions are reactive processes and do not </li></ul></ul></ul><ul><ul><ul><li>contain granulomas). </li></ul></ul></ul>
  10. 18. Specific Lesions <ul><li>Maculopapular sarcoid </li></ul><ul><li>Nodular sarcoid </li></ul><ul><li>Plaque sarcoid </li></ul><ul><li>Darier-Roussy Nodules (Subcutaneous) </li></ul><ul><li>Scar sarcoid </li></ul><ul><li>Lupus pernio </li></ul><ul><li>Acquired ichthyosis </li></ul><ul><li>Ulcerative sarcoid </li></ul><ul><li>Rare variants: </li></ul><ul><ul><ul><ul><ul><li>Psoriasiform, Hypopigmented, Verrucous, folliculitis, Eruptive, Lichenoid, Erythrodermic, Cicatricial alopecia, Erythematous plaques of palms and soles, Nodular fingertip lesions, Unilateral lower extremity edema, granulomatous cheilitis, palmar erythema, morpheaform, angiolupoid, perforating, lupus-erythematosus-like, umbilicated, rosacea-like syndrome. </li></ul></ul></ul></ul></ul>
  11. 21.                                                       < TD>
  12. 26. Non-specific Skin Lesions <ul><li>Erythema nodosum </li></ul><ul><ul><ul><li>Lofgren’s Syndrome: EN + bilateral hilar adenopathy +/- migratory polyarthritis, fever and iritis. Good prognosis (83% remit in 2 years). </li></ul></ul></ul><ul><li>Calcifications </li></ul><ul><li>Prurigo </li></ul><ul><li>Erythema multiforme </li></ul><ul><li>Clubbing </li></ul><ul><li>Onychodystrophy +/- underlying bone cysts </li></ul><ul><li>Subungual hyperkeratosis/onycholysis. </li></ul>
  13. 30. <ul><li>Ocular </li></ul><ul><ul><ul><li>Found in 22-50% </li></ul></ul></ul><ul><ul><ul><li>Granulomatous uveitis (anterior (80%)>posterior) </li></ul></ul></ul><ul><ul><ul><li>Uveitis – acute or chronic (adhesions) </li></ul></ul></ul><ul><ul><ul><li>Keratoconjunctivitis, retinal hemorrhages, band keratopathy, proptosis. </li></ul></ul></ul><ul><li>Peripheral Lymphadenopathy </li></ul><ul><ul><ul><li>Found in 75% </li></ul></ul></ul><ul><ul><ul><li>Cervical, axillary, epitrochlear and inguinal </li></ul></ul></ul>
  14. 31. <ul><li>Heart </li></ul><ul><ul><ul><li>Found in 5% - conduction disturbances; MI; pericardial disease; sudden death & cor pulmonale. </li></ul></ul></ul><ul><li>Liver </li></ul><ul><ul><ul><li>20% hepatomegaly (autopsy granulomas 63-87%) </li></ul></ul></ul><ul><ul><ul><li>Usually asymptomatic </li></ul></ul></ul><ul><li>Spleen </li></ul><ul><ul><ul><li>15% splenomegaly; usually silent (50% on autopsy) </li></ul></ul></ul>
  15. 32. <ul><li>Kidneys </li></ul><ul><ul><ul><li>Found in 4-40% </li></ul></ul></ul><ul><ul><ul><li>From hypercalcemia/hypercalciuria; granulomatous infiltration of the renal parenchyma, glomerular disease or renal arteritis secondary to granulomas. </li></ul></ul></ul><ul><li>Salivary Glands </li></ul><ul><ul><ul><li>found in 6% parotidomegaly; subclinical 50% (but usually only with hilar adenopathy); sarcoidal ranula; tongue, tonsils. </li></ul></ul></ul><ul><li>Upper Respiratory Tract </li></ul><ul><ul><ul><li>found in 5-20% </li></ul></ul></ul><ul><ul><ul><li>Nasal granulomas. </li></ul></ul></ul><ul><ul><ul><li>Laryngeal involvement 5%. </li></ul></ul></ul>
  16. 33. <ul><li>Muscular </li></ul><ul><ul><ul><li>Progressive proximal muscle weakness; myopathic pattern EMG, increased CpK. </li></ul></ul></ul><ul><li>Neurological </li></ul><ul><ul><ul><li>found in 5% </li></ul></ul></ul><ul><ul><ul><li>Mononeuritis multiplex, Guillian-Barre, pure motor, pure sensory polyneuropathies, meningitis, seizure, arachnoiditis, myasthenia gravis-like symptoms, myelopathy. </li></ul></ul></ul><ul><ul><ul><li>Uhthoff phenomenon reported (visual loss after exposure to heat). </li></ul></ul></ul><ul><ul><ul><li>CNVII > but any cranial nerve. </li></ul></ul></ul><ul><li>Endocrine </li></ul><ul><ul><ul><li>Pituitary & hypothalamus – diabetes,. </li></ul></ul></ul><ul><ul><ul><li>Multinodular goiter and hyperthyroidism </li></ul></ul></ul><ul><ul><ul><li>May see elevated prolactin. </li></ul></ul></ul><ul><ul><ul><li>Intermittent hypercalcemia – 17-19%. </li></ul></ul></ul>
  17. 34. <ul><li>Rheumatologic </li></ul><ul><ul><ul><li>25-39% joint involvement; 2 forms – acute and chronic (<6 months). </li></ul></ul></ul><ul><ul><ul><li>May precede other manifestations by years </li></ul></ul></ul><ul><ul><ul><li>Knees > ankles > elbows > wrists > small joints of hands (typically 2-6 joints). </li></ul></ul></ul><ul><ul><ul><li>Periarticular swelling > effusions </li></ul></ul></ul><ul><ul><ul><li>Synovial fluid often non-inflammatory </li></ul></ul></ul><ul><ul><ul><li>Tenosynovitis and heel pain may occur </li></ul></ul></ul><ul><ul><ul><li>Chronic: </li></ul></ul></ul><ul><ul><ul><ul><ul><li>knees, PIPs (associated with chronic cutaneous sarcoid lesions). </li></ul></ul></ul></ul></ul><ul><ul><ul><ul><ul><li>transient or chronic </li></ul></ul></ul></ul></ul><ul><ul><ul><ul><ul><li>dactylitis. </li></ul></ul></ul></ul></ul><ul><ul><ul><ul><ul><li>Cystic lytic lesions often noted in middle & distal phalanges of hands; trabecular changes that give the bone a honeycomb appearance; always associated with chronic skin lesions. </li></ul></ul></ul></ul></ul>
  18. 36. <ul><li>Basic Sarcoidosis Work-up </li></ul><ul><ul><ul><li>History/PE </li></ul></ul></ul><ul><ul><ul><li>CBC, BUN, Cr, LFTs, electrolytes, Ca. </li></ul></ul></ul><ul><ul><ul><li>Urinalysis </li></ul></ul></ul><ul><ul><ul><li>ACE levels </li></ul></ul></ul><ul><ul><ul><li>CXR </li></ul></ul></ul><ul><ul><ul><li>PFTs (spirometry, volumes, diffusion measurements) </li></ul></ul></ul><ul><ul><ul><li>Tuberculin test with anergy panel. </li></ul></ul></ul><ul><ul><ul><li>Ophthalmologic examination with both slit-lamp and fundus. </li></ul></ul></ul><ul><ul><ul><li>Histology. </li></ul></ul></ul>
  19. 38. Laboratory Manifestations <ul><li>CBC </li></ul><ul><ul><ul><li>Anemia 5% </li></ul></ul></ul><ul><ul><ul><li>Leukopenia – reflects bone marrow involvement – 28% </li></ul></ul></ul><ul><ul><ul><li>Eosiniphilia 24-34% </li></ul></ul></ul><ul><ul><ul><li> ESR 66%; Elevated haptoglobin 40%. </li></ul></ul></ul><ul><ul><ul><li>Hypercalcemia (increased intestinal Ca absorption due to production of (3H)1,25(OH) 2 D 3 – 19% </li></ul></ul></ul><ul><ul><ul><li> ACE 60% (false + DM, alcoholic liver disease, Hep C, tuberculosis, leprosy, lymphoma, hyperthyroidism, Whipple’s, PBC, silicosis, histoplasmosis, berylliosis, Gaucher’s);  serum lysozyme, B 2 -microglobulins. </li></ul></ul></ul>
  20. 39. <ul><ul><ul><li>Cutaneous anergy 66% </li></ul></ul></ul><ul><ul><ul><li>CD4:CD8 – normal 1.8:1; low-activity sarcoid 1.4:1; high-intensity alveolitis 0.8:1. </li></ul></ul></ul><ul><ul><ul><li>Hypergammaglobulinemia 50% </li></ul></ul></ul><ul><ul><ul><li>Bronchoalveolar lavage – fluid lymphocyte count >7% lymphocytes; CD4/CD8 >3.5. </li></ul></ul></ul><ul><ul><ul><li>67 Gallium scan may demonstrate panda or lambda signs (from parotid & lacrimal gland granuloma gallium uptake [panda] and bilateral hilar lymph node involvement [lambda]). </li></ul></ul></ul><ul><ul><ul><li>Kviem-Siltzbach test (90% with hilar adenopathy). </li></ul></ul></ul>
  21. 41. Prognosis of Sarcoidosis <ul><li>Highly variable, with a tendency to wax and wane, either spontaneously, or in response to therapy </li></ul><ul><li>Spontaneous remissions occur in nearly 2/3 of patients, but the course is chronic or progressive in 10 to 30 % </li></ul><ul><li>Serious extrapulmonary involvement (eg, cardiac, central nervous system, hepatic) occurs in 4 to 7 % of patients with sarcoidosis at presentation </li></ul><ul><li>the incidence is higher as the disease evolves </li></ul>
  22. 42. Adverse prognostic factors <ul><li>Lupus pernio </li></ul><ul><li>Chronic uveitis </li></ul><ul><li>Age at onset greater than 40 years </li></ul><ul><li>Chronic hypercalcemia </li></ul><ul><li>Nephrocalcinosis </li></ul><ul><li>Black race </li></ul><ul><li>Progressive pulmonary sarcoidosis </li></ul><ul><li>Nasal mucosal involvement </li></ul><ul><li>Cystic bone lesions </li></ul><ul><li>Neurosarcoidosis </li></ul><ul><li>Myocardial involvement </li></ul><ul><li>Chronic respiratory insufficiency </li></ul>
  23. 43. <ul><li>Sarcoidosis Syndromes: </li></ul><ul><ul><ul><li>Lofgren’s Syndrome </li></ul></ul></ul><ul><ul><ul><ul><ul><li>Hilar lymphadenopathy, erythema nodosum, migratory polyarthritis, fever, iritis. </li></ul></ul></ul></ul></ul><ul><ul><ul><li>Darier-Roussy Type </li></ul></ul></ul><ul><ul><ul><ul><ul><li>Presence of subcutaneous non-painful nodules. </li></ul></ul></ul></ul></ul><ul><ul><ul><li>Heerfordt-Waldenstrom Syndrome </li></ul></ul></ul><ul><ul><ul><ul><ul><li>Fever, parotid enlargement, anterior uveitis, facial nerve palsy (can get lethargy, papilledema, meningism) </li></ul></ul></ul></ul></ul><ul><ul><ul><li>Mikulicz’s Syndrome </li></ul></ul></ul><ul><ul><ul><ul><ul><li>Bilateral sarcoidosis of the parotid, submandibular, sublingual and lacrimal glands. </li></ul></ul></ul></ul></ul>
  24. 44. Sarcoidosis in pregnancy <ul><li>Sarcoidosis does not affect pregnancy adversely, but the disease may worsen after parturition; therefore, a chest roentgenogram should be obtained within 6 months of delivery. </li></ul><ul><li>The incidence of spontaneous abortion, miscarriage, and congenital fetal abnormalities for patients with sarcoidosis is no different from that found in mothers without sarcoidosis . </li></ul>
  25. 45. <ul><li>Treatment </li></ul><ul><li>► 60% experience spontaneous resolution (83% with acute). </li></ul><ul><li>► An additional 10-20% have resolution with steroids. Major goal : prevent fibrosis . </li></ul><ul><li>Steroids first line : </li></ul><ul><ul><ul><ul><li>Help prevent fibrosis. </li></ul></ul></ul></ul><ul><ul><ul><ul><ul><li>Scarring cutaneous lesions </li></ul></ul></ul></ul></ul><ul><ul><ul><ul><ul><li>Hypercalcemia </li></ul></ul></ul></ul></ul><ul><ul><ul><ul><ul><li>Severe lung disease </li></ul></ul></ul></ul></ul><ul><ul><ul><ul><ul><li>Liver disease </li></ul></ul></ul></ul></ul><ul><ul><ul><ul><ul><li>Cardiac inflammation </li></ul></ul></ul></ul></ul><ul><ul><ul><ul><ul><li>Posterior uveitis </li></ul></ul></ul></ul></ul><ul><ul><ul><ul><ul><li>Neurosarcoidosis </li></ul></ul></ul></ul></ul><ul><ul><ul><ul><ul><li>Severe sarcoidosis of other organs </li></ul></ul></ul></ul></ul>
  26. 46. ATS Guidelines <ul><li>Prednisolone 20-40 mg daily Max, 60 mg </li></ul><ul><li>For 8-12 weeks then taper down to keep patient on 5-10 mg and contiue for 1 year </li></ul>
  27. 47. Rheumatology Guidelines <ul><ul><ul><li>Prednisone 0.5 mg/kg/day; single daily dose, tapered by 5 mg q2 weeks until at 0.15 mg/kg/day, maintained for 4-8 months, then tapered off completely. Follow PFTs. </li></ul></ul></ul><ul><ul><ul><li>Steroid sparing: Methotrexate. </li></ul></ul></ul><ul><ul><ul><li>Arthritis: colchicine, salicylates. </li></ul></ul></ul>
  28. 48. <ul><li>Dermatology Guidelines: </li></ul><ul><li>Systemic Sarcoidosis : </li></ul><ul><ul><ul><li>Prednisone 1 mg/kg/d x 4-6 weeks followed by a slow taper over 2-3 months. </li></ul></ul></ul><ul><ul><ul><li>Hydroxychloroquine 2-3 mg/kg/day x 12 weeks (30-50% success). </li></ul></ul></ul><ul><ul><ul><li>MTX 15 mg/week in 3 divided doses at 12 hour intervals x 9-11 months (94% success). </li></ul></ul></ul><ul><ul><ul><li>Thalidomide (TNF blocker) </li></ul></ul></ul><ul><ul><ul><li>Infliximab (Ab against TNF- ά ); Etanercept (receptor for TNF). </li></ul></ul></ul><ul><ul><ul><li>Cutaneous Sarcoidosis : </li></ul></ul></ul><ul><ul><ul><li>Superpotent topical corticosteroids (+/- occlusion). </li></ul></ul></ul><ul><ul><ul><li>Intralesional triamcinolone qmonthly </li></ul></ul></ul><ul><ul><ul><li>Intralesional chloroquine (50 mg/mL monthly) </li></ul></ul></ul><ul><ul><ul><li>Minocycline 200 mg qd; doxycycline 100 mg bid </li></ul></ul></ul><ul><ul><ul><li>Carbon dioxide laser </li></ul></ul></ul><ul><ul><ul><li>Pulse dye laser </li></ul></ul></ul><ul><ul><ul><li>Allopurinol 100-300 mg qd for 4 weeks </li></ul></ul></ul><ul><ul><ul><li>Thalidomide 200 mg/d x 2 weeks followed by 100 mg/d x 11 weeks, then 100 mg every other day. </li></ul></ul></ul><ul><ul><ul><li>PUVA </li></ul></ul></ul><ul><ul><ul><li>Oral isotretinoin 1 mg/kg/day x 8 months. </li></ul></ul></ul><ul><ul><ul><li>Systemic steroids, hydroxychloroquine, MTX. </li></ul></ul></ul>
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