Cutaneous Sarcoidosis, Sarcoidosis, Approach to cutaneous sarcoidosis, Management of cutaneous sarcoidosis

1. Sarcoidosis
Presenter : Sanjay Singh

2. Introduction
 Sarcoidosis is a multisystem disorder of unknown origin, characterized by the
accumulation of noncaseating epithelioid granulomas
 Also known as Boeck’s disease and “Mortimer’s malady “
 Sarcoidosis is presently considered a great dermatologic masquerader and its
incidence is on increase

3. History of Sarcoidosis
 Earliest description of sarcoid given by Besnier in 1889 – described as Lupus Pernio
 Tenneson gave first histopathological description in 1892
 Boeck introduced term “Sarcoid” in 1899 and concept that disease involves both
skin and internal organs
 In Greek sarco means “flesh,” eidos means “like,” and osis means “condition” -
sarcoidosis means a flesh like condition

4. Epidemiology
 Sarcoidosis affects all races, both sexes, and all ages
 Peaks between the ages of 25 and 35 years; a second peak occurs in women aged 45
to 65 years
 Sex ratio, M:F :: 1.5:1
 US : 10 - 40 cases per 1 lakh
 Scandinavia : 50-60 cases/1 lakh

5.  African Americans have more severe and rapidly progressive disease course
 Chronic skin lesions are also more common in African Americans

6. INDIAN SCENARIO
 True burden of sarcoidosis in India is not clearly known
 Due to high prevalence of TB in India and also resemblance in clinicoradiological features
 All ethnic groups in Indian sub-continent are affected by sarcoidosis
 M > F
 Majority of them present in their 4th or 5th decade of life

7. • Cutaneous involvement occurs in about 11 to 34 percent of patients with sarcoidosis
Sarcoidosis in India: Not so rare.
Sharma SK, Mohan A. JIACM 2004;5:12–21.

8. Etiopathogenesis
 Etiopathogenesis of sarcoidosis is not completely known
 Granulomatous disease caused by hyperactivity of CD4 +T cells
 Induced by exposure to infectious agent or environmental substance in genetically
predisposed individuals

9. Etiologic agents proposed to be associated with sarcoidosis
Environmental and occupational Infectious
 Mildew
 Mold
 Insecticides
 Combustible wood
 Firefighting
 Building materials
 Industrial organic dusts
 Mycobacteria
 Propionibacterium acnes
 Viruses (herpes, Coxsackie B, CMV, retrovirus,
and Epstein-Barr, HHV-8, Hepatitis-C)
 Borrelia burgdorferi
 Mycoplasma
 Chlamydia
 Yersinia

11. Genetic predisposition
• Familial clustering is reported in sarcoidosis (10% probability in sibling )
Consistent Human Leukocyte Antigen Associations
HLA Allele Association
B8 Susceptibility
DQB1*0201 Protection, good prognosis, Lofgren’s syndrome
DRB1*0301 Acute onset, good prognosis, Lofgren’s syndrome
DRB1*04 Protection
DRB1*1101 Susceptibility in Caucasians and African Americans
DRB3*0101 Susceptibility, disease progression in Caucasians

12. Genetic predisposition
 Angiotensin Converting Enzyme (ACE) gene polymorphism might play a role
 Presence of GLU residue at position 69 of HLA-DPB1 is also implicated
 Expression of the acute phase reactant genes ORM1 (orosomucoid) and HP1
(haptoglobin) is also increased in sarcoidosis

13. Clinical Features

14. CUTANEOUS DISEASE
Based on Histopathology
Specific Non-specific

15. Specific Forms
Frequent Types Less frequent Types Specific location
Papular
Maculopapular
Plaque
Annular
Lupus pernio
Subcutaneous nodules
Scar
Angiolupoid
Hypopigmented
Lichenoid
Ulcerative
Atrophic
Psoriasiform
Verrucous
Necrobiosis lipoidica-like
lesions
Ichthyosiform
Erythrodermic
Morpheaform
Polymorphous
Photodistributed
Tumoral
Oral cavity
Scalp
Nail
Genital

16. Papular sarcoidosis
• Often present on face, especially around the eyelids and nasolabial folds
• Presents with numerous nonscaly skin-coloured, yellow-brown, red-brown, violaceous or
Hypopigmented 1 to 10 mm papules
• Coalescence of lesions may lead to formation of annular or non-annular plaques
• Associated with favourable disease prognosis, and lesions usually resolve without
significant scarring
• Sometimes, upon resolution, faintly discolored, occasionally atrophic macules develops

17. (i) Multiple dusky red papules over cheeck, nasal and chin area (ii) Over upper back

18. Papular sarcoidosis of the knees
 Recently described
 Papules in linear array
 Considered a transitional form between
papular and scar sarcoidosis
 Associated with Erythema nodosum
 Good prognosis

19. Maculopapular Sarcoidosis
• Most commonly involves neck, trunk, extremities and mucous membranes
• Commonly associated with acute organ involvement
• Sometimes transient and appear to herald onset of disease
• Sign of good prognosis, in most cases systemic disease is inactive within <2 years

20. Plaque Sarcoidosis
• Plaque sarcoidosis have a similar frequency to papules
• More commonly develops on back, buttocks, face, and extensor surfaces of extremities
• Consist of one or multiple round or oval infiltrated patches, brownish red in colour, and
may be due to a confluence of papules
• Larger than 5 mm in diameter and tend to be thicker and more indurated than papules

21.  Lesions are persistent
 Commonly associated with chronic forms of sarcoidosis
 Plaques tend to recur and on resolution frequently leave permanent scarring

22. Plaque Sarcoidosis Over lower back

23. Annular sarcoidosis
 Circinate or annular papules and/or plaques
predominate mainly on forehead, face and
neck
 Central area may become depigmented and
scarred
 Ulceration is rare

24. Lupus pernio
 Most characteristic cutaneous lesion of sarcoidosis
 Hallmark of chronic fibrotic disease
 More commonly seen in black women and west indian with long standing sarcoidosis
 Chronic, violaceous to telangiectatic, induration, predominantly on nose and cheeks

25.  Lesions enlarge and become confluent to form progressively disfiguring nodular plaques on
nose and adjacent cheeks
 Can involve upper respiratory tract and cause nasal ulceration, obstruction, and perforation
of nasal septum
 Rarely involve dorsal hands, finger and toes, and lytic and cystic lesions in underlying bones
 Commonly coexists with other cutaneous involvement, particularly with plaques

26. Perthes Jungling disease
• Lytic and cystic bone lesions in hands and feet underlying lesions of lupus pernio
• When terminal phalanx is affected the nail may be dystrophic
• Drumstick dactylitis : A severe form with bulbous swelling of fingertips

27. Perthes Jungling disease : Osteolysis of Distal Phalanyx (index finger)

28.  Lupus pernio usually follows a very chronic course
 Frequent association with systemic involvement
Mutilating sarcoidosis
 Severe form of lupus pernio
 Large centrofacial tumors/plaques extending into oral and upper respiratory tissue

29. (i) Lupus Pernio (nodular type) (ii) Mutilating lesion with extension in nasal muosa

30. Subcutaneous sarcoidosis
 Also known as Darier-Roussy sarcoidosis
 Appears as non-tender, firm, mobile, subcutaneous nodules 0.5–2cm in diameter
 1 to 100 in number, sometimes appearing in clusters, and arise deep in the dermis
and subcutaneous tissue of extremities and trunk
 More common on forearms, where they tend to coalesce to form linear band

31.  Lower extremity : Differentiated from erythema nodosum by absence of tenderness
and inflammation
 Often associated with stage I on chest radiograph, along with other non-severe systemic
findings of disease

32. Multiple Skin coloured nodules in linear array over forearm

33. Scar sarcoidosis
 Characterized by infiltration of noncaseating sarcoidal granulomas in surgical scars, tattoos, skin
piercings, and other sites of trauma
 Difficult to clinically distinguish from a granulomatous foreign body reaction in a scar
 Tends to persist according to activity of systemic sarcoidosis, and usually resolves slowly and
spontaneously
 New scar infiltration in patients with sarcoidosis in remission suggests a reactivation of disease

34.  Old scars should always be examined when sarcoidosis is suspected
 Controversial reports have suggested an increased incidence of systemic involvement
while others have reported isolated cutaneous disease

36. Angiolupoid sarcoidosis
 Variant of plaque sarcoidosis characterized by the presence of prominent large telangiectasias
 Lesions are orange-red or reddish-brown in colour and have a more livid hue than other forms
 Usually presents as a single raised plaque on the bridge of the nose, central face, ears or scalp
 Little tendency to spontaneous resolution
 Easily mistaken for rosacea

37. Erythematous to violaceous plaque over nose with prominent telangiectesia

38. Hypopigmented sarcoidosis
• Affects almost exclusively dark-skinned persons of African descent
• Lesions manifest as hypopigmented, well demarcated, round to oval patches located
mainly on extremities
• Fried egg appearance : Erythematous papules can be found in the centre of some
lesions, leading to an appearance resembling a fried egg
• Histopathology : Interface dermatitis

39. Hypopigmented macules over extremities with central erythematous plaque (fried egg appearance

40. Lichenoid sarcoidosis
 Multiple 1 to 3 mm, erythematous or violaceous, slightly scaling maculopapules
involving an extensive area of the skin
 Occur singly or in groups, especially localized on the trunk, limbs, and face
 Wickham striae are absent
 Lichenoid lesions have been particularly reported in young children
 Specific triad of skin, joint, and eye disease
 Pulmonary disease is not usually found

41. Infiltrated, nonfollicular, lichenoid papules over knee Dermoscopy : Absence of white Wickham striae

42. Dermoscopy for discriminating between lichenoid sarcoidosis and lichen planus
Vazquez-Lopez F, Palacios-Garcia L, Gomez-Diez S, et al. Arch Dermatol. 2011;147(9):1130.

43. Ulcerative Sarcoidosis
• Generally develop in papulonodular lesions, some appear de novo
• Ulcer can develop in psoriasiform, atrophic, lymphedematous, erythrodermic and
verrucous lesions
• Located primarily on lower legs and tend to heal with scarring
• Trauma can be inciting factor in other sarcoidosis lesions

44. Ulcerative Sarcoidosis Venous ulcer
Edema - +
Hyperpigmentation in
surrounding skin
- +
Granuloma + -

46. Ulcerative sarcoidosis. Case report and review of the literature
Albertini JG, Tyler W, Miller OF. Arch Dermatol 1997;133(2):215-9.
• Total no. of Cases : 35
• Leg ulcer was present in 29 patients
• 11 patients presented with ulcers as initial sign of sarcoidosis
• Various cutaneous sarcoid lesions were presenting complaint in 15 others
• Ulcers generally developed in papulonodular lesions

47. Clinical Feature No. of Cases
Respiratory symptoms 12
Ocular disease 9
Splenomegaly 8
Hepatomegaly 7
Lymphadenopathy 7
Bone cysts 6
Arthropathy 2

48. Psoriasiform sarcoidosis
• Presents with well-demarcated, erythematous, scaly plaques that may be clinically
indistinguishable from psoriasis
• Involves extensor surface of extremities, face, scalp, back, and buttocks
• Multiple configurations, including discrete, confluent, annular, and polycyclic, have
been reported

51. Sarcoidosis and psoriasis: a case series and review of the literature exploring co-incidence vs
coincidence
Wanat KA, Schaffer A, Richardson V et al. JAMA Dermatol 2013;149(7):848-52.
• 7 patients with both sarcoidosis and psoriasis vulgaris
• 3 patients had cutaneous sarcoidosis, and one had evidence of both psoriasis and
sarcoidosis in same cutaneous specimen
• Similar pathogenesis of TH1 and TH17 in both sarcoidosis and psoriasis suggest that a
common pathway may exist and that association may be more than coincidental.

52. Verrucous sarcoidosis
• Most commonly seen on face or areas such as the groin and axillae where there is
constant friction
• Well demarcated, exophytic, hyperkeratotic plaques or discrete papillomatous, skin-
coloured papules

55. Necrobiosis lipoidica-like lesions
• Pink to violaceous plaques with depressed centres located on shins

56. Ichthyosiform sarcoidosis
• Adherent, irregular, polyclonal, dry, grey or brown
scales varying in size from 0.1cm to 1cm
• Most commonly located on lower extremities
• Biopsy : Typical sarcoidal granulomatous
inflammation with changes of ichthyosis vulgaris

57. Erythrodermic sarcoidosis
• Presence of large areas of skin with significant
erythema, induration and scaling
• Typically begins with slightly infiltrated,
erythematous to yellow-brown plaques that
subsequently coalesce over large areas
• Skip areas can be seen

58. Morpheaform Sarcoidosis
 Indurated and atrophic plaques indistinguishable
from morphea
 Predominantly located on the thighs of black
woman
 Histopathology : Epithelioid granulomas along
with dermal sclerosis is observed

59. Polymorphous sarcoidosis
• Presence of different types of lesions, both specific and nonspecific, in same patient
• Usually associated with multisystem disease
Photodistributed sarcoidosis
• Rare form of sunlight-induced papular sarcoidosis with negative phototesting

60. Sarcoidosis of oral cavity
 Usually consist of diffuse enlargement at submucous level or a firm, nodular lesion,
with normal overlying mucosa
 Papules, superficial ulcerations and strawberry gums have also been described
 Usually symptomless
 Most commonly seen on buccal mucosa, followed by gums, lips, floor of mouth,
tongue and palate

61. (i) Nontender, indurated mass in the right buccal submucosa with overlying intact mucosa (ii) Erythematous infiltrated gingiva

62. Scalp alopecia
 Usually scarring alopecia
 Less commonly nonscarring alopecia
 Scale is usually absent, although follicular plugging can be seen
 Late stage : Indistinguishable from pseudopelade of Brocq

64. Nail sarcoidosis
 Present as subungual hyperkeratosis, clubbing, pitting, trachyonychia, paronychia
with nail fold fissuring, pterygium, onycholysis, dactylitis, longitudinal ridging, and
discoloration of nail bed
 Nail involvement is usually a marker of chronic disease
 Often accompanied by phalangeal bone disease, which is frequently associated with
intrathoracic sarcoidosis

66. Non specific cutaneous manifestations
 Symmetric, tender, erythematous nodules and raised plaques
 Present on anterior aspect of lower extremity
 Most common non-specific lesion, develops in up to 25% of sarcoidosis cases
 Good prognostic significance (self resolving nature of disease)
Erythema nodosum

67. Lofgren Syndrome
 > 80% cases resolve spontaneously within 2 years
 HLA-DRB1 alleles affect disease prognosis in Lofgren syndrome
Erythema
nodosum
Acute
Polyarthritis
B/L Bronchohilar
Lymphadenopathy

68. Other Non specific cutaneous manifestations
 Calcinosis cutis
 Erythematous rash resembling viral exanthem or drug reaction
 Pruritus and prurigo nodularis
 EM like lesions
 Lower limb swelling

69. Childhood sarcoidosis
• Uncommon in children
• Affects both sexes equally
• Early onset <5 year and late onset ≥ 5 year
• Classic presentation is with triad of arthritis, erythema nodosum, and uveitis in <5 year group
• Older children usually present with a multisystem disease similar to adult manifestations, with
frequent hilar LAD and pulmonary infiltrations

70.  Most frequent cutaneous eruptions include soft, red to yellowish brown, or
violaceous, flat-topped papules, found most frequently on face
 If no skin lesions then lymph nodes are best for biopsy
 Spontaneous resolution more common

71. Specific cutaneous lesions in patients with systemic sarcoidosis: relationship to severity
and chronicity of disease
Marcoval J, Mañá J, Rubio M. Clin Exp Dermatol 2011;36(7):739-44.
• Total Patients : 86 pts of systemic sarcoidosis with follow up of > 2 years
• Cutaneous lesions developed before or at time of diagnosis of systemic sarcoidosis in 80.23%
of patients
• Plaque : 31
• Maculopapules : 28
• Subcutaneous : 14
• Scar : 7
• Lupus pernio : 6
• Erythema nodosum : 30

72.  Plaques and LP were associated with persistence of systemic sarcoidosis and requirement for
systemic corticosteroids
 Maculopapules and subcutaneous sarcoidosis are usually associated with EN and radiological
stage I, and indicate good prognosis

73. Cutaneous involvement in sarcoidosis: analysis of the features in 170 patients
Yanardağ H, Pamuk ON, Karayel T. Respir Med 2003;97(8):978-82.
• Total no. of patients : 516
• Cutaneous involvement : 170 (32.9%)
n % Parenchymal involvement, n %
Erythema nodosum 106 (20.5) 11 (10.4)
Skin plaques 22 (4.3) 4 (18.2)
Subcutaneous nodules 22 (4.3) 4 (18.2)
Maculopapular rash 19 (3.7) 2 (10.5)
Scar lesions 15 (2.9) 6 (40)
Lupus pernio 14 (2.7) 9 (64.3)
Psoriasiform lesions 5 (0.9) -

74. Good Prognosis Poor Prognosis No prognostic significance
Papular sarcoidosis Plaque sarcoidosis Scar sarcoidosis
Maculopapular Lupus pernio
Subcutaneous sarcoidosis Angiolupoid sarcoidosis
Childhood sarcoidosis Ichthyosiform sarcoidosis
Erythema Nodosum Verrucous sarcoidosis
Lofgren syndrome Hypopigmented
Lichenoid Sarcoidosis Nail sarcoidosis
Scalp sarcoidosis
Sarcoidal alopecia
Polymorphous sarcoidosis
Ulcerative sarcoidosis

75. Systemic manifestations
Lung manifestations (90%)
• B/L hilar lymphadenopathy
• Granulomas involve interstitial areas, bronchioles, alveoli and blood vessels
• Primary alveolitis
• Irreversible fibrosis
• Pleural effusions
• Presents with dyspnea, cough and rarely hemoptysis

76. Stage I & II
Normal X-ray Stage I Stage II

77. Stage III
Normal X-ray Stage III

78. Stage IV
Normal X-ray Stage IV

79. Upper respiratory tract lesions ( 5% to 20% )
• Can present as lupus pernio
• Granulomatous invasion of nasal and oral mucosa, larynx and pharynx, salivary
glands (sarcoidal ranula), tonsil and tongue
• Enlargement of parotid gland : 6% of patients
• Presents with nasal congestion, palatal obstruction and disfigurement

80. Ocular manifestations
(30% to 50%)
 Gritty sensation
 Conjunctivitis sicca
 Acute anterior uveitis
 Iris nodules
 Scleral plaques
 Lacrimal gland enlargement
 Chorioretinitis

81. Musculoskeletal involvement (30 - 40% )
 Manifestations include weakness, pain, tenderness, and erythema
 Bone cysts and osteolytic Lesions
 Chronic myopathy
 Muscle nodules
 Arthralgias, arthritis, fever, weight loss
 Tenosynovitis

82. Investigations

83. Histopathology
• Histopathologic hallmark : superficial and deep dermal epithelioid cell granulomas
devoid of prominent infiltrates of lymphocytes or plasma cells (“naked tubercles”)
• Central caseation is usually absent
• Fibrinoid deposition may be observed in up to 10% of cases
• Multinucleated histiocytes (“giant cells”) are usually of Langhans type, with nuclei
arranged in a peripheral arc or circular fashion

84. • Asteroid bodies : Stellate eosinophilic inclusions made up of complex lipids
• Schaumann (conchoidal) bodies : round or oval inclusions consisting of laminated
calcium oxalate; these may represent residual bodies of lysosomes
• Crystalline inclusions : Colorless, round or oval, refractile, nonlaminated inclusion
bodies composed of calcium oxalate that may represent precursors of Schaumann
bodies
• Neither finding is specific for sarcoidosis

85. 4X

86. 10X

87. Asteroid bodies Schaumann bodies

88. Granulomas containing strands of staining reticulin

89. Feature Sarcoidosis Tuberculosis
General Monomorphic Caseating
Tubercles Discrete, naked Diffuse, confluent
Epithelioid cells Large, grouped Irregular or at margin of
caseation, less than 50%
Giant cells Large and sparse Small and numerous
Inclusion bodies Frequent Occasional
Vessels Normal or dilated Fibrinoid changes
Reticulin Fine and abundant destroyed
Fibrinoid necrosis centre In vessels
Healing Hyalinization from periphery Dense collagen mesh,
retraction and calcifications

90. Ancillary diagnostic tests
• Cutaneous anergy ≈ 90%
• Kviem-Siltzbach test (90% with hilar adenopathy)
SACE – serum angiotensin levels
• Only an adjunctive investigation
• Not diagnostic, not prognostic, not useful for monitoring
• Because of false-positive rate of 10% and a false-negative rate of 40%

91. Increased Serum ACE Levels
 Leprosy
 Alcoholic liver disease (cirrhosis),
 α1-antitrypsin deficiency
 Diabetes mellitus
 Kaposi’s sarcoma/HIV
 Melkersson-Rosenthal syndrome
 Histoplasmosis
 Asbestosis
 Silicosis

92. • Gallium - 67 scan
• Bronchoalveolar lavage
• 24 hour urine calcium

93. Anergy to tuberculin in sarcoidosis is not influenced by high prevalence of tuberculin
sensitivity in the population
Gupta D, Chetty M, Kumar N et al. Sarcoidosis Vasc Diffuse Lung Dis 2003;20(1):40-5.
Group I
N = 50
Group II
N = 62
Control = 130
Tuberculin Anergy
N = 46 (92%)
1 TU tuberculin
Tuberculin Anergy
N = 55 (88.7%)
Control : 21 (16.2%)
1 TU tuberculin
Tuberculin Anergy
N = 39 (70.9%)
Tuberculin Anergy
Control : 6 (28.6%)
5 TU tuberculin

94. Assessment for systemic sarcoidosis
Recommended basic assessment for sarcoidosis in patients presenting with specific
(granulomatous) cutaneous lesions
 History (including occupational and environmental exposures)
 Physical examination
 Ophthalmological examination (slit lamp and ophthalmoscopic examination)
 Chest radiograph
 Standard haematological and biochemistry profiles (including urine and serum calcium level,
liver and renal function tests), and serum angiotensin-converting enzyme (SACE) level
 Electrocardiogram
 Pulmonary function tests (including spirometry and diffusion of carbon monoxide)
 Tuberculin skin test

95. Treatment

96. • Sarcoidosis is a self limiting disease 60% of patients with spontaneous resolution in 6-18
months
Topical therapy
• High-potency topical corticosteroids
• Intralesional triamcinolone injections
• Tacrolimus
• Cryotherapy and radiotherapy
• PUVA therapy has been successful in hypopigmented sarcoidosis and in Erythrodermic
sarcoidosis
• In certain types of cutaneous sarcoidosis, for example lupus pernio, cosmetic camouflage
is helpful

97. Indications for Systemic Treatment
1. Symptomatic pulmonary disease
2. Progressive or persistent parenchymal lung disease after 2 years
3. Posterior ocular disease or anterior disease not responding to local steroids
4. Persistent fever or weight loss
5. Liver disease with significant dysfunction or hepatosplenomegaly
6. Disfiguring skin disease or lymphadenopathy
7. Nervous system disease

98. 8. Hypercalcaemia
9. myocardial disease
10. myopathy or myositis
11. Thrombocytopenia
12. other significant organ involvement—for example, kidneys
Sarcoidosis.
Johns CJ, Scott PP, Schonfled SA. Ann Rev Med 1989; 40: 353–71.

99. American Thoracic Society/European Respiratory Society/World Association of Sarcoidosis and
Other Granulomatous Disorders.
Criteria for considering corticosteroid treatment in sarcoidosis
• Progressive symptomatic pulmonary disease
• Asymptomatic pulmonary disease with persistent infiltrates or progressive loss of lung function
• Cardiac disease
• Neurological disease
• Eye disease not responding to topical therapy
• Symptomatic hypercalcaemia
• Other symptomatic/progressive extrapulmonary disease

100. Summary of pharmacologic agents used for treatment of cutaneous sarcoidosis
Treatment Usual dose Indications Level of
evidence
Class I ultrapotent
topical corticosteroids
0.05% ointment applied twice weekly
or under occlusive dressing
Limited and discrete papules
and plaques
IIB
I/L triamcinolone 3-10 mg/mL every 3-4 wks until
resolution occurs
Limited & discrete papules,
plaques, and nodules
IIB
Oral corticosteroid Initially, 0.5-1 mg/kg/d (prednisone
equivalent); gradually taper to lowest
effective dose (often 10 mg/d) and
switch to an every other day schedule
1. Widespread, disfiguring,
chronic lesions
2. Lesions refractory to
local therapy
3. Recalcitrant LP
4. Ulcerative lesions
IIB

101. Chloroquine 250-750 mg daily; maximum dose is
3.5 mg/kg/d
Steroid-sparing agent or as
monotherapy is effective in
all lesions
Very effective for LP
IIB
Hydroxychloroquine 200-400 mg daily; maximum dose is
6.5 mg/kg/d
Same as for chloroquine IIB
Methotrexate 7.5-25 mg/wk orally, SQ or IM;
maintenance dose may be
administered biweekly
Steroid-resistant lesions
or patients unable to take
steroids; especially useful
for ulcerative sarcoidosis
IIB
Tetracycline Minocycline, 200 mg/d
Tetracycline, 1,000 mg/d
Helpful in selected cases IIB
Thalidomide 50-400 mg/d Refractory skin disease,
especially LP
IIB

102. Infliximab 3-7 mg/kg IV at 0, 2, and 6 wks (3-
10 mg/kg) and then every 6 wks
Widespread disease, severely disfiguring
lesions, and refractory lesions
IIB
Adalimumab 40 mg every 1-2 wks Widespread disease, severely disfiguring
lesions, and refractory lesions
III

103. Algorithm for treatment

104. Specific skin lesions
Cosmetically insignificant or asymptomatic
Cosmetically significant or symptomatic
No treatment indicated
Limited, mild to moderate disease
Widespread, severe
disfigurement or Lupus Pernio
Topical or I/L corticosteroids
Systemic Corticosteroids
&
Steroid Sparing Agent
Steroid sparing agent :
Anti-malarials
Or
Methotrexate
Or
MMF
Slowly taper medication
TNF-α antagonist
Slowly taper systemic corticosteroids
Maintain adjuvant therapy
Anti-malarials
Or
Methotrexate
Or
Tetracycline
Try alternative
Anti-malarials
Or
Methotrexate
Or
Tetracycline
Systemic Corticosteroids
Slowly taper systemic
corticosteroids maintain
steroid-sparing
Experimental therapy
Or
Laser Ablation
Or
Surgery
No improvement
No improvement
No improvement
Clinical response
No improvement
Systemic steroids
contraindicated
Clinical response
Clinical response
No improvement