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TitleAtlas of Sarcoidosis - V. Mihailovic-Vucinic, O. P. Sharma (Springer, 2005) WW
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LanguageEnglish
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Page 2

ATLAS OF
SARCOIDOSIS

Page 59

52 Atlas of Sarcoidosis

FIGURE 8.11 Maculopapular skin lesions in a patient with chronic sarcoidosis. Her chest X-ray
resembled parenchymal infiltrations in the upper lobes.6 The skin biopsy showed many epithelioid
granulomas, histiocytes, and lymphocytes.

SUBCUTANEOUS NODULES

Acute or subacute lesions in sarcoidosis is termed
Darier–Roussy (D–S) Syndrome. The lesions consist of
dermal and subcutaneous nodules. Occasionally, the
nodules are associated with erythema nodosum. The
nodules are painless and biopsy shows noncaseating
granuloma.1,3

ERYTHEMA NODOSUM

Robert Willan, the father of modern dermatology, first
described erythema nodosum, which is a nonspecific skin
reaction that has many conditions associated with it.

Irrespective of the precipitating cause, the ultimate
developments of polyarthralgia and erythema nodosum
depend on the following1,7:

Page 60

Chapter 8: Sarcoidosis of the Skin 53

FIGURE 8.12 (A) Subcutaneous nodule on the back of the forearm of an African-American
woman. (B) Large subcutaneous induration in a Caucasian woman. These lesions are usually non-
tender and may persist for years.1

A B

FIGURE 8.13 The first drawing of the skin lesions representing
erythema nodosum by Robert Willan, the father of modern der-
matology. (From his Cutaneous Diseases.)

• Racial or constitutional predisposition,
• Hormonal factors
• Geographic location

As a result, it is prevalent in women of childbearing years,
particularly in association with pregnancy and lactation;
Irish women in London, Puerto Rican migrants to New
York, and Martinique women in Paris; patients with his-
toplasmosis in Ohio; patients with coccidioidomycosis
in California; leprosy patients in Africa; and tuberculosis
(primary) patients in undeveloped countries. In Europe,
polyarthralgia and erythema nodosum are common pre-
sentations of sarcoidosis, while the oral contraceptive may
precipitate erythema nodosum.

In sarcoidosis, erythema nodosum is associated with
hilar adenopathy and a positive Kveim–Siltzbach skin
test.1 Fever and polyarthralgia are usual at the onset and,
until the end, skin lesions commonly show a play of
colors. The period ranges from 1 to 20 weeks. Recurrences
of erythema nodosum are seen in 10% of patients usually
within three months.2

Page 118

116 Index

Pulmonary sarcoidosis (cont.)
uncommon presentations, 36
unilateral hilar adenopathy, 36

R
Radiographic tests, 31

for bone lesions, 93
neurosarcoidosis diagnosed by, 91
of pleural sarcoidosis, 43

Renal biopsy, 75
Renal carcinoma, 74
Renal stones, 74
Respiratory tract, 107–108
Reticulonodular sarcoidosis, 31
Rhinophyma, 49
Rhythm disorder (VES), 78, 80

S
SACE. See Serum Angiotensin Converting

Enzyme
Sarcoid granulomas

biopsy of, 41
bronchiolar, 9
hydrocephalus results from, 86
inclusion bodies in, 11
late, 10
on liver, 71
noncaseating, 13

Sarcoidosis. See also Erythema nodosum;
Neurosarcoidosis; Pleural
sarcoidosis; Pulmonary sarcoidosis

acute, 17, 56
of airways, 36
beginning stages of, 5–7, 17
bone, 93–101

differential diagnosis of, 97
lesions in, 93–97
nasal, 97

bronchoalveolar lavage in, 22
calcaneal, 97
calcium in, 74
cause of, 5
cerebellar ataxia localization of, 88
chest x-rays, 18, 19, 27
chronic, 18

lesions, 26
pleural thickening in, 45

clinical features, 17–20
appearance of, 17–18
fatigue, 17–18
fever, 17
for various disciplines, 19

clubbling in, 100
conference on, 3
death from, 77
diagnosis of, 9–10, 11, 14

biopsy results in, 9
clinical patterns in, 14

differential, 11, 14, 22
extra pulmonary manifestations in,

17
inclusions in, 10

effector cells in, 23
endocrine involvement in, 103–104
etiology of, 9
extrapulmonary, ACE in, 22
first case of, 1
gastrointestinal tract in, 105–106
geographic distribution of, 3
giant cells, 10
glandular, 63–64
granulomas, 9, 11
of heart, 77–81
hematological manifestations, 21
hepatic, 69–70
hydrocephalus in, 85
hypercalcemia in, 73
hypersplenism in, 21
immunologic investigations in, 22
labial, 106
liver in, 69–71
malignancy occurring with, 109–111
multisystem, 74, 78, 92
muscles, 98
myocardial, 77
nails in, 100
nasal mucosa, 108
nodular, 36
ocular, 61–62
pleural involvement in, 43
renal, 73–76

clinical presentation of, 73–74
of respiratory tract, 107–108
reticulonodular, 31
skin, 57

alopecia, 58
lupus pernio, 47–48, 49
maculopapular eruptions, 48, 51
plaques, 48, 50, 51
subcutaneous nodules, 52, 53

spinal cord in, 88
spleen in, 65–67
unusual syndromes of, 14
vertebral, 97
vitamin D role in, 74–75

Sarcoidosis of the upper respiratory tract
(SURT), 107–108

Scars, 57
Schaumann bodies, 10, 11, 69
Schaumann, Jorgen, 2, 3, 10
Schremer’s test, 61
Seizures, 85
Serum Angiotensin Converting Enzyme

(SACE), 22, 31
Sharma, Om P., 4
Siltzbach, Louis Eliot, 3

Sjögren’s syndrome, 64, 90
Skin

biopsy of, 52
grafts, 56
granulomas in, 11
lesions

chronic, 26, 31
from chronic sarcoidosis, 30
descriptions of, 2
in erythema nodosum, 53, 54
NSG, 14
in sarcoidosis, 9

sarcoidosis, 57
alopecia, 58
lupus pernio, 47–48, 49
maculopapular eruptions, 48, 51
plaques, 48, 50, 51
subcutaneous nodules, 52, 53

Spinal cord, 88
Spleen

disorders of, 65
endoscopic evaluation of, 66
enlargement, 65, 67
lesions of, 66
noncaseating granulomas in, 67
sarcoidosis in, 65–67

Splenectomy, 67
Steroids

neurosarcoidosis treated with, 85
treatment with, chest x-rays of, 38

SURT. See Sarcoidosis of the upper
respiratory tract

T
T-cells, 5, 6
Thrombocytopenia, 21
Thyroid, 103
Tuberculosis, 12, 97

chest x-ray, 36
dactylitis in, 97
lupus vulgaris from, 49
primary, 54, 55
pulmonary, 43

Tumors, 109

U
Ulcers, 56, 105
Ultrasound testing, 99
Uveitis

chronic, 47
eye, 61
subacute, 61

Uveoparotid fever, 64

V
Vertebral biopsy, 97
VES. See Rhythm disorder

Page 119

Index 117

W
Wheals, 2
Willan, Robert, 2, 52, 53
World Association of Sarcoidosis and

Other Granulomous Diseases
(WASOG), 4

X
X-rays

bone
cysts, 93

edema, 50
lesions, 50

chest, 18, 19, 27, 72, 74
BHL in, 80, 87, 90, 95, 109
bilateral hilar lymph nodes, 107
caseous bronchopneumonia, 55
chronic sarcoidosis, 38
composite images, 38
in diagnosis, 108
Ghon complex, 37
hilar adenopathy in, 70, 98

of lesions, 39
metal graft in, 96
myocardial sarcoidosis, 79, 80
pleural sarcoidosis, 44, 45
pulmonary sarcoidosis, 29, 30, 32, 33,

66, 109
sarcoidosis, 50
tuberculosis, 36, 37

foot, 97
hands, 95

with arthritis, 94

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