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8.1 · Scleroderma (Systemic Sclerosis)
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c
. Fig. 8.1.1 An illustration demonstrates the fi nger discoloration stages of Raynaud’s phenomenon: ( a ) normal fi ngers, ( b ) pale fi ngers, ( c )
bluish discoloration due to ischemia, and ( d ) reddish discoloration due to postischemic hyperemia
d
fasciae by lymphocytes, plasma cells, and occasionally
eosinophils. Laboratory ndings show high ESR, blood
eosinophilia (characteristic), and hyperglobulinemia.
Diagnosis is usually based on MRI and laboratory
ndings; however, de nite diagnosis requires
full- thickness skin-to-muscle biopsy.
How can you di erentiate between hypothenar hammer
syndrome and Raynaud’s disease?
5 HHS has a male predominance, while Raynaud’s disease
has a female predominance.
5 HHS is an occupational disease, while Raynaud’s disease
is a primary disease or secondary to systemic disease.
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Chapter 8 · Dermatology
Signs on Radiographs
5 Resorption of the terminal phalanges
(acro-osteolysis) of the hands and the distal
portion of the radius and ulna are the most
common radiological features of scleroderma
(80 %).
5 Mandibular resorption resembling “Gorham
syndrome osteolysis” may be seen.
5 Soft-tissue calcinosis may be seen, especially in the
digits (
. Figs. 8.1.4 and 8.1.5 ).
5 Widening of the periodontal space on dental
radiographs may be seen.
. Fig. 8.1.2 An illustration demonstrates linear scleroderma
aff ecting the left forehead
5 Pulmonary fibrosis can be seen in advanced
chronic stages of scleroderma.
8
. Fig. 8.1.3 An illustration demonstrates Blaschko lines over the
right forehead. The same lines can be found on the left side
5 HHS has an asymmetric distribution (a ects one hand),
while Raynaud’s disease is typically symmetrical,
a ecting the hands or toes.
. Fig. 8.1.4 Plain thumb radiograph of a patient with
scleroderma shows fi ngertip calcinosis ( arrowhead )
8.1 · Scleroderma (Systemic Sclerosis)
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8
. Fig. 8.1.5 Lateral plain elbow radiograph of a patient with
scleroderma shows calcinosis around the elbow joint
( arrowheads )
Signs on Barium Swallow
The esophagus in CREST syndrome shows weak
peristalsis with no stripping waves (
esophagus may show fi ne, wavy horizontal lines due to
muscular contraction of the esophagus wall ( Feline
esophagus ).
. Fig. 8.1.6 ). The
. Fig. 8.1.6 Anteroposterior barium swallow radiograph of
the esophagus in a patient with scleroderma shows poor
esophageal motility ( arrowheads )
Signs on MRI
5 I n linear scleroderma , there are intracranial
parenchymal calcifications affecting mainly the
thalami, and the basal ganglia ipsilateral to the
skin lesion may be seen. Progressive multiple brain
aneurysms can be seen in linear scleroderma.
5 I n hypothenar hammer syndrome , the axial wrist
images will show hyperintense mass usually seen
on T1W images located around the ulnar artery at
the level of the hook of the hamate, representing
hematoma or thrombus (
5 I n deep morphea , there is T2 hyperintensity signal
observed over the skin and subcutaneous tissue
that may involve the muscles and the bone
beneath. The bone shows bone marrow edema
signal without bone erosions. Contrast
. Fig. 8.1.7 ).
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Chapter 8 · Dermatology
Further Reading
enhancement of the affected tissues reflects
ongoing inflammatory reaction. Enhancement
around the tendons may be observed due to
inflammation of the synovial sheath (synovitis).
5 I n eosinophilic fasciitis , there is increased
thickening and T2 signal intensity of the superficial
muscle fasciae with marked contrast enhancement
after contrast injection (
Characteristically, there is little or no signal change
within muscles, and the pathological changes are
confined only to the superficial muscle fasciae and
to a lesser degree to the deep muscle fasciae.
. Fig. 8.1.8 ).
8
. Fig. 8.1.7 Axial T2W wrist MR illustrations show high signal
intensity at the tip of the hook of hamate within the Gyon’s
canal representing hematoma of the ulnar artery ( arrowhead )
Abudakka M, etal. Hypothenar hammer syndrome: rare or
underdiagnosed? Eur J Vasc Endovasc Surg. 2006;32:
257–60.
Ahathya RS, et al. Systemic sclerosis. Indian J Dent Res.
2007;18:27–30.
Baumann F, et al. MRI for diagnosis and monitoring of
patients with eosinophilic fasciitis. AJR Am J Roentgenol.
2005;184:169–74.
Bologina JL, etal. Lines of Blaschko. J Am Acad Dermatol.
1994;31:157–90.
Christen-Zaech S, etal. Pediatric morphea (localized sclero-
derma): review of 136 patients. J Am Acad Dermatol.
2008;59:385–96.
Genchellac H, et al. Hypothenar hammer syndrome: gray-
scale and color Doppler sonographic appearance. J Clin
Ultrasound. 2008;36(2):98–100. doi:10.1002/jcu.
Grosso S, etal. Linear scleroderma associated with progres-
sive brain atrophy. Brain Dev. 2003;25:57–61.
Horger M, etal. MRI ndings in deep and generalized mor-
phea (localized scleroderma). AJR Am J Roentgenol.
2008;190:32–9.
Jacobson L, etal. Super cial morphea. J Am Acad Dermatol.
2003;49:323–5.
James WD, etal. Nodular (keloidal) scleroderma. J Am Acad
Dermatol. 1984;11:111–4.
Krell JM, etal. Nodular scleroderma. J Am Acad Dermatol.
1995;32:343–5.
Kreitner KF, etal. Hypothenar hammer syndrome caused by
recreational sports activities and muscle anomaly in the
wrist. Cardiovasc Intervent Radiol. 1996;19:356–9.
Mueller LP, etal. Hypothenar hammer syndrome in sports.
Knee Surg Sports Traumatol Arthrosc. 1996;4:167–70.
Robitschek J, etal. Treatment of linear scleroderma “en coup
de saber” with AlloDerm tissue matrix. Otolaryngol Head
Neck Surg. 2008;138:540–1.
Soma Y, etal. Frontoparietal scleroderma (en coup de saber)
following Blaschko’s lines. J Am Acad Dermatol. 1998;38:
366–8.
. Fig. 8.1.8 Axial T1W postcontrast, fat-sat, thigh MR
illustration demonstrates the typical fi ndings in eosinophilic
fasciitis. Notice the marked thickening and enhancement of the
superfi cial and deep fascial planes with no signal intensity or
contrast enhancement of the muscles of the subcutaneous
tissues
8.2 Lipoid Proteinosis (Urbach–Wiethe
Disease)
Lipoid proteinosis (LP) is a rare, autosomal recessive disease
characterized by in ltration of the skin, oral cavity, larynx,
vocal cords, and internal organs by a hyaline material composed of carbohydrates, proteins, and lipids.
LP is caused by defective basement membrane collagen
metabolism. e patient commonly presents with hoarseness
of voice since infancy due to deposition of the hyaline material within the vocal cords. Beaded, whitish papules along the
margins of the eyelids ( Blepharosis moniliformis ) are classical
8.2 · Lipoid Proteinosis (Urbach–Wiethe Disease)
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b
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c
. Fig. 8.2.1 Multiple images from a 28-year-old patient with lipoid proteinosis show the dermatological features of this disease. In ( a ), there are
multiple psoriatic-like lesions over the elbow. In ( b ), a whitish papule (blepharosis moniliformis) along the margin of the upper eyelid can be seen
( white arrowhead ). In ( c ), the tongue is thickened and shows multiple nodules ( black arrowhead ). In ( d ), there is a focal area with reduced hair on
the scalp ( arrow ). The patient has a history of hoarseness of voice since the age of 3 years
features of this disease (. Fig. 8.2.1 ). Pock-like scars over the
face and the body, waxy papules, less mobile tongue, and
thickened oral mucosa with yellowish tinges are other
d
shows positive periodic acid–Schi (PAS) stain result.
Di erential diagnoses of LP in adults include amyloidosis,
lipoidoses, and myxoedema.
common ndings. Deposition of the hyaline material in the
scalp can lead to patchy loss of hair ( alopecia areata ).
Hyaline deposition can be found in some cases in the trachea, stomach, esophagus, testes, pancreas, and vagina.
Diabetes mellitus, epilepsy, and calci ed cerebral vessels can
be seen associated with LP occasionally. Diagnosis can be
Signs on Chest Radiographs
In severe cases, LP can present as a bilateral alveolar lung
disease that mimics lung edema. This pattern is seen due
to deposition of the hyaline material within the alveoli.
con rmed by pathological skin biopsy. e hyaline material
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Chapter 8 · Dermatology
Signs on CT
5 Thickening and infiltration of the vocal cords by a
hypodense hyaline material can be observed
(
. Figs. 8.2.2 and 8.2.3 ).
5 Calcification within the cerebral hemispheres can
be seen when calcified vessels are present.
a
a
b
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b
. Fig. 8.2.2 Bronchoscopic image ( a ) correlated with axial CT
image ( b ) of the hypopharynx shows multiple submucosal
nodules. After biopsy these lesions, the results stated that these
nodules are composed of hyaline lipoid material deposited
within the submucosa. The nodules in ( b ) are almost completely
replacing the valleculae ( arrowheads )
. Fig. 8.2.3 Bronchoscopic image ( a ) correlated with axial CT
image ( b ) of the vocal cords of the same patient shows
thickening of both vocal cords, with the right one markedly
thickened compared to the left one in ( a ). In ( b ), the CT image
shows hypodense areas found in the anterior aspect of both
vocal cords bilaterally involving the anterior commissure
( arrowheads ), with subtle left vocal cord thickening anteriorly
Further Reading
Behera SK, etal. Lipoid proteinosis in two siblings. Indian J
Dermatol. 2006;51(1):47–8.
Mirancea N, etal. Vascular anomalies in lipoid proteinosis
(hyalinosis cutis et mucosae): basement membrane components and ultrastructure. J Dermatol Sci. 2006;42:
231–9.
Mukhija P, et al. Lipoid proteinosis. Indian J Dermatol.
2006;51(1):51–2.
Orton CI, etal. Lipoid proteinosis– e oro-facial manifesta-
tions. Br J Oral Surg. 1975;12:289–91.
Savage MM, etal. Lipoid proteinosis of the larynx: a cause of
voice change in the infant and young child. Int J Pediatr
Otorhinolaryngol. 1988;15:33–8.
Sen S, et al. Lipoid proteinosis. Indian J Dermatol.
2006;51(1):49–50.
Simpson HE.Oral manifestations in lipoid proteinosis. Oral
Surg. 1972;33(4):528–31.
8.3 · Dermatomyositis
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8.3 Dermatomyositis
Dermatomyositis (DM) is an in ammatory connective tissue
disorder characterized by in ammation of the muscles and
skin. DM is closely related to another in ammatory muscle
disease called “polymyositis” (PM).
DM is diagnosed by speci c criteria that include:
5 Proximal symmetrical muscle weakness : the typical clinical
presentation is bilateral symmetrical muscle weakness of
the limb-girdle muscles, o en a ecting the shoulders and
anterior neck exors. Progressive weakness is experienced
over weeks to months. Patients o en rst note an inability
to groom their hair or to rise from a sitting position.
Proximal dysphagia may be seen if the cricopharyngeus
muscle and muscles of the pharynx are involved.
Respiratory muscles of the chest wall can be a ected.
5 Muscle biopsy : muscle biopsy classically shows muscle
necrosis and in ammatory changes; however, muscle
biopsy can be normal in 10–15 % of cases.
5 Elevated muscle enzymes : elevated muscle enzymes like
creatine kinase (CK), serum transaminases, and lactic
dehydrogenase (LDH) is a common nding in DM and
PM.CK is a normal serum enzyme with three
isoenzymes: CK-MM (found in skeletal muscles),
CK-MB (found in cardiac muscles), and CK-BB (found
in neural tissue). In PM and DM, CK-MM and CK-BB
are o en elevated. However, up to 40 % of DM cases have
normal CK levels. CK is not so speci c to muscular
diseases, as it can be elevated in metabolic and
neurological diseases as well.
5 Speci c dermatological lesions : two cutaneous lesions are
very speci c for DM, among other dermatological
nonspeci c manifestations. e rst lesion is Gottron ’ s
sign (80 % of cases), which is characterized by
erythematous papules and plaques that are found over
bony prominences, particularly the metacarpal–
phalangeal and proximal and distal interphalangeal joints
(. Fig. 8.3.1 ). ey can also be found over the elbows and
knees. e second lesion is heliotrope rash (60 % of cases),
which is composed of violaceous to erythematous hue
rash with or without edema located in the periorbital
region (. Fig. 8.3.2 ). Photosensitivity occurs in 75 % of
patients with DM.Some patients with DM may develop
poikiloderma of Civatte with Gottron’s papules.
Poikiloderma of Civatte is de ned as a skin area with
extra-pigmentation, demonstrating a variety of shades
and associated with widened capillaries (telangiectasia).
5 Exclusion of other disorders causing a myopathy : like
endocrinopathies, neurological diseases, and muscular
dystrophies.
DM can be precipitated by viral infections (e.g., retrovirus) or parasitic infections (e.g., toxoplasmosis). DM can
also be associated with autoimmune disorders (e.g., scleroderma) and tumors. Arthralgia, Raynaud’s phenomenon, and
polyarthritis are seen with DM overlapped with autoimmune
diseases. Cardiac symptoms are uncommonly seen in
. Fig. 8.3.1 An illustration demonstrates Gottron’s signs
. Fig. 8.3.2 An illustration demonstrates heliotrope rash
DM.When the heart is a ected, atrioventricular (AV) conduction disturbance, arrhythmias, and mitral valve prolapse
are commonly seen.
DM can be classi ed into four groups:
Group 1 : pure PM
Group 2 : PM with cutaneous lesions (DM)
Group 3 : DM with autoimmune disease
Group 4 : DM with malignant neoplasm
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Chapter 8 · Dermatology
DM has a juvenile form that a ects young adults <12years
old. It is o en associated with high serum levels of Coxsackie
B virus antibody titers. Patients show same signs and clinical
manifestations as the adult form. Esophageal dysmotility
occurs in 50 % of cases.
Signs on Radiographs
5 DM patients show soft-tissue calcifications in up to
40 % chronic cases. The calcifications can be
superficial or deep and often located mainly
within the girdle muscle areas. The calcifications
are described as linear, reticular, or calcareal
(
. Fig. 8.3.3 ).
5 Pulmonary fibrosis is seen in the advanced stages
of the disease.
Signs on MRI
5 In the early stages of PM, there is tissue edema and
high signal intensity of the muscles on T2W
images.
5 In chronic cases, the muscles are replaced by fat.
There is high T1 signal intensity within the muscles
due to fat replacement of the muscular tissue, with
reduced muscle size due to chronic muscle
wasting and inflammation.
What is the di erence between calci cation and ossi ca-
tion?
5 Calci cation is the presence of an area of calcium
deposition within so tissue that does not form a
trabecular or cortical structure (no real bone formation
8
within the so tissue, only small area of calcium
deposition).
5 Ossi cation is the presence of an area of calcium
deposition within the so tissue that forms a trabecular
or cortical structure (a real bone formation within the
so tissue).
. Fig. 8.3.3 Anteroposterior plain radiograph of the left leg
shows multiple fi ne soft-tissue calcifi cations in a patient with
dermatomyositis ( arrowhead )
What are the types of calci cations and ossi cations?
5 Calci cation can be divided into metastatic calci cation
(calcium and phosphate metabolism disturbance that
leads to ectopic calci cation in normal tissues),
dystrophic calci cation (deposition of calcium in
damaged tissues while the serum calcium level is
normal), and calcinosis (deposition of calcium in so
tissue in the presence of normal calcium level).
Dystrophic calci cation is seen usually in posttrauma or
a er neoplastic therapy. Calcinosis is typically seen in
rheumatic diseases and DM.
5 Ossi cation is typically seen in cases like posttraumatic
ligamentous ossi cation (e.g., Pellegrini – Stieda disease ),
neurogenic heterotopic ossi cation (so -tissue
ossi cation a er long period of denervation), myositis
Ossi cans traumatica ( Sterner ’ s tumor ), and brodysplasia
ossi cans progressiva ( Munchmeyer disease ).
Pellegrini–Stieda disease is characterized by ossi cation
of the medial collateral ligament of the knee, commonly
a er trauma. Myositis ossi cans is a rare condition
characterized by progressive skeletal muscles ossi cation,
usually a er a major trauma. Fibrous dysplasia ossi cans
is a rare disease characterized by disabling ossi cation of
muscles, tendons, ligaments, and fascial planes (the
normal so tissues are transforming into bones).
Further Reading
Agarwal V, et al. Calcinosis in juvenile dermatomyositis.
Radiology. 2007;242:307–11.
8.4 · Ochronosis (Alkaptonuria)
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Lee LA, etal. Lipodystrophy and metabolic abnormalities in
a case of adult dermatomyositis. J Am Acad Dermatol.
2007;57:S85–7.
Magill HL, etal. Duodenal perforation in childhood derma-
tomyositis. Pediatr Radiol. 1984;14:28–30.
Marfatia YS, etal. Dermatomyositis in a human immunode -
ciency virus infected person. Indian J Dermatol Venerol
Leprol. 2008;74:241–3.
Stiglbauer R, etal. Polymyositis: MRI-appearance at 1.5 T and
correlation to clinical ndings. Clin Radiol. 1993;48:244–8.
8.4 Ochronosis (Alkaptonuria)
Ochronosis, also known as alkaptonuria, is a very rare autosomal recessive metabolic disease characterized by accumulation of homogentisic acid (HGA) in body tissues due to an
inherited de ciency of the enzyme HGA oxidase. HGA is a
main product of the amino acids tyrosine and phenylalanine.
Acquired form of ochronosis can be seen a er exposure to
some chemicals like hydroquinone.
Ochronosis has an incidence of 1:1,000,000in the general
population. e urine of patients with ochronosis turns dark
when it is exposed to air or alkaline environment because of
HGA polymerization a er its exposure to oxygen. e name
“alkaptonuria” comes from Arabic and Greek words referring
to the relationship between oxygen and urine kept standing.
Although HGA deposition may occur in any body tissue,
the disease severity is mainly related to the musculoskeletal
system. HGA accumulates particularly in the tendon ligament tissues and cartilage-rich joints. e tissues acquire
black color grossly due to the HGA pigment.
Patients typically present in the third decade of life complaining of back pain and sti ness. Skin and so -tissue
lesions are seen in the fourth and h decades. HGA acts as
a chemical irritant that leads to joint degeneration and
in ammation. Patients develop osteoarthritis arthropathy in
almost all large joints, but the main severity is classically
observed in the vertebral column. e typical feature of this
disease in the vertebral column involves intervertebral disk
calci cation.
Bluish-brownish discoloration of the skin is seen due to
deposition of HGA in the subcutaneous tissues, and it is a
pathognomonic nding of this disease. e skin pigment is
observed in cartilage-rich tissue as the ear (70 %) and nose
(. Fig. 8.4.1 ). e bluish pigment can also be seen in the cor-
nea and the sclera (. Fig. 8.4.1 ). It may also be excreted in the
sweat, causing changes of color in clothing.
Rarely, cardiac involvement of ochronosis may be seen in
advanced stages. Mitral and aortic valve stenoses are the
main pathologic complications seen. Renal function can
deteriorate when the pigment accumulates in the prostate.
Prostatic calci cation causes obstructive renal uropathy and
hydronephrosis.
a
. Fig. 8.4.1 Two illustrations show bluish discoloration of the sclera ( a ) and the earlobe ( b ) in a patient with ochronosis
b
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Chapter 8 · Dermatology
Signs on Radiographs
5 Typically, the intervertebral disks are calcified in
ochronosis, with severe intervertebral disk narrowing
(
. Fig. 8.4.2 ). The disease affects the lumbar region
first and then progresses to the thoracic and the
cervical vertebrae.
5 Osteoarthritic changes of the large joints with
sclerosis and osteophytes formation.
5 Progressive formation of marginal intervertebral
bridges and obliteration of disk spaces resulting in
“pseudo-block vertebrae.”
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. Fig. 8.4.2 Anteroposterior and lateral plain radiograph of the lower thoracic vertebrae in a patient with ochronosis shows severe
intervertebral disk space narrowing ( arrowheads )
e main di erential diagnosis of ochronotic changes of the
vertebral column is ankylosing spondylitis. How can you differentiate between the two diseases?
5 Ankylosing spondylitis a ects the sacroiliac joint in a
bilateral symmetrical fashion, while sacroiliac joint
a ection is not necessarily observed in ochronosis.
5 Ankylosing spondylitis shows syndesmophytes, bamboo
spine appearance on radiographs, and positive HLA-B 27.
All the past features are not part of ochronosis.
Demir S.Alkaptonuric ochronosis: a case with multiple joint
replacement arthropathies. Clin Rheumatol. 2003;22:
437–9.
Güar D, etal. Ochronosis and lumbar disc. Acta Neurochir
(Wien). 2006;148:891–4.
Lagier R, et al. Hip arthropathy in ochronosis: anatomical
and radiological study. Skeletal Radiol. 1980;5:91–8.
Şahin G, etal. A case of ochronosis: upper extremity involve-
ment. Rheumatol Int. 2001;21:78–80.
5 Bluish skin pigmentation and calci cation of the
intervertebral disks are not features of ankylosing
spondylitis.
Further Reading
Bal S, etal. Ochronosis with cardiovascular involvement: a
case report. Rheumatol Int. 2008;28:479–82.
Çapkin E, et al. Ochronosis in di erential diagnosis of
patients with chronic back ache: a review of the literature.
Rheumatol Int. 2007;28:61–4.
8.5 Lymphedema
Lymphedema is a pathological condition characterized by
excessive, regional interstitial accumulation of protein-rich
uid, typically due to secondary lymph drainage failure in the
presence of normal capillary ltration.
e lymph drainage system parallels the venous drain-
age system, and it moves through the lymphatic vessels
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