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334 Chapter 9 Dermatology
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9.1
9.1
Scleroderma (Systemic Sclerosis)
Scleroderma is a systemic disease characterized by
progressive fi brosis of the skin and multiple organs.
The term “scleroderma” means literally “hard skin”
In scleroderma, the dermis is infi ltrated by
T-lymphocytes, causing abnormal fi broblasts activation, which leads to increased production of extracellular collagen type-I. This increase in collagen causes
skin thickening and tightening, which is the main manifestation of this disease.
Scleroderma is divided into two major forms: diffuse and focal. In the generalized form, diffuse skin
disease with organ involvement is typically seen. In
the focal form, there is limited cutaneous involvement
of the skin. Linear scleroderma and morphea are examples of focal scleroderma.
Females are affected by scleroderma seven times as
often as males. Patients present classically with fl exural
contracture of the terminal phalanges (claw-hand like
appearance), loss of the skin folds around the mouth
(mask-like appearance), atrophied nasal alae (mouse
facies), reduced opening of the mouth with jaw fi xation,
pathological changes of minor salivary glands mimicking Sjögren’s syndrome, and uncommonly trigeminal
neuralgia (4%). Laboratory investigations show positive antinuclear antibodies (70–90%), rheumatoid factor (25%), and hyperglobulinemia.
CREST syndrome is a variant of diffuse scleroderma
characterized by C alcinosis cutis, R aynaud’s phenome-
non, E sophageal dysmotility with dysphagia, S clero-
dactyly, and Telangiectasia . Calcinosis cutis is deposition
of calcium in the skin producing hard cutaneous nodules. Raynaud’s phenomenon is a series of fi nger discol-
oration after exposure to either temperature alternation
or emotional disturbance. First, fi ngers become pale
(white) due to small vessels vasoconstriction, then turn
blue as the vessels dilate to keep blood fl ow, and fi nally
turn red as blood fl ow returns (
means skin thickening of the fi ngers and toes that produces claw-hand deformity . Telangiectasia is dilatation
of the small vessels over the skin and the mucus membranes ranging between 0.51 mm in diameter. They are
commonly seen over the face and the neck as small red
marks on the skin.
Fig. 9.1.1 ). Sclerodactyly
Morphea is a superfi cial localized form of sclero-
derma characterized by a plaque of thickened skin,
often with an active, violaceous border with a yellow
to white center. It is commonly seen in children, with
an incidence of 1 per 100,000 individuals. Generalized
morphea is a term used to describe morphea lesions
covering >30% of the body surface. Pansclerotic dis-
abling morphea (Deep morphea) is a term used to
describe morphea that extends deep into the soft tissues with fi xation to the underlying structures.
Nodular (keloid) scleroderma is a rare form of cuta-
neous scleroderma that can occur in association with
diffuse scleroderma or morphea. It is seen as keloidal
hyperpigmented papules that develop early in the
course of the disease. An infl ammatory infi ltrate is
present during the period of active fi brosis.
Linear scleroderma , also known as (en coup de
sabre) , is a localized form of scleroderma seen as a linear, ivory-colored, deforming depression on the scalp
and the forehead mimicking a blow of a sword, which
is described as “coup de sabre” (
Fig. 9.1.2 ). The lesion
usually results in furrowing of the forehead causing
signifi cant facial asymmetry and cosmetic deformity.
Linear scleroderma lesions can be seen following
“ Blaschko’s lines ” (Fig. 9.1.3 ). These lines determine
Fig . 9.1.1 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
9.1 Scleroderma (Systemic Sclerosis) 335
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Fig . 9.1.2. An
illustration demonstrates linear
scleroderma affecting
the left forehead
Fig. 9.1.3. An
illustration demonstrates Blaschko lines
over the right
forehead. The same
lines can be found on
the left side
the distribution of many congenital and acquired skin
diseases (e.g., epidermal nevi). Many authors believe
that these lines represent the pattern of embryonic
migration of skin cells.
Linear scleroderma is usually seen in children and
in the young population, and it can affect the limbs,
especially the lower limbs, resulting in unilateral, atrophic limb. Linear scleroderma is usually confused with
Parry-Romberg syndrome when it extensively affects
half of the face. Brain involvement in linear scleroderma usually presents in the form of epilepsy, with or
without brain abnormalities.
tive trauma. The distal ulnar artery is most vulnerable
to trauma at the level of the hook of the hamate, which
works as an anvil against the dital ulnar artery as the
patient uses his/her hypothenar eminence of the hand.
The disease usually occurs in males who engage in
activities that expose the hypothenar eminence to
repetitive hand injuries. Workers using vibrating hand
tools are commonly affected. Sportsmen who use their
hands extensively in sports activities such as baseball,
handball, karate, and weightlifting or dumbbell training are also affected. Patients usually present with
palm pain, paraesthesia, and numbness with cold fi ngers and pallor, usually affecting the dominant hand.
HHS can be mistaken for Raynaud’s disease.
Raynaud’s disease is defi ned as episodic ischemia of
the fi ngers and toes, clinically presenting as pallor
(arterial vasospasm), cyanosis (deoxygenated static
venous blood), and rubor (reactive hyperemia).
Eosinophilic fasciitis ( Shulman’s syndrome ) is a rare
condition with scleroderma-like illness characterized clinically by infl ammatory swelling and induration of the arms and legs. Patients with eosinophilic
fascitis are usually females presenting with painful
thickening and induration of the skin and subcutaneous tissues of the affected limb. The disease may
affect the upper limbs, trunk, and lower limbs but
spares the face. Raynaud’s phenomenon is usually
not present, and organs are not affected. The skin is
typically thickened with orange-peel appearance.
Bilateral symmetrical muscle weakness and stiffness
of the joint may occur. Pathologically, there is infl ammation and infi ltration of the superfi cial muscle fasciae by lymphocytes, plasma cells, and occasionally
eosinophils. Laboratory fi ndings show high ESR,
blood eosinophilia (characteristic), and hyperglobulinemia. Diagnosis is usually based on MRI and
laboratory fi ndings; however, defi nite diagnosis
requires full-thickness skin-to-muscle biopsy.
D i ff erential Diagnoses and Related Diseases
Hypothenar hammer syndrome: hypothenar hammer
syndrome (HHS) is a rare condition characterized by
episodic digital ischemia as a result of occlusion of the
distal ulnar artery at the level of the hamate bone, typically due to repetitive blunt trauma to the ulnar artery
at the hypothenar eminence. The ulnar injury usually
results from thrombosis or aneurysms from the repeti-
How can you differentiate between hypothenar ham-
mer syndrome and Raynaud’s disease?
HHS has a male predominance, while Raynaud’s
disease has a female predominance.
HHS is an occupational disease, while Raynaud’s
disease is a primary disease or secondary to systemic
disease.
HHS has an asymmetric distribution (affects one
hand), while Raynaud’s disease is typically symmetrical, affecting the hands or toes.
336 Chapter 9 Dermatology
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Signs on Radiographs
Resorption of the terminal phalanges (acro-osteolysis) of the
hands and the distal portion of the radius and ulna are the
9.1
most common radiological features of scleroderma (80%).
Mandibular resorption resembling “Gorham syndrome
osteolysis” may be seen.
Soft-tissue calcinosis may be seen, especially in the digits
(Figs. 9.1.4 and 9.1.5 ).
Widening of the periodontal space on dental radiographs may
be seen.
Pulmonary fi brosis can be seen in advanced chronic stages of
scleroderma.
Fig. 9.1.5. Lateral plain elbow radiograph of a patient with scleroderma shows calcinosis around the elbow joint ( arrowheads )
Fig. 9.1.4. Plain thumb radiograph of a patient with scleroderma shows fi ngertip calcinosis ( arrowhead )
Signs on Barium Swallow
The esophagus in CREST syndrome shows weak peristalsis with
no stripping waves (Fig. 9.1.6 ). The esophagus may show fi ne,
wavy horizontal lines due to muscular contraction of the
esophagus wall ( Feline esophagus ).
9.1 Scleroderma (Systemic Sclerosis) 337
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Signs on MRI
I n linear scleroderma , there are intracranial parenchymal
calcifi cations aff ecting 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.
In
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 (Fig. 9.1.7 ).
In 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 enhancement
of the aff ected tissues refl ects ongoing infl ammatory
reaction. Enhancement around the tendons may be observed
due to infl ammation of the synovial sheath (synovitis).
eosinophilic fasciitis , there is increased thickening and T2
In
signal intensity of the superfi cial muscle fasciae with marked
contrast enhancement after contrast injection (Fig. 9.1.8 ).
Characteristically, there is little or no signal change within
muscles and the pathological changes are confi ned only to
the superfi cial muscle fasciae, and to a lesser degree to the
deep muscle fasciae.
Fig. 9.1.6. Anteroposterior barium swallow radiograph of the
esophagus in a patient with scleroderma shows poor esophageal
motility ( arrowheads )
Fig. 9.1.7. 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 )
338 Chapter 9 Dermatology
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9.1
Fig. 9.1.8. 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
For Further Reading
1 . Ahathya RS et al Systemic sclerosis. Indian J Dent Res 2007;
18:27–30
2 . Jacobson et al Superfi cial morphea. J Am Acad Dermatol
2003;49:323–5
3 . Christen-Zaech et al Pedatric morphea (localized sclero-
derma): review of 136 patients. J Am Acad Dermatol 2008;
59:385–96
4 . JM et al Nodular scleroderma. J Am Acad Dermatol 1995;
32:343–5
5 . WD et al Nodular (keloidal) scleroderma. J Am Acad
Dermatol 11:111–114
6 . Soma et al Frontoparietal scleroderma (en coup de saber)
following Blaschko’s lines. J Am Acad Dermatol 1998;38:
366–8
7 . Grosso et al Linear scleroderma associated with progres-
sive brain atrophy. Brain Dev 200357–61
8 . Robitschek et al Treatment of linear scleroderma “en coup
de saber” with AlloDerm tissue matrix. Otolaryngol Head
Neck Surg 2008138540–1
9 . Bologina et al Lines of Blaschko. J Am Acad Dermatol 1994;
31:157–90
10 . Genchellac et al Hypothenar hammer syndrome: gray-scale
and color Doppler sonographic appearance. J lin ltrasound
2008; 362. doi 10.1002/jcu
11 . Mueller et al Hypothenar hammer syndrome in sports.
Knee Surg Sports Traumatol Arthroscopy 19964:167–70
12 . Kreitner et al Hypothenar hammer syndrome caused by
recreational sports activities and muscle anomaly in the
wrist. Cardiovasc Intervent Radiol 199619:356–9
13 . Abudakka et al Hypothenar hammer syndrome: rare or
underdiagnosed? Eur J Vasc Endovasc Surg 32257–60
14 . Horger et al MRI fi ndings in deep and generalized mor-
phea (localized scleroderma). AJR 2008;190:32–9
15 . Baumann et al MRI for diagnosis and monitoring of
patients with eosinophilic fasciitis. AJR 2005;184:169–74
9.2 Lipoid Proteinosis (Urbach-Weithe Disease) 339
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9.2
Lipoid Proteinosis
(Urbach-Weithe Disease)
Lipoid proteinosis (LP) is a rare, autosomal recessive
disease characterized by infi 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. The 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 features of this
disease (
the body, waxy papules, less mobile tongue, and thickened oral mucosa with yellowish tinges are other
Fig. 9.2.1 ). Pock-like scars over the face and
common fi 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 calcifi ed cerebral
vessels can be seen associated with LP occasionally.
Diagnosis can be confi rmed by pathological skin
biopsy. The hyaline material shows positive periodicacid-Schiff (PAS) stain result. Differential diagnoses
of LP in adults include amyloidosis, lipoidoses, and
myxoedema.
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.
Fig. 9.2.1. 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 arrow-
head ). 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
340 Chapter 9 Dermatology
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Signs on CT
Thickening and infi ltration of the vocal cords by a hypodense
hyaline material can be observed (Figs. 9.2.2 and 9.2.3 ).
9.2
Calcifi cation within the cerebral hemispheres can be seen
when calcifi ed vessels are present.
Fig. 9.2.2. Bronchoscopic image ( a ) correlated with axial CT
image ( b ) of the hypopharynx shows multiple submucosal nod-
ules. 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 replac-
ing the valleculae ( arrowheads )
Fig. 9.2.3. Bronchoscopic image ( a ) correlated with axial CT
image ( b ) of the vocal cords of the same patient shows thicken-
ing 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
For Further Reading
1 . Mukhija P et al Lipoid proteinosis. Ind J Dermatol. 2006;
51(1):51–2
2 . Sen S et al Lipoid proteinosis. Ind J Dermatol. 2006;
51(1):49–50
3 . Behera SK et al Lipoid proteinosis in two siblings. Ind J
Dermatol. 2006;51(1):47–8
4 . Simpson HE. Oral manifestations in lipoid proteinosis.
Oral Surg. 1972;33(4):528–31
5 . Savage MM et al Lipoid proteinosis of the larynx: a cause of
voice change in the infant and young child. Int J Pediatr
Otorhinolaryngol. 1988;15:33–8
6 . Orton CI et al Lipoid proteinosis - The oro-facial manifes-
tations. Br J Oral Surg. 1975;12:289–91
7 . Mirancea N et al Vascular anomalies in lipoid proteinosis
(hyalinosis cutis et mucosae): Basement membrane components and ultrastructure. J Dermatol Sci 2006;42:231–9
9.3 Dermatomyositis 341
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9.3
Dermatomyositis
Dermatomyositis (DM) is an infl ammatory connectivetissue disorder characterized by infl ammation of the
muscles and skin. DM is closely related to another
infl ammatory muscle disease called “polymyositis”
(PM).
DM is diagnosed by specifi c criteria that include:
Proximal symmetrical muscle weakness: the typical
clinical presentation is bilateral symmetrical muscle
weakness of the limb-girdle muscles, often affecting
the shoulders and anterior neck fl exors. Progressive
weakness is experienced over weeks to months.
Patients often fi 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 affected.
Muscle biopsy: muscle biopsy classically shows
muscle necrosis and infl ammatory changes; however,
muscle biopsy can be normal in 10–15% of cases.
Elevated muscle enzymes: elevated muscle enzymes
like creatine kinase (CK), serum transaminases, and
lactic dehydrogenase (LDH) is a common fi 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 often elevated. However, up to 40% of
DM cases have normal CK levels. CK is not so specifi c to muscular diseases, as it can be elevated in
metabolic and neurological diseases as well.
Specifi c dermatological lesions: two cutaneous
lesions are very specifi c for DM, among other dermatological nonspecifi c manifestations. The fi 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. 9.3.1 ). They can also be
found over the elbows and knees. The 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. 9.3.2 ).
Fig. 9.3.1. An illustration demonstrates Gottron’s signs
Fig. 9.3.2. An illustration demonstrates heliotrope rash
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 defi ned as a skin area with extra-pigmentation demonstrating a variety of shades and associated with widened capillaries (telangiectasia).
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Exclusion of other disorders causing a myopathy:
like endocrinopathies, neurological diseases, and
muscular dystrophies.
DM can be precipitated by viral infections (e.g., retro-
9.3
virus), or parasitic infections (e.g., toxoplasmosis).
DM can also be associated with autoimmune disorders
(e.g., scleroderma) and tumors. Arthlargia, Raynaud’s
phenomenon, and polyarthritis are seen with DM overlapped with autoimmune diseases. Cardiac symptoms
are uncommonly seen in DM. When the heart is
affected, atrioventricular (AV) conduction disturbance,
arrhythmias, and mitral valve prolapse are commonly
seen.
DM can be classifi 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.
DM has a juvenile form that affects young adults
<12 years old. It is often associated with high serum
levels of Coxsackie-virus B antibody titers. Patients
show same signs and clinical manifestations as the adult
form. Esophageal dysmotility occurs in 50% of cases.
Signs on Radiographs
DM patients show soft tissue calcifi cations in up to 40%
chronic cases. The calcifi cations can be superfi cial or deep, and
often located mainly within the girdle muscle areas. The
calcifi cations are described as linear, reticular, or calcareal
(Fig. 9.3.3 ).
Pulmonary fi brosis is seen in advanced stages of the disease.
Signs on MRI
In the early stages of PM, there is tissue edema and high
signal intensity of the muscles on T2W images.
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 infl ammation.
Fig. 9.3.3. Anteroposterior plain radiograph of the left leg shows
multiple fi ne soft tissue calcifi cations in a patient with dermatomyositis ( arrowhead )
9.3 Dermatomyositis 343
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What is the difference between calcifi cation and
ossifi cation?
Calcifi cation is the presence of an area of calcium
deposition within soft tissue that does not form a trabecular or cortical structure (no real bone formation
within the soft tissue, only small area of calcium
deposition).
Ossifi cation is the presence of an area of calcium
deposition within the soft tissue that forms a trabecular or cortical structure (a real bone formation within
the soft tissue).
What are the types of calcifi cations and ossifi cations?
Calcifi cation can be divided into metastatic calcifi ca-
tion (calcium and phosphate metabolism disturbance
that leads to ectopic calcifi cation in normal tissues),
dystrophic calcifi cation (deposition of calcium in
damaged tissues while the serum calcium level is
normal), and calcinosis (deposition of calcium in soft
tissue in the presence of normal calcium level).
Dystrophic calcifi cation is seen usually in posttrauma
or after neoplastic therapy. Calcinosis is typically
seen in rheumatic diseases and DM.
Ossifi cation is typically seen in cases like posttrau-
matic ligamentous ossifi cation (e.g., Pellegrini-Stedia
disease ), neurogenic heterotopic ossifi cation (soft tis-
sue ossifi cation after long period of denervation),
myositis Ossifi cans traumatica ( Sterner’s tumor ), and
fi brodysplasia ossifi cans progressiv a ( Munchmeyer
disease ). Pellegrini-Stedia disease is characterized by
ossifi cation of the medial collateral ligament of the
knee, commonly after trauma. Myositis ossifi cans is a
rare condition characterized by progressive skeletal
muscles ossifi cation, usually after a major trauma.
Fibrous dysplasia ossifi cans is a rare disease characterized by disabling ossifi cation of muscles, tendons,
ligaments, and fascial planes (the normal soft tissues
are transforming into bones).
For Further Reading
1 . Marfatia YS et al Dermatomyositis in a human immunode-
fi ciency virus infected person. Indian J Dermatol Venerol
Leprol. 2008;74:241–43
2 . Agarwal V et al Calcinosis in juvenile dermatomyositis.
Radiology. 2007;242:307–11
3 . Lee LA et al Lipodystrophy and metabolic abnormalities in
a case of adult dermatomyositis. J Am Acad Dermatol.
2007;57:S85–7
4 . Magill HL et al Duodenal perforation in childhood der-
matomyositis. Pediatr Radiol. 1984;14:28–30
5 . Stiglbauer R et al Polymyositis: MRI-appearance at 1.5 T
and correlation to clinical fi ndings. Clin Radiol. 1993;48:
244–48
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