Transcript Diapositive 1

"On-line Atlas"
of
Dermatology and
Rheumatology
Cutaneous Manifestations of Rheumatic Diseases
www.archrheumatol.net/atlas/
The face in diffuse Systemic Sclerosis:
note the radial furrows surrounding the mouth,
pinched nose and scattered telangectasia.
Case 1a: Clinical Background
This patient had a 20 year history of Raynaud's
phenomenon and hand stiffness.
In the last few years she noticed the appearance of several
telangectasia on the skin and started suffering from
dysphagia and heartburn.
For one year before coming to our Department she had
been treated with nifedipine 20mg/d.
Physical examination revealed :
- sclerodactyly and pitted scars on the finger-tips,
- widespread telangectasia involving the face, upper trunk
and extremities,
- microstomia with angular creases,
- radial furrows
- mild hypertension.
Case 1b: Clinical Background
EGDS showed severe esophagitis, distal esophageal dilation and
hiatus hernia.
Esophageal manometry showed decreased amplitude and
incoordination of peristaltic waves in the distal esophagus and
inadequate lower esophageal sphincter competence.
In addition 24-hour pH monitoring showed an abnormal acid exposure
of the distal esophagus and decreased clearing ability.
Chest radiograph and pulmonary function tests (including carbon
monoxide diffusing capacity) were normal.
She had no electrocardiographic or echocardiographic evidence of
cardiac involvement.
Urinalysis and other indicators of renal function were within normal
limits.
She was discharged on penicillamine (150 mg/d), nifedipine (20
md/bid) and gastric antacids.
Radial furrows:
initial manifestations in a 38 year old woman with
diffuse Systemic Sclerosis.
Indurative phase: the skin is shiny, taut and
tightly adherent to the underlying structures
The "neck sign"
with beading, ridging, and hypopigmentation.
Yellow-brown sclerotic plaque on the neck with beading.
Case 2 : Clinical background
A 42 year old woman with a one-year history of Raynaud's phenomenon.
Previous extensive investigations were not significant: the patient had
anti-Scl-70 antibodies and an initial scleroderma pattern on capillaroscopy
but no evidence of visceral or cutaneous involvement.
One month before coming to our Department (while being treated with
nifedipine 10mg bid alone) she noticed the appearance of a slightly indurated
plaque on both sides of the neck.
Physical examination revealed two 4-5 cm in diameter superficial
yellow-brown plaques extending to the lateral sides of the neck and the
supraclavear area. The skin appeared thickened but still somewhat elastic
and the surface was smooth but irregular due to a network of closely
packed broad based papules (beading).
There was no sclerodactyly, pitted scars, telangectases or other
cutaneous evidence of systemic sclerosis.
She was treated with griseofulvin (500 mg/d) for 9 months with initial
improvement.
However, during the following months, she started complaining of hand
stiffness and developed slight sclerodactyly.
Penicillamine treatment was therefore started.
Raynaud's phenomenon:
white areas due to vasoconstriction.
Hand involvement in diffuse systemic sclerosis:
- note flexion contractures of the fingers,
- shortening of the terminal phalanges
as a result of distal bone resorption,
- and nail dystrophy.
Case 3: Clinical Background
• A 36 year old male patient who, at the age of
30 yrs, noticed Raynaud's phenomenon and
progressive stiffness and tightness involving
the skin of the hands and face.
• He received no medical treatment for 6 years
and when he was finally admitted to our
Department he had advanced acrosclerosis
and a severe pulmonary involvement.
• Antinuclear autoantibodies on HEp-2 cells
and anti-Scl-70 were found.
Hand involvement:
note dystrophic scarring of the fingertips.
Case 4 : Clinical Background
A 38 year old male patient.
Onset at the age of 34 years with stiffness and Raynaud's
phenomenon soon followed by swelling of the fingers due to soft
tissue edema and a symmetric polyarthritis involving the hands,
wrists, elbows, ankles and knees.
For two years he received NSAIDs alone with significant
improvement in the articular symptoms.Because of the progression
of hand involvement he was referred to our Department.
Physical examination revealed sclerodactyly and dystrophic
scarring of the fingertips but no typical face involvement or proximal
scleroderma.
Laboratory investigations showed antinuclear and anti-Scl 70
autoantibodies.
Respiratory function tests were not significant while esophageal
manometry demonstrated initial asymptomatic esophageal
involvement with normal acid exposure on 24-hour pH-monitoring.
No signs of calcinosis on the hand X-rays and a scleroderma pattern
(slow) at capillaroscopy were observed.
The patient was discharged on penicillamine and nifedipine.
Gangrene of the distal phalanges in a patient with
severe Raynaud's phenomenon and acrosclerosis.
Ischemic ulcers of the toes in a patient
with diffuse Systemic Sclerosis.
Ischemic toe in a patient with diffuse
Systemic Sclerosis: note nail dystrophy.
Calcinosis: cutaneous nodules of the fingers.
Localized morphea (1)
Different stages of the clinical evolution
Bright red erythema surrounding a central area of indurated skin
with a yellow-brown color and a smooth surface.
Localized morphea (2)
Different stages of the clinical evolution
Long-standing plaques of morphea.
Both plaques have an erythematous halo (lilac-ring) and a sclerotic
ivory-white central areas with a mottled brown hyperpigmentation.
Localized morphea (3)
Different stages of the clinical evolution
Sclerotic, ivory-white, shiny plaque on the back.
Localized morphea (4)
Different stages of the clinical evolution
Active plaque of morphea with an erythematous halo (lilac-ring)
and a thickened, sclerotic, yellowish area in the center.
Localized morphea (5)
Different stages of the clinical evolution
Irregular sclerotic plaque of morphea
on the popliteal fold extending upwards.
Dermatology Image Atlas - Johns Hopkins University
dermatlas.med.jhmi.edu/derm/
Case
Generalized fibrotic bound down skin with sclerodactyly
and hypopigmentation
This 8-year-old girl developed progressive systemic sclerosis
with sclerodactyly.
Tight fibrotic atrophic skin with narrowing of the fingers and patchy
depigmentation
This 28-year-old woman had progressive systemic sclerosis with
Raynaud phenomenon and sclerodactyly.
Necrotic purple papules with black crusts and ulcers
This 20-year-old man complained of shortness of breath.
Evaluation revealed a history of Raynaud syndrome,
digital infarcts, and other findings typical of scleroderma.
Extensive 1-4 mm telangiectasias
This 39 year old woman with Raynaud's syndrome and scleroderma
demonstrates extensive telangiectasias on her fingers.
She also had widespread telangiectasias on her face, trunk and extremities.
Telangiectasias on palms
A 52 year old woman with the CRESTsyndrome variant of scleroderma
developed diffuse progressivetelangiectasias which were most
prominent on her hands.
Telangiectasias on palms
A 52 year old woman with the CREST
syndrome variant of scleroderma
developed diffuse progressive
telangiectasias which were most
prominent on her hands.
A 34 year old woman with CREST syndrome developed
widepsread telangiectasias on her face, trunk and extremities.