Yasin, a 15-year-old boy, presented with progressive tightening and hardening of the skin leading to limited joint movement. Examination found tight, bound skin and flexion deformities of several joints but no joint swelling. His sister has a similar condition. Scleroderma is a collagen vascular disease characterized by fibrosis of the skin and internal organ involvement. It can be classified as limited or diffuse based on the extent of skin involvement and degree of organ damage. Treatment focuses on modifying disease progression, interfering with fibrosis, and managing vascular complications.

1. Scleroderma
Dr.Tanzilur Rahman
Resident,Phase-B,
BSMMU
yasin
Esha

3. Yasin molla a 15 years old boy hailing from Manikgonj came with the history of
progressive tightening of skin which is progressively increasing and causing
limitation of extension of multiple joints that is hampering his day to day activity.
He had no history of fever,joint pain,skin rash,photosensitivity and oral ulcer.
His younger sister is also suffering from same type of illness.
On locomotor system examination there is no joint swelling ,tenderness but there is
flexion deformity of knee,elbow,wrist and small joints of hand.
All the skin are tightened and pt can not sit on floor.
Case summary

6. Introduction
Multisystem collagen vascular disease of unknown etiology characterised by
• Fibrosis of the skin (caused by accumulation of connective tissue,collagen types
I and III) with
• Involvement of the internal organs(lungs,GIT,musculoskeletal,kidney,heart)
Scleroderma is derived from greek words Skleros(hard or indurated) and Derma(skin).
First detailed description was given by Carlo cruzio(1752).

7. Epidemiology
Acquired sporadic disease.
Affects all races.
Incidence is 9-19 cases/m/year.
Female predominance.
Peak age 20-50 yrs.

8. Etiology
• Exact etiology is unclear.
• Environmental factors trigger for the development of SSc-
silica exposure,vinyl chloride,epoxy resins,benzene,ccl4.
• Radiation exposure.
• CMV,HHV5,PBV,B19.
• Drugs-Bleomycin,pentazocine

11. classification
According to the extent of skin involvement
• Limited cutaneous systemic sclerosis (lcSSc), or limited scleroderma
• Diffuse cutaneous systemic sclerosis (dcSSc), or diffuse scleroderma
• Other types

12. Limited Scleroderma
• Skin thickening is distal to elbows and knees, not involving trunk.
• Can cause perioral skin thickening (pursing of lips).
• Less organ involvement.
• Isolated pulmonary hypertension can occur.
• The limited symptoms of scleroderma are referred to as CREST
syndrome.

13. CREST syndrome

14. Diffuse Scleroderma
• Skin thickening proximal to elbows and knees, involving the
trunk.
• More likely to have organ involvement.
• Pulmonary fibrosis and Renal Crisis are more common.

15. Limited and Diffuse cutaneous systemic
sclerosis

16. Other types
• Rarer type.
• Includes systemic sclerosis sine scleroderma
• Internal organ involvement without the skin changes

18. Clinically evident skin thickening is the hallmark of SSc-distinguises from others
 Symetrical,bilateral.
Skin is firmly bound to subcutaneous fat-thinning and atrophy.
Macular telangietasia-Localised scleroderma.
Atrophy of skin-slow healing ulceration on extensor surface of PIP
joints,volar pads.
Digital pits-Healed ischaemic ulcers.

19. • Vitiligo like hypopigmentation.
• Resorption of terminal phalanges-Acro osteolysis.
• Salt and pepper appearance of skin -scalp,upperback,chest.
• Obliteration of appendages of hair.
• Loss of transverse creases on dorsum of fingers.
• Fixed flexion deformity of fingers.

24. Local ischaemia

39. The axial CT scan shows linear opacities(yellow arrow),subpleural cysts,ground glass
opacity(white arrow) and thickening of interlobular septae,primarily at the base of the lung.

49. Renal manifestations
1) pathology
- intimal hyperplasia of the interlobular artery
- fibrinoid necrosis of afferent arterioles
- glomerulosclerosis
2) proteinuria, abnormal sediment, azotemia,
microangiopathic hemolytic anemia (scleroderma renal crisis), renal failure

50. Cardiac manifestations(10%)
1) Pericarditis
2) heart failure
3) arrhythmia
4) myocardial fibrosis

51. Treatment

52.  Disease modifying treatment:
 Glucocorticoids
 Cyclophosphamide
 Methotrexate
 Stem cell transplantation
Drugs that interfere with fibrotic process
 D-penicillamine
 Minocycline
Vascular therapy

53. Scleroderma Osteogenesis imperfecta
Multisystem collagen vascular disease of unknown
etiology characterised by fibrosis of skin and
involvement of internal organ
It is genetically heterogenous group of disorders
manifested by increased fragility of bones,
osteoporosis, blue sclera,deafness & as well as dental
abnormalities, lax joints and thin skin due to
abnormality of type-i collagen.
More occurs in female Equal frequency among male and female
It affects the skin and other internal organs like
(lungs,GIT,musculoskeletal,kidney,heart)
Classical clinical triad of OI is fragility of bone, blue
sclera, and deafness (due to ankylosis of ossicles &
osteosclerosis) .
Pathogenesis:Excessive fibriblast
productio,obliteration of small arteriole and production
of autoantibody .
Pathogenesis: Osteoblasts are abnormal and increased
in number. An immature form of osteoid matrix
formed with thin trabecula which are easily
broken.Type-i collagen are also defective so that the
sclera,cornea,joint and skin are abnormal.

54. Radiological
features of
Osteogenesis
imperfecta
1.Reduced bone density with cortical thinning
2.Multiple fracture of bones.
3.Excessive callus formation.
3.Vertebra shows cod fish biconcave deformities.
4.Multiple wormian bones in the skull.
5.Pelvis is very deformed with severe protrusio acetabuli.
6.Pop corn lesion in metaphyses.
7.Pseudoarthrosis may be present.
8.Dentinogenesis imperfecta in the teeth.
9.Soft and thin ribs with their posterior portion convex
downwards.
10.Kyphoscoliosis with vertebral collapse.

57. References
 Textbook of radiology and imaging:David sutton
 Andrews' disease of skin
 MSK findings in systemic sclerosis:European society of MSK radiology
 Oesophageal change in systemic sclerosis:Michael Atkinson and M.D.Summerling
 Scleroderma and lung:Tatjana Peros
 Radiopedia.org