Scleroderma is a condition characterized by hardening and thickening of the skin. It is an autoimmune disorder with a wide spectrum, ranging from localized to systemic forms. It commonly presents as round or oval plaques of hardened skin on the trunk. Females are more often affected, especially young adults. Variants include morphea, systemic sclerosis, and CREST syndrome. While some cases improve, others persist long-term. Diagnosis involves skin biopsy and blood tests. Treatment options include topical corticosteroids, phototherapy, and systemic medications like methotrexate.

1. SCLERODERMA
DR ABU SURAIH SAKHRI

2. • It is the sclerosis of skin characterized by
appearance of circumscribed or diffuse
,fibrous thickening and hardening of areas
that are hidebound to underlying tissues.
• AI disorder( autoimmune disorder)
• Spectrum is wide with localised and
systemic forms.

4. • round or oval, localised indurated
plaques. or bands of sclerosis.
• Internal organs are not involved.
• purely or largely localised to skin.

7. • Commonest in trunk .
• females are affected more often amongst
children or young adults.
• loss of hair , sweating, central
depigmentation with atrophic shiny
depressed skin are final sequelae of the
disease.

8. Variants of morphea
• Guttate MORPHEA
• Linear or segmental MORPHEA
• Pansclerotic MORPHEA
• En Coup de sabre with /without facial hemiatrophy .
• Bullous MORPHEA
• Keloid MORPHEA
• MORPHEA profundus
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12. Course
• Some improve while some persist.
•Histopathology
• epidermis is normal.
• perivascular lymphohistiocytic infiltrate in the
dermis.
• reticular dermis shows thickend collagen
bundles initially followed by hyalinization of
collagen.

13. Diagnosis
• Skin biopsy
• Serological test for ANA, anti histone ,anti
DNA.
• DD
• Pseudosclerosis
• Lichen sclerosis.

14. Rx
• Topical ; topical Corticosteroids,
Intralesional corticosteroids, Calcipotriol,
Tacrolimus, retinoids.
• Phototherapy; PUVA, UVA , narrow band
UVB, UVA -1.
• Systemic Rx ; Hydroxy chloroquine,
methotrexate, systemic steroids
,cyclosporine , D penicillamine .......

16. Initial manifestation is often Raynaud's
phenomenon, of one to sev years , following
which skin changes appear.
• Clinically SS are of 2 types;
• Progressive SS (Diffuse cutaneous SS)
• CREST syndrome ( limited cutaneous SS )
• Calcinosis , Raynaud's
phenomenon, esophagial
dysmotility, Sclerodactyly,
Telengectasia)

17. • The skin of forearms, fingers and lower
legs becomes tight waxy and stiff.
• Facial skin contracts and appears as
tightly bound to underlying bone..

19. Pursed lips.................

22. 1980 criteria for diagnosis of SS
• single major criterion
• Scleroderma proximal to the digits
, involving limbs, face ,neck or
trunk.
• At least 2 minor criterion ;
• Sclerodactyly

24. • Digital pitted scaring or loss of
substance of finger pad.
• B/l basal pulmonary fibrosis
• It is 97%sensitive and 98%
specific.

25. Course
• variable but generally progressive.
• Histopathology
• Atrophic epidermis.
• Hyalinization of derma l collagen.
• Intimal thickening of blood vessels.
• Replacement of subcutaneous tissue with
collagen.

26. Diagnosis
• Skin biopsy.
• Testing for Autoantibodies ; ANA and Anti -
Scl 70 Ab.
• Radiographic examination.
• Ba studies of upper GIT
• X-ray of chest , hand and soft tissues (
calcification)
• Other test - PFT.

28. DD
• Scleredema
• Scleromyxedema.
• Rx
• No definite therapy has shown to stop
progression of skin and visceral
invinvolvement.
• Mx of Raynaud's phenomenon;Nifedipine
10-30mg orally TID , Diltiazem 120-180mg
per day, topical Nitroglycerin.

29. • Physiotherapy for chest and hands.
• Immunomodulatory drugs;pulse
therapy with dexamethasone is useful if
started early.
• Cyclosporine, Methotrexate,
Mycophenolate mofetil and stem cell
therapy D-penicillamin, Relaxin and
Interferon gamma are the other drugs.

30. THANK YOU