spontaneous keloids rare variety

1. Spontaneous
keloids
Dr Musaddik Hossain Khan
Resident,phaseB
Dermatology and venereology,BSMMU

2. keloids
– In 1806 Alibert given the first description of keloid in the
name of cheloides
– In 1854 Addison used the term keloid

3. keloids
– Keloids are benign fibroproliferative tumours
– Occurs as a response to any kind of injury to the skin of
susceptible individual
– Extends beyond the margin of the wound
– Tends to grow symptomless usually
– Itching and pain may be present

4. Keloids cont
– Enlarged raised scar
– Pink,red,skin colour or darker than surrounding skin
– Usually feel firm and smooth
– Shiny and hairless
– Number may vary from single to multiple

5. Keloids cont
– Can affect anyone but more common in people with dark
skin(African,Hispanic or Chinese descent)
– Most commonly arise between puberty and 30 years of
age
– May develop from surgical scar
– May appear or enlarge during pregnancy

6. Keloids cont.
– Skin injury on the upper chest, sternum, shoulder, chin,
neck, lower legs and earlobes has predisposition to
produce keloids
– Most likely to form following burns, acne scars, wounds
that become infected or are under tension whilst healing

7. Types of keloid
– Primary (spontaneous)
– Secondary

8. Etiology

9. Factors associated with keloids
– TGF-β
– PDGF
– VEGF
– Collagen
– Cytokines(IL-1,IL6)
– Cytokines (IL-1,IL-6)
– Cyclooxygenase-2
– Plasminogen activator
inhibitor-1
– Matrix
metalloproteinase-2

10. Stimulous of keloid formation
– Keratin from other hair follicle or sebaceous gland
– Sebum
– Higher skin tension
– Hypoxic nature of wounds
– Occlution of microvessel
– Histamine and mast cell
– TGF-β

12. – Finite element analysis
of mechanical force
distribution around
keloids showed higher
tension at the keloidal
edges than central

13. Proposed mechanism

16. Spontaneous keloids
– Are those that result without a significant history of
trauma
– It is generally believed that they are trigerred by
microtrauma or minimal cutaneous inflammation in
genetically susceptible patients
– Exact etiology is unknown
– Have been reported in association with certain genetic
abnormality

17. Spontaneous keloids
– Genetic association
– Medical condition
– Spontaneous keloids without medical condition
– Medication associated

18. Genetic association
– Rubinstein-Taybi syndrome
– Bethlem myopathy
– Dubowitz syndrome
– Noonan syndrome
– Goeminne syndrome
– Conjunctivocorneal dystrophy
– X linked recessive polyfibromatosis

19. Bethlem myopathy
– Caused by dominant and recessive mutation in collagen
VI genes
– Proximal muscle weakness
– Distal joint contracture
– Spontaneous keloid
– Hyperkeratosis pilaris

20. Bethlem myopathy

21. Goeminne syndrome
– X linked trait (xq28)
– Torticolis
– Cryptorchidism
– Renal dysplasis
– Spontaneous keloid
– Multiple Nevi

24. Noonan syndrome
Low set ears
Ptosis
Webbed neck

25. Noonan syndrome
Ptosis in left eye
Wide set nipple
Webbed neck
Pectus
excavatum

26. Dubowitz syndrome
– Rare disorder
– Growth retardation
– Mental retardation
– Microcephaly
– Broad nasal bridge
– Bulbous nose

27. Dubowitz syndrome
– Abnormal ears
– Palpebral fissure slanting
– Dental malocclusion
– Cryptorchidism
– Eczema
– keloids

28. Polyfibromatosis
– X linked recessive disease
– Fibrosis
– Erosive arthritis
– Keloid formation

30. Rubinstein Taybi syndrome
– 1 in 100000 live birth
– Genetic disease
– Mutation in CBP or P300 or microdeletion of
chromosome 16p13.3
– Isolated loss of histone acetyl transferase activity of CBP
– Cytogenetic or molecular diagnosis can be detected in
55%

31. Rubinstein Taybi syndrome
– Growth retardation
– Mental retardation
– Speech delay
– Mood disorder
– Long eyelashes
– High arched eyebrow
– Palpebral fissure slanting
– Prominent nose
– Broad thumb and toes
– Keloid formation
– Atropic scar
– Pilomatrixoma
– Constipation
– Dyaphagia

32. Rubinstein Taybi syndrome
– Valvular defect
– Otitis media
– Glaucoma
– Corneal opacity
– Seizure
– Neural tumour
– Genitourinary problem
– Dental problem
– High arched palate
– Incomplete or delayed
descent of testes
– Deviation of fingers
– Increased risk of benign
and malignant tumour

33. Rubinstein Taybi syndrome

34. Rubinstein Taybi syndrome

35. Medication associated
– Letrozol (aromatase inhibitor)
– Isotretinoin

36. Treatment
– Intralesional injection of Triamcinolone
– Application of potent corticosteroid cream or steroid containing tape
– Silicone dressing or gel
– Compression with bandage or devices
– Radiotherapy after surgical removal
– Cryotherapy
– Laser therapy
– Intralesional injection of 5 FU

37. Take home message
– All keloids are not due to visible injury to the skin
– Secondary keloids are common
– Spontaneous keloids are rare
– Spontaneous keloids may be part of some genetic
syndromes
– Mangement of spontaneous keloids is usually
unsatisfactory