Palmoplantar Keratodermas

PPKs are inherited or acquired heterogeneous group of skin conditions with chronic thick hyperkeratotic palms/soles

1. Palmoplantar Keratodermas facebook.com/groups/dermatologycourseonline

2. Palmoplantar keratodermas Also known as ‘ keratosis palmaris et plantaris ’

3. Palmoplantar keratodermas Its inherited or acquired heterogeneous group of skin conditions with chronic thick hyperkeratotic palms/soles

4. PPK It is important to determine whether other features are present

5. PPK must perform complete dermatological examination including skin (atrophy, knuckle pads, blisters, pseudoainhum ) nails , hair , and mucosa . also impaired hearing cardiomyopathy (in patients with woolly hair ), starfish keratoses

6. PPK TRANSGREDIENS = hyperkeratosis that crosses palm/sole edge contiguously or as callosities on pressure points on the fingers or knuckles, or elsewhere NON-TRANSGRADIENT = hyperkeratosis involves only the palms and soles

7. Classification of PPK

8. HEREDITARY ACQUIRED

9. Hereditary keratoderma the condition runs in families, its AD or AR usually most severe (i.e . mal de Meleda, Papillon – Lefèvre )

10. Acquired keratoderma more common & occurs as a result of a change in the health or the environment of the affected person or associated with skin disorders

11. Classification PPK is classified clinically as DIFFUSE FOCAL PUNCTUATE

12. Palmoplantar keratodermas Diffuse type : uniform involvement of the palmoplantar surface

13. Palmoplantar keratodermas Focal type: localized areas of hyperkeratosis located mainly on pressure points and sites of recurrent friction

14. Palmoplantar keratodermas Punctate type: multiple small, hyperkeratotic papules, spicules, or nodules on the palms and soles may involve the entire palmoplantar surface or may be restricted to certain locations

15. Palmoplantar keratodermas The genetic basis of many keratodermas particularly involves mutations in genes encoding Keratins Connexins desmosomal components

16. Hereditary Palmoplantar Keratoderma

17. Diffuse inherited keratoderma of Unna Thost

18. Diffuse non- epidermolytic palmoplantar keratoderma. Note the sharp demarcation at the wrist and side of the thumb

19. Diffuse epidermolytic palmoplantar keratoderma

20. Diffuse hyperkeratosis with involvement of the lateral aspect of the foot; note the erythema at the border with uninvolved skin

21. Bart– Pumphrey syndrome. Leukonychia and knuckle pads in a patient with sensorineural deafness

22. Hidrotic ectodermal dysplasia ( Clouston syndrome). Note the “pebbled ” skin on the dorsal aspect of the toes as well as the dystrophic nail plates

23. Papillion– Lefèvre syndrome. Plantar keratoderma

24. Naxos Disease

25. Diffuse PPK Unna- Thost PPK Vörner PPK Mal de Meleda Nagashima -type PPK Vohwinkel syndrome Bart- Pumphrey syndrome Loricrin keratoderma Clouston syndrome (Hidrotic ectodermal dysplasia ) Olmsted syndrome Huriez syndrome Papillon-Lefèvre syndrome Naxos disease

26. Focal PPK Striate type on the palm

27. Focal PPK Areata type on the sole

28. Pachyonychia congenita . A Thickening of palmar skin & hypertrophic nail dystrophy with wedgeshaped subungual hyperkeratosis. B Painful focal plantar keratoderma w/associated erythema & blistering

29. Pachyonychia Congenita

30. Howel –Evans syndrome. Focal palmoplantar keratoderma inassociation with carcinoma of the esophagus.

31. Focal type Focal PPK areata type or striate type ( Brunauer - Fohs -Siemens syndrome) Focal palmoplantar and gingival keratosis ( characterized clinically by focal PPK with leukoplakic appearance on the labial surface of the attached gingival lesion , and histologically by focal epidermolytic PPK) Focal keratoderma with oral leukokeratosis Pachyonychia congenita Focal PPK associated w/esophageal carcinoma

32. Punctate PPK Keratotic papules , some coalescing to form plaques, on the palm (A) and sole (B).

33. Punctate keratoderma

34. Punctate keratoderma This lady's daughter had exactly the same lesions.

35. Spiny Keratoderma

36. Punctate type Buschke -Fischer syndrome

37. Acquired palmoplantar keratodermas

38. Acquired keratoderma due to psoriasis

39. Acquired keratoderma due to psoriasis

40. Acquired keratoderma due to psoriasis

41. Acquired keratoderma due to chronic eczema

42. Acquired PPK NOT inherited as a primary genetic condition. They may occur as part of a generalised skin condition (some of which may be inherited) or as a result of another illness

43. Acquired PPK more likely to present in adulthood

44. CAUSES OF ACQUIRED KERATODERMA INFLAMMATORY SKIN CONDITIONS INFECTIONS CIRCULATORY PROBLEMS 2 ry TO INHERITED CONDITIONS THAT MAY NOT USUALLY RESULT IN PPK DRUGS AND TOXINS INTERNAL DISORDERS MISCELLANEOUS

45. Complications of PKK

46. Complications of PPK Severe pain Difficulty in walking Secondary infection

47. Histopathology of PPK

48. Histologic features of nonepidermolytic PPK. Note the massive hyperkeratosis, acanthosis & hypergranulosis

49. Treatments of PPK

50. Treatment Treatment of all types of hereditary and nonhereditary keratodermas can be difficult . Most treatment options only result in short-term improvement and are compounded frequently by side effects

51. GENERAL MEASURES TOPICAL SYSTEMIC SURGICAL

52. General Measures Evaluation of family members in hereditary case and genetic counseling In acquired cases, identify and address possible underlying causes Regular foot care , careful selection of footwear , and treatment of fungal infections are important

53. Topical Keratolytics e.g. 5 – 10% salicylic acid, 10% lactic acid, or 10% urea in a neutral base OR 6 % salicylic acid in 70% propylene glycol Emollients Benzoic acid compounds Topical retinoids tretinoin Calcipotriol Topical steroids in conditions where there is an inflammatory component

54. Systemic Retinoids effective , especially in some hereditary PPKs such as Mal de Meleda, Papillon-Lefevre syndrome , and erythrokeratodermia variabilis but require long-term treatment

55. Surgery Dermabrasion CO2 laser Surgical excision: For severe, difficult to treat keratoderma. Total excision of hyperkeratotic skin followed by grafts has been successful in a number of cases

56. REFERENCES Dermatology Illustrated Study Guide & Comprehensive Board Review 2012 Google images emedicine.medscape.com dermnetnz.org globalskinatlas.com

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