palmoplantar keratodermas
TRANSCRIPT
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Palmoplantar Keratodermasfacebook.com/groups/dermatologycourseonline
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Palmoplantar keratodermas
Also known as ‘keratosis palmaris et plantaris’
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Palmoplantar keratodermas
Its inherited or acquired heterogeneous group of skin conditions with chronic thick hyperkeratotic palms/soles
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PPK
It is important to determine whether other features are present
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PPKmust 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
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PPKTRANSGREDIENS = hyperkeratosis that crosses palm/sole edge contiguously or as callosities on pressure points on the fingers or knuckles, or elsewhereNON-TRANSGRADIENT = hyperkeratosis involves only the palms and soles
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Classification of PPK
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HEREDITARYACQUIRED
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Hereditary keratoderma
the condition runs in families, its AD or AR usually most severe (i.e. mal de Meleda, Papillon–Lefèvre)
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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
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Classification
PPK is classified clinically asDIFFUSEFOCALPUNCTUATE
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Palmoplantar keratodermas
1.Diffuse type: uniform involvement of the palmoplantar surface
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Palmoplantar keratodermas
2.Focal type: localized areas of hyperkeratosis located mainly on pressure points and sites of recurrent friction
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Palmoplantar keratodermas
3.Punctate type: multiple small, hyperkeratotic papules, spicules, or nodules on the palms and soles may involve the entire palmoplantarsurface or may be restricted to certain locations
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Palmoplantar keratodermas
The genetic basis of many keratodermas particularly involves mutations in genes encoding
KeratinsConnexinsdesmosomal components
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HEREDITARY PALMOPLANTAR KERATODERMA
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Diffuse inherited keratoderma of Unna Thost
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Diffusenon-epidermolyticpalmoplantarkeratoderma. Note the sharp demarcation at the wrist and side of the thumb
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Diffuseepidermolyticpalmoplantarkeratoderma
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Diffusehyperkeratosis with involvement of the lateral aspect of the foot; note the erythema at the border with uninvolved skin
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Bart–Pumphrey syndrome. Leukonychia and knuckle pads in a patient with sensorineural deafness
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Hidrotic ectodermal dysplasia (Clouston syndrome). Note the “pebbled” skin on the dorsal aspect of the toes as well as the dystrophic nail plates
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Papillion–Lefèvre syndrome. Plantar keratoderma
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Naxos Disease
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Diffuse PPK1. Unna-Thost PPK2. Vörner PPK3. Mal de Meleda4. Nagashima-type PPK5. Vohwinkel syndrome6. Bart-Pumphrey syndrome7. Loricrin keratoderma8. Clouston syndrome (Hidrotic ectodermal dysplasia)9. Olmsted syndrome10.Huriez syndrome11.Papillon-Lefèvre syndrome12.Naxos disease
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FocalPPK Striatetype on the palm
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FocalPPK Areatatype on the sole
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Pachyonychia congenita. A Thickening of palmar skin & hypertrophic nail dystrophy with wedgeshaped subungual hyperkeratosis. B Painful focal plantar keratoderma w/associated erythema & blistering
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Pachyonychia Congenita
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Howel–Evans syndrome. Focal palmoplantar keratoderma inassociation with carcinoma of the esophagus.
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Focal type1. Focal PPK areata type or striate type
(Brunauer-Fohs-Siemens syndrome)2. 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)
3. Focal keratoderma with oral leukokeratosis4. Pachyonychia congenita5. Focal PPK associated w/esophageal
carcinoma
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Punctate PPK Keratotic papules, some coalescing to form plaques, on the palm (A) and sole (B).
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Punctate keratoderma
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Punctate keratoderma This lady's daughter had exactly the same lesions.
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Spiny Keratoderma
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Punctate type
Buschke-Fischer syndrome
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ACQUIRED PALMOPLANTAR KERATODERMAS
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Acquired keratoderma due to psoriasis
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Acquired keratoderma due to psoriasis
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Acquired keratoderma due to psoriasis
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Acquired keratoderma due to chronic eczema
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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
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Acquired PPK
more likely to present in adulthood
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CAUSES OF ACQUIRED KERATODERMAI. INFLAMMATORY SKIN CONDITIONSII. INFECTIONSIII. CIRCULATORY PROBLEMSIV. 2ry TO INHERITED CONDITIONS THAT
MAY NOT USUALLY RESULT IN PPKV. DRUGS AND TOXINSVI. INTERNAL DISORDERSVII. MISCELLANEOUS
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Complications of PKK
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Complications of PPK
1. Severe pain2. Difficulty in walking3. Secondary infection
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Histopathology of PPK
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Histologic features of nonepidermolyticPPK. Note the massive hyperkeratosis, acanthosis & hypergranulosis
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Treatments of PPK
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TreatmentTreatment of all types of hereditary and nonhereditary keratodermas can be difficult. Most treatment options onlyresult in short-term improvement and are compounded frequently by side effects
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I. GENERAL MEASURES
II.TOPICALIII.SYSTEMICIV.SURGICAL
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General MeasuresEvaluation of family members in hereditary case and genetic counselingIn acquired cases, identify and address possible underlying causesRegular foot care, careful selection of footwear, and treatment of fungal infections are important
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Topical1.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
2.Emollients3.Benzoic acid compounds4.Topical retinoids tretinoin5.Calcipotriol6.Topical steroids in conditions where there
is an inflammatory component
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Systemic
Retinoids effective, especially in some hereditary PPKs such as Mal de Meleda, Papillon-Lefevre syndrome, and erythrokeratodermia variabilis but require long-term treatment
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Surgery
1. Dermabrasion2. CO2 laser 3. Surgical excision: For severe,
difficult to treat keratoderma. Total excision of hyperkeratotic skin followed by grafts has been successful in a number of cases
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REFERENCES
Dermatology Illustrated Study Guide & Comprehensive Board Review 2012Google imagesemedicine.medscape.comdermnetnz.orgglobalskinatlas.com
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