Palmoplantar Keratodermas

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Palmoplantar keratodermas

Also known as ‘keratosis palmaris et plantaris’

Palmoplantar keratodermas

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

PPK

It is important to determine whether other features are present

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

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

Classification of PPK

HEREDITARYACQUIRED

Hereditary keratoderma

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

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

Classification

PPK is classified clinically asDIFFUSEFOCALPUNCTUATE

Palmoplantar keratodermas

1.Diffuse type: uniform involvement of the palmoplantar surface

Palmoplantar keratodermas

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

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

Palmoplantar keratodermas

The genetic basis of many keratodermas particularly involves mutations in genes encoding

KeratinsConnexinsdesmosomal components

HEREDITARY PALMOPLANTAR KERATODERMA

Diffuse inherited keratoderma of Unna Thost

Diffusenon-epidermolyticpalmoplantarkeratoderma. Note the sharp demarcation at the wrist and side of the thumb

Diffuseepidermolyticpalmoplantarkeratoderma

Diffusehyperkeratosis with involvement of the lateral aspect of the foot; note the erythema at the border with uninvolved skin

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

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

Papillion–Lefèvre syndrome. Plantar keratoderma

Naxos Disease

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

FocalPPK Striatetype on the palm

FocalPPK Areatatype on the sole

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

Pachyonychia Congenita

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

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

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

Punctate keratoderma

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

Spiny Keratoderma

Punctate type

Buschke-Fischer syndrome

ACQUIRED PALMOPLANTAR KERATODERMAS

Acquired keratoderma due to psoriasis

Acquired keratoderma due to psoriasis

Acquired keratoderma due to psoriasis

Acquired keratoderma due to chronic eczema

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

Acquired PPK

more likely to present in adulthood

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

Complications of PKK

Complications of PPK

1. Severe pain2. Difficulty in walking3. Secondary infection

Histopathology of PPK

Histologic features of nonepidermolyticPPK. Note the massive hyperkeratosis, acanthosis & hypergranulosis

Treatments of PPK

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

I. GENERAL MEASURES

II.TOPICALIII.SYSTEMICIV.SURGICAL

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

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

Systemic

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

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

REFERENCES

Dermatology Illustrated Study Guide & Comprehensive Board Review 2012Google imagesemedicine.medscape.comdermnetnz.orgglobalskinatlas.com

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