Approach to

Palmoplantar
Keratodermas

Presenter : Dr Nisha Madaan

Moderator: Dr Deepashree
 CONTENT

• What are PPK

• Approach to a patient of PPK

• History

• Examination

• Workup

• Management

• Classification of PPK

• Syndromes associated with PPK

Palmoplantar
keratodermas

Palmoplantar keratodermas (PPKs) -diverse

group of hereditary and acquired disorders

defined by excessive thickening of palms

and soles.
Palmoplantar
keratodermas

Also known as ‘keratosis palmaris et plantaris’

 BASIS OF CLASSIFICATION OF PPK

• DIFFUSE
uniform involvement of
the palmoplantar
surface
• FOCAL-
 localized areas of
hyperkeratosis
 pressure points and
sites of recurrent
friction
 nummular or striate
• STRIATE
• longitudinal on the
fingers
• overlies flexor tendons.
• PUNCTATE
multiple small,
hyperkeratotic papules,
spicules, or nodules on
the palms and soles, not
bearing a consistent
relationship to pressure
points.
PPK

 TRANSGREDIENS = hyperkeratosis that crosses

palm/sole edge contiguously or as callosities on

pressure points on the fingers or knuckles, or elsewhere

 PROGREDIENS
• APPROACH TO A PATIENT WITH
PPK
 HISTORY
CUTANEOUS COMPLAINTS

• H/O thickening of palms and soles , may be associated with redness

of the underlying skin

- Age of onset

- Progression of lesions : worsening or improving with age

- Pattern of involvement of palms and soles : diffuse , focal ,striate

- Extent of involvement : progression beyond palms and soles,

involvement of other sites as knees, elbows, perioral

- Ass. symptoms as itching, pain, difficulty in carrying out daily activities

- H/O blistering or erosions over palms and soles

• H/O associated generalised dryness and scaling over the skin
(icthyosis)
• H/O formation of constriction bands around the digits and
autoamputation of digits

APPENDAGEAL INVOLVMENT
• H/O nail involvement : thickening , lustureless and rough nails

• H/O hair involvement : hair loss ( diffuse, patchy ) , sparse brittle

hair
• H/O dental involvement- caries, periodontitis with premature loss of
teeth
SYSTEMIC INVOLVEMENT
• H/O hearing loss since childhood

• H/O ocular involvement : photophobia, discharge and watering of

eyes, decreased vision
• H/O hoarseness / stridor

• H/O growth and development to rule out MR

• H/O cardiac involvement : arrthymias, syncope, chest pain

R/O Malignancy
• H/O weight loss
• H/O dysphagia to solids and liquid foods to r/o oesophageal carcinoma
• H/O any lumps noticed by the patient

R/O Acquired causes

• H/O seasonal variation
• Occupational history
• H/O drug intake
• H/O associated metabolic disorders
• Birth History
- H/O consanguinity
- antenatal and postnatal history
- H/O collodion membrane

• Family history and Pedigree charting

 EXAMINATION

• CUTANEOUS EXAMINATION
Palms and soles :
- thickness of palms and soles, whether there is associated
erythema
- pattern - diffuse, focal, punctate, striate
- transgrediens +/-
- involvement of other sites as knees , elbows, perioral.
- involvement of rest of the body for icthyosis, or any other
lesions s/o acquired causes
• Associated sclerodactyly , pseudoainhum , loss of any
digits
• Associated hyperhidrosis ,maceration and secondary
infection
• Nails examn : thickening , wedge shaped subungual
HK, dystrophy, anonychia

• Hair examn : alopecia ( diffuse, patchy) , sparse brittle

hair, woolly hair

• Dental examn : natal teeth, periodontitis , premature

loss of teeth

• Oral mucosa - leucokeratosis over tongue/buccal

mucosa
Systemic examn

• Ocular examn : corneal ulceration, visual acuity, slit lamp

examination

• ENT evaluation to r/o SNHL

• Skeletal examn : bow legs, ankylosis

• Cardiovascular examination to r/o cardiomyopathy

• Neurological evaluation
LASSIFICATION OF PPK

PPK

HEREDITARY ACQUIRED

AD AR
AD PPK AR PPK

• Vorner • Huriez
• Unna thost • Gamborg-Neilsen
• Camisa • Olmsted
• Griether • Naxos disease
• Huriez • Carvajal
• Watchers • Papillon-Lefevre
• PC type 1 • Richner Hanhart
• PC type 2 • Schopf-Schulz-passarge
• Brunauer-Fuhs-Siemens
• Clouston
• Howel Evans
• Vohwinkel
• Brauer-Buschke-Fischer
 HEREDITARY PPK

1. Diffuse PPK

2. Diffuse PPK with associated syndromes

3. Focal PPK

4. Focal PPK with associated syndromes

5. Punctate PPK
CLASSIFICATION ON THE BASIS OF
ASSOCIATED FEATURES
DIFFUSE PPK WITH SYNDROMES
 PPK DIFFUSE TYPE
1. Diffuse Non-epidermolytic (Unna–Thost type) and Epidermolytic Palmoplantar Keratoderma (Vörner
type) :
 2. Mal de Meleda

• Lesions present on the dorsal surfaces of the hands and feet as well as the
elbows and knees.
• Severe hyperhidrosis accompanied by malodor
• Periorificial lesions
•Thickened nails with koilonychia or subungual hyperkeratosis are common,
3. Greither Syndrome
 DIFFUSE PALMOPLANTAR KERATODERMA
WITH ASSOCIATED FEATURES
1.Mutilating Palmoplantar Keratoderma ( Vohwinkel
syndrome)
2.Bart–Pumphrey
Syndrome
3.Huriez Syndrome
4.Olmsted Syndrome
5. Papillon–Lefèvre Syndrome
6. Naxos Disease
Naxos
Diseas
e
 7.Hidrotic Ectodermal Dysplasia

• Diffuse PPK with hypotrichosis and

nail dystrophy.
• Hypotrichosis involving the scalp,
eyebrows, eyelashes and axillary and
genital regions
• Nails which may be normal at birth,
gradually become thickened and
dystrophic, displaying short nail plates
that are easily shed
 FOCAL PALMOPLANTAR KERATODERMA
(ISOLATED)
• Striate keratoderma
 FOCAL PALMOPLANTAR KERATODERMA
WITH ASSOCIATED FEATURES

1.Howel–Evans Syndrome
2. Richner–Hanhart Syndrome
3. Pachyonychia Congenita
4. Carvajal syndrome
 PUNCTATE PPK AND
RELATED CONDITIONS
• Filiform keratodermas
• Marginal papular
keratodermas
 Acquired PPK
• non-hereditary, non-frictional hyperkeratosis of the
palms and/or soles that involves ≥50% of the surface of
involved acral areas and that may or may not be
associated with clinical and histologic inflammation.
Acquired PPK

more likely to present in adulthood

CAUSES OF ACQUIRED PPKD
CAUSES OF ACQUIRED PPKD
 ACQUIRED PPKD

1.Keratoderma
Climactericum

• Seen in women >45 yrs of age

• No preceding history of skin
disorders, including eczema or
psoriasis.
• Obesity , Htn and cold dry
climates aggravate the
condition
• Initial involvement of the feet,
the presence of erythema and
fissuring, and lack of
transgrediens
2.Aquagenic PPKD

• develop thickening and white

to translucent, “pebbly”
changes on their palms shortly
after immersion in water (e.g.
within 3 minutes)
• Onset during second decade
of life, predilection for girls
and women
• Common in patients with cystic
fibrosis (CF)
3. Chemically Associated PPKD

• arsenic and chloracnegens, has been reported to present as PPK.

• major dermatologic signs of chronic arsenic toxicity - diffuse or

spotted hypo- and/or hyperpigmentation, particularly of the
abdomen
4.Drug-Related PPK

• glucan, tegafur, lithium, venlafaxine, and quinacrine (mepacrine).

• patients should be questioned about any new medications and the

time frame during which symptoms appeared
5.Systemic Disease-Related PPK

• hypothyroidism, myxedema, chronic lymphedema, and other

circulatory disorders, associated with PPK
• marked severity, sometimes verrucous in nature, with diffuse plantar
involvement and more limited involvement of the palms.
6. PPK and Malignancy

• Isolated nonspecific keratoderma -paraneoplastic marker for

internal malignancy
• Associated with esophageal, lung, bronchial, breast, urinary
bladder, gastric, colon, and skin cancer.
• Sézary syndrome is commonly associated with palmoplantar
hyperkeratosis
• Hyperkeratosis shows a cobblestone pattern with disruption of the
dermatoglyphic palmar skin markings
• Acrokeratosis paraneoplastica(Bazex syndrome)-associated with
acquired PPK in the context of internal malignancy
• Primarily affects men aged >40 years and is mainly associated with
neoplasia of the upper aerodigestive tract
• Also seen in patients with prostate carcinoma, primary SCC of the
leg, adenocarcinoma of the stomach and SCC of the vulvar region
• Can also present as a clinically unique paraneoplastic type of
keratoderma called tripe palms.
• Thickened, moss-like or velvety texture ,exaggerated
dermatoglyphics of the palmar surface of the hands and fingers.
• An underlying malignancy is found in >90% of cases.

• Carcinomas of the lung and stomach -most frequently observed

neoplasms
7. PPK Due to Dermatoses and Infections
Acquire
d
keratod
erma
due to
psoriasi
s
Acquired
keratoderma due
to chronic eczema
 WORKUP OF PATIENT

• Histopathology

-Histological diagnosis is usually relatively non-specific.

-Helps in differentiating epidermolytic PPK .

- An ultrastructural analysis can reveal relatively specific

changes in individual forms of PPK
Histologic
features of NE
PPK. Note the
massive
hyperkeratosis,
acanthosis &
hypergranulosis
• Vacuolation of
spinous layer
keratinocytes in
epidermolytic
PPK
• Papillon–Lefèvre Syndrome - HK with parakeratotic patches ,
acanthosis and pv infiltrates

• Oculocutaneous Tyrosinemia- eosinophillic cytoplasmic

inclusions

• Voh Winkel ds.- HK with round retained nuclei with

hypergranulosis

• Punctate Porokeratosis- columnar PK which resembles

coronoid lamella of porokeratosis and poorly developed granular
layer
• Eosinophilic
cytoplasm but
without overt
epidermolysis and
inflammatory
infiltrate in
pachyonychia
congenita
• Molecular genetic analysis should be
performed for patient with PPK for
reaching a specific diagnosis.
• Workup acc. to the clinical diagnosis made-
• Detailed ENT evaluation as audiometry

• Dental evaluation

• Opthal evaluation

• Cardiac evaluation - ECG, ECHO

• Esophageal studies

• KOH scraping to r/o Scabies and fungal infections

• Endocrine evaluation to r/o hypothyroidism

• Malignancy screen
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
I. GENERAL MEASURES

II. TOPICAL

III. SYSTEMIC

IV. SURGICAL
General
Measures
• Evaluation of family members in hereditary cases
and genetic counseling
• In acquired cases, identify and address possible
underlying causes
• Regular foot care, careful selection of footwear are
important
 TOPICALS
Along with Physical debridement.

• Topical keratolytic agents

- cause peeling of epidermis and decrease the thickness of stratum corneum

1. UREA - humectant and keratolytic ,improves peneteration of other topicals,

• Pregnancy Cat C
• BD- QID in 10-40 % creams and lotions

2. SALICYLIC ACID - alters corneocyte adhesion by disrupting desmosomal

proteins
• Pegnancy Cat C
• BD-QID salicyclic acid ointment (5-20%) , corn plasters( 20-40% SA)
• Causes salicylism ( max dose 2gm/24 hrs in adults )
3.Alpha Hydroxy Acids- lactic acid,glycolic acid ( >20%)
• Not contraindicated in pregnancy

4.Propylene Glycol -humectant ,occlusive and keratolytic

• 40-60% propylene glycol in aqueous cream under occlusion

5. Benzoic acid ointment

• Topical Emollients

• Topical retinoids

-treatment limited due to side effects as skin irritation

• Topical steroids

-In dermatoses with inflammatory component

• Treatment of Hyperhidrosis-

-potassium permanganate soaks/ aluminium chloride hexahydrate

lotion

-iontophoresis
• Treatment of secondary bacterial or fungal infection
 SYSTEMIC

• Oral Retinoids

• -Effective, especially in some hereditary PPKs such as Mal de Meleda,

Papillon-Lefevre syndrome,but require long-term treatment
• -Acitretin 10-20 mg/day can be given in severe cases

• -Isotretinoin can be used as alternative

• -Alitretinoin has been used succesfully in few cases

• -Should be started at a low dose, then inc gradually

• -Use limited by their side effects mucocutaneous dryness, liver and TG

abnormalities
Surgery
1. Dermabrasion
2. 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
• Patients with oculocutaneous tyrosinemia may benefit from dietary
restriction of phenylalanine and tyrosine.

• Paraneoplastic keratodermas are refractory to local treatment and

may only respond to removal of the underlying neoplasm

• Acquired PPK are treated by removal of the underlying cause

• References
- Rooks textbook of dermatology-8th ed

- Fitzpatricks dermatology-8th ed

- Bolognia dermatology-3rd ed

- Hereditary Palmoplantar Keratodermas,Markus Braun-Falco,JDDG; 2009 • 7:971–984

-Papillon–Lefèvre syndrome: clinical presentation and management options,Clinical,

Cosmetic and Investigational Dentistry 2015:7 75–81

-Palmoplantar keratoderma (PPK): acquired and genetic causes of a not so rare

disease,JDDG | 1610-0379/2014/1209

-Acquired Palmoplantar Keratoderma,Am J Clin Dermatol 2007; 8 (1): 1-11

Thank
you