# Palmoplantar Keratoderma

> Palmoplantar keratoderma for NEET-PG Dermatology covers classification by pattern, syndromes such as Mal de Meleda and Papillon-Lefevre, and treatment.

- Canonical URL: https://prepelephant.com/topics/neet-pg/dermatology/palmoplantar-keratoderma
- Exam / course: NEET-PG · Subject: Dermatology
- Publisher: PrepElephant (https://prepelephant.com) — Prepared and reviewed by the PrepElephant Academic Review Team
- First published: 2026-10-08
- Last updated: 2026-10-08
- Reference basis: Rook's Textbook of Dermatology; Fitzpatrick's Dermatology; Andrews' Diseases of the Skin
- How to cite: "Palmoplantar Keratoderma", PrepElephant, https://prepelephant.com/topics/neet-pg/dermatology/palmoplantar-keratoderma

## Direct answer

Palmoplantar keratoderma is a group of disorders in which the stratum corneum of the palms and soles thickens, and it may be inherited or acquired. Hereditary forms are classified by distribution and pattern, and several carry systemic associations, such as periodontitis in Papillon-Lefevre syndrome. Management is largely supportive, with keratolytics and emollients, and oral retinoids are reserved for severe disease.

## What you must remember

- **Classification:** diffuse (uniform), focal (over pressure areas), punctate (small keratotic papules) and striate (linear bands along flexor creases).
- **Mal de Meleda:** autosomal recessive diffuse keratoderma with a red, waxy border at the palmoplantar margins, caused by SLURP1 mutations.
- **Papillon-Lefevre syndrome:** keratoderma with knee and elbow hyperkeratosis and severe early periodontitis with loss of teeth, caused by CTSC mutations.
- **Richner-Hanhart syndrome:** tyrosinaemia type II, with painful palmoplantar keratoderma, corneal ulceration and sometimes intellectual disability.
- **Vohwinkel syndrome:** mutilating keratoderma with starfish-shaped keratoses and constricting bands; the classic variant is linked to GJB2 mutations and deafness, while the ichthyotic variant is a loricrin (LOR) disorder without deafness.
- **Acquired causes:** chronic arsenic exposure, and keratoderma with malignancy or drugs, so new onset in adults needs a search for a cause.
- **Treatment:** urea or salicylic acid preparations with emollients first; acitretin for severe diffuse disease.

## Matching pattern and syndrome in a stem

Most stems give the pattern, the associated organ and the gene, so identify these three in order. The table links common syndromes to their key clue.

| Syndrome | Gene or cause | Key associated clue |
|---|---|---|
| Mal de Meleda | SLURP1 | Red waxy palmoplantar border |
| Papillon-Lefevre | CTSC | Severe periodontitis, early tooth loss |
| Richner-Hanhart | TAT (tyrosinaemia type II) | Corneal ulcers, intellectual disability |
| Vohwinkel, classic variant | GJB2 | Sensorineural deafness, constricting bands |
| Arsenical keratoderma | Environmental arsenic | Hyperpigmentation and skin cancer risk |

A keratoderma with periodontal destruction points at once to Papillon-Lefevre syndrome, while corneal ulcers with keratoderma point to tyrosinaemia type II. Arsenical keratoderma matters because the skin lesions carry a cancer risk that needs monitoring.

Treatment also follows the pattern, because focal disease responds to keratolytics and emollients while severe diffuse disease may need acitretin. Corneal or dental findings in the same patient should prompt a search for the associated syndrome rather than treatment of the skin alone.

## Where the keratoderma stems catch candidates

The traps come from mixing up pattern with syndrome.

- **Trap:** assuming all palmoplantar keratoderma is inherited. **Correct:** acquired causes such as arsenic, and new onset in adults, need a search for the cause.
- **Trap:** matching Papillon-Lefevre syndrome to isolated keratoderma. **Correct:** its defining association is severe periodontitis leading to early loss of teeth.
- **Trap:** choosing systemic retinoids first line for a mild focal keratoderma. **Correct:** keratolytics and emollients are first line, and acitretin is for severe diffuse disease with contraception counselling.
- **Trap:** expecting topical steroids to correct a hereditary keratoderma. **Correct:** steroids do little for keratinisation, and keratolytics are the usual approach.

## Frequently asked questions

### Which gene is mutated in Papillon-Lefevre syndrome?

Papillon-Lefevre syndrome is caused by mutations in CTSC, which encodes cathepsin C, an enzyme needed to activate neutrophil serine proteases. The combination of palmoplantar keratoderma with severe periodontitis is the key clue.

### What is the classic feature of Mal de Meleda?

Mal de Meleda is characterised by diffuse palmoplantar keratoderma with a sharply demarcated, red, waxy border at the margins of the palms and soles. It is autosomal recessive and results from SLURP1 mutations.

### Which keratoderma is linked to tyrosinaemia type II?

Richner-Hanhart syndrome, caused by tyrosinaemia type II, shows painful palmoplantar keratoderma with corneal ulceration and sometimes intellectual disability. Dietary tyrosine and phenylalanine restriction forms part of management, so early recognition matters.

### What is the first-line treatment for focal palmoplantar keratoderma?

Keratolytic preparations such as urea or salicylic acid, combined with regular emollients, are first line for focal palmoplantar keratoderma. Oral acitretin is reserved for severe or widespread disease because of its teratogenicity and lipid effects.

### Can arsenic cause palmoplantar keratoderma?

Yes, chronic arsenic exposure from contaminated drinking water can cause palmoplantar keratoderma, which is often punctate and accompanied by hyperpigmentation. It is an important acquired cause in parts of India and carries a risk of skin cancer that needs monitoring.