What is parakeratosis pustulosa?
Parakeratosis pustulosa describes a deformed fingernail or toenail surrounded by red, often scaly skin. It usually affects the thumb or index fingernail of young children, but occasionally involves another digit including a toenail. It is three times more common in girls.
It often starts with reddening of the skin under the free margin of the thumb or index finger nail. The redness later extends down to the nail fold resulting in swelling and loss of the cuticle. Thickened skin pushes up and deforms the nail plate.
Parakeratosis pustulosa is not itchy, but it may be a little painful.
The cause is unclear.
What does an affected nail look like?
The following signs may be seen.
- Subungual hyperkeratosis (skin thickening under the distal edge of the nail)
- Onycholysis (lifting of the nail plate off the nail bed)
- Onychomadesis (shedding of the nail plate)
- Transverse ridging
- Ragged or absent cuticles
- Red skin around the nail
- Blisters on the fingertips
What investigations should be done?
Parakeratosis pustulosa is a clinical diagnosis, so investigations are performed to rule out other nail conditions.
- Swabs may be helpful in culturing microorganisms associated with paronychia such as Staphylococcus aureus, Streptococcus pyogenes, Pseudomonas species, Proteus species, anaerobes or Candida albicans.
- Tinea infection can be confirmed by the presence of fungal hyphae in skin scrapings (mycology).
- Biopsy can differentiate atopic dermatitis and psoriasis but is rarely necessary.
What treatments are available?
No specific therapy is used for parakeratosis pustulosa. The following have occasionally been found useful.
- Stopping thumb sucking
- High potency topical steroids
- Topical retinoids
- Calcipotriol / calcipotriene
The condition usually improves with time. Some affected children later develop psoriasis.
- Pandhi D, Chowdhry S, Grover C, Reddy BS. Parakeratosis pustulosa - a distinct but less familiar disease. Indian Journal of Dermatology, Venereology and Leperology. 2003; 69 (1):48-50
- Hjorth, N., & Thomsen, K. (1967). Parakeratosis pustulosa. British Journal of Dermatology, 79(10), 527-532
- Luxinberg E, Silverman R. Nail Disorders in Children. Dermatology Nursing Januaray/Feburary 2010;22
- Aver O, Gunes AT. Parakeratosis pustulosa with Dyskeratotic Cells. Dermatology 1994;189:413-41
- Bolognia JL, Jorizzo JL, Rapani RP. Dermatology: second edition. 2008: 1026
On DermNet NZ:
- Nail Disorders in Children: Parakeratosis Pustulosa – Erin L. Luxenberg and Robert A. Silverman, Medscape Dermatology
Books about skin diseases:
See the DermNet NZ bookstore