Porokeratosis plantaris, palmaris et disseminata

Author. Dr Karen Koch, Dermatologist, WITS Donald Gordon Mediclinic, Johannesburg, South Africa. DermNet NZ Editor in Chief: Adjunct A/Prof Amanda Oakley, Dermatologist, Hamilton, New Zealand. July 2018.


What is porokeratosis plantaris, palmaris et disseminata?

Porokeratosis is an uncommon skin condition of abnormal keratinisation [1].

Porokeratosis plantaris, palmaris et disseminata (PPPD) is an extremely rare form of porokeratosis in which scaly red-brown annular patches arise on the palms and soles and spread to the extremities and trunk [2].

Who gets porokeratosis plantaris, palmaris et disseminata?

The onset of PPPD is usually during the teenage years. There are rare reports of the onset of PPPD later in life [3]. PPPD was originally reported to be twice as common in men than women [2].

PPPD generally worsens during summer [2].

What causes porokeratosis plantaris, palmaris et disseminata?

Most often, PPPD occurs as a genetic autosomal dominant condition that runs in families [4]. However sporadic cases may arise [5].

What are the clinical features of porokeratosis plantaris, palmaris et disseminata?

PPPD usually presents initially as red-brown annular patches on the palms of the hands and soles of the feet. These spread to the extremities and trunk [2] forming reddish and slightly thinned small patches with defined borders [1]. 

  • Skin lesions on the palms and soles can be painful in about 25% of cases [2].
  • In some cases of PPPD, the skin lesions on the palms and soles are pinpoint scaly papules called punctate porokeratosis and it unclear whether this is a variant of PPPD [6].
  • PPPD on the body resembles disseminated superficial actinic porokeratosis (DSAP) [1]. 

What are the complications of porokeratosis plantaris, palmaris et disseminata?

PPPD is chronic and progressive and can affect the quality of life. 

There is a small risk of progression to cutaneous squamous cell carcinoma in long-standing cases [7].

How is porokeratosis plantaris, palmaris et disseminata diagnosed?

The diagnosis of porokeratosis is usually made clinically but sometimes a biopsy is needed. The pathology of porokeratosis is very distinct.

What is the differential diagnosis of porokeratosis plantaris, palmaris et disseminata?

The differential diagnosis of PPPD is mainly other forms of porokeratosis, especially DSAP. DSAP has similar skin lesions on the limbs, but they are uncommon on the trunk, and usually, the hands and feet are spared. DSAP tends to affect sun-exposed areas whereas PPPD can affect any part of the body.

What is the treatment for porokeratosis plantaris, palmaris et disseminata?

There is no known cure for PPPD and treatment is generally disappointing. Some patients respond well to the oral retinoidsacitretin or isotretinoin.

Topical treatments may include:

Physical destructive treatments may include:

Sun protection is very important as exposure to ultraviolet radiation may result in the development of skin cancer within the porokeratosis lesion.

What is the outcome for porokeratosis plantaris, palmaris et disseminata?

PPPD is chronic and progressive. It tends to fluctuate in severity often worsening in summer. Some people respond well to oral isotretinoin or acitretin but most often the condition relapses when the medication is stopped.

There is a small risk of progression to cutaneous squamous cell carcinoma, so the skin lesions should be monitored.

 

Related information

 

References

  1. Gutierrez EL, Galarza C, Ramos W et al. Facial porokeratosis: A series of six patients. Australas J Dermatol. 2010 Aug;51(3):191-4. doi: 10.1111/j.1440-0960.2009.00616.x. PubMed PMID: 20695858.
  2. Guss SB, Osbourn RA, Lutzner MA. Porokeratosis plantaris, palmaris, et disseminata. A third type of porokeratosis. Arch Dermatol. 1971 Oct;104(4):366-73. PubMed PMID: 5114411.
  3. Hartman R, Mandal R, Sanchez M, Stein JA. Porokeratosis plantaris, palmaris, et disseminata. Dermatol Online J. 2010 Nov 15;16(11):22. PubMed PMID: 21163173.
  4. Wei SC, Yang S, Li M, et al. Identification of a locus for porokeratosis palmaris et plantaris disseminata to a 6.9-cM region at chromosome 12q24.1-24.2. Br J Dermatol. 2003 Aug;149(2):261-7. PubMed PMID: 12932230.
  5. Shaw JC, White CR Jr. Porokeratosis plantaris palmaris et disseminata. J Am Acad Dermatol. 1984 Sep;11(3):454-60. PubMed PMID: 6237133.
  6. Sasson M, Krain AD. Porokeratosis and cutaneous malignancy. A review. Dermatol Surg. 1996 Apr;22(4):339-42. Review. PubMed PMID: 8624658.
  7. Marschalkó M, Somlai B. Porokeratosis plantaris, palmaris, et disseminata. Arch Dermatol. 1986 Aug;122(8):890-1. PubMed PMID: 2943230.

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