Disseminated superficial actinic porokeratosis
What is disseminated superficial actinic porokeratosis?
Disseminated superficial actinic porokeratosis, or DSAP, is an inherited skin condition causing dry patches mainly on the arms and legs.
DSAP is a special type of inherited "sun spot" and is sometimes confused with actinic keratoses, but actinic keratoses are more likely to arise on the face and hands.
Who gets DSAP?
DSAP was first recognised in Texas but it is not uncommon in New Zealand. It appears on the sun-exposed skin of people of European descent. It tends to be more prominent in the summer and may appear less prominent in winter. New lesions have been provoked by ultraviolet light in sun lamps.
The average age which patients first notice DSAP is about 35 to 40, and its frequency in affected families increases steadily with age. It is rare in childhood.
The tendency to DSAP is inherited as an autosomal dominant characteristic, which means on average half of the children of an affected parent will also have the tendency. A locus on chromosome 12 was found to be responsible for DSAP in a Chinese family (J Invest Dermatol 2000 Jun;114(6):1071-4).
Clinical appearances of DSAP
DSAP mainly affects the lower arms and legs and arises more frequently on the lower legs of women than those of men. The forehead and cheeks are rarely affected and it almost never occurs on the scalp, palms or soles.
The DSAP lesion begins as a 1-3 mm conical papule, brownish red or brown in colour and usually around a hair follicle containing a keratotic (scaly) plug. It expands and a sharp, slightly raised, keratotic ring, a fraction of a millimetre thick, develops and spreads out to a diameter of 10 mm or more. The skin within the ring is somewhat thinned and mildly reddened or slightly brown, but a pale ring may be seen just within the ridge. The ridge itself is sometimes a dark brown. The central thickening usually disappears, but it may persist with an attached scale, follicular plug or central dell.
Sweating is absent within the lesions. Sun exposure may cause them to itch. In sunny areas, lesions may be present in very large numbers and may change from a circular to a polycyclic outline (overlapping circles). In less sunny climates patients have fewer lesions. In a few cases, the centre of the area becomes very red or may be covered by thick scale.
Development of true skin cancer within a DSAP lesion is very uncommon. However, many patients with DSAP have had significant exposure to the sun and may also have actinic keratoses and other forms of skin cancer.
How is DSAP diagnosed?
The diagnosis of porokeratosis is usually clinical, but it is sometimes made by characteristic features on pathology.
Treatment of DSAP
Unfortunately in our present state of knowledge there is no very satisfactory treatment for DSAP. Over the years we have tried:
- 5-Fluorouracil cream
- Imiquimod cream
- Tretinoin cream
- Alpha hydroxy acid cream
- Calcipotriol ointment
- Oral acitretin
- Photodynamic therapy
Nothing has proved very effective long term. Most people settle for just having the larger lesions frozen lightly and returning as necessary for further treatments, using a moisturiser to reduce the dry feeling.