What is DSAP?
DSAP stands for Disseminated Superficial Actinic Porokeratosis, a rare autosomal dominant keratinization disorder characterized by multiple superficial keratotic lesions with raised hyperkeratotic borders and slightly atrophic centers, typically appearing on sun-exposed areas. 1, 2
Clinical Characteristics
- Lesion morphology: Numerous annular papules with subtle raised hyperkeratotic borders and slightly atrophic centers, creating a distinctive appearance 2
- Distribution: Primarily affects sun-exposed areas, particularly the extremities and trunk 3
- Inheritance pattern: Usually inherited in an autosomal dominant fashion with genetic heterogeneity 2, 4
Pathophysiology
- Clonal keratinocyte disorder: The leading hypothesis suggests multifocal expansion of atypical clones of keratinocytes, potentially unmasked by actinic (UV) damage 2
- Genetic loci identified: Four susceptible loci have been mapped: 12q23.2-24.1, 15q25.1-26.1, 1p31.3-p31.1, 18p11.3, and a novel locus at 16q24.1-24.3 4
- UV exposure as trigger: Chronic ultraviolet light exposure is considered an etiological factor, with cases reported following narrowband UVB therapy 3
Malignancy Risk
- Squamous cell carcinoma development: There is an increased risk of cutaneous squamous cell carcinoma developing within DSAP lesions 2
- Histologic atypia: Lesions often show keratinocytic atypia centrally, which may progress to malignancy 2
- Immunosuppression as risk factor: Immunosuppression is a well-documented precipitant of porokeratosis and can cause rapid, widespread progression, as demonstrated in cases following bone marrow transplant induction therapy 2
Treatment Approach
- Simvastatin 2%/cholesterol 2% cream: This represents a promising long-term targeted treatment showing significant improvement in disease severity (measured by Investigator Global Assessment) with most progress occurring in the first 3 months 1
- Maintenance regimen: After initial daily application, tapering to a proactive approach twice weekly maintains disease control and may be important for skin tumor prevention 1
- Quality of life improvement: Treatment significantly decreases Dermatology Life Quality Index (DLQI) scores within the first 3 months 1
- Tumor prevention: No new skin tumors developed during 18 months of treatment with simvastatin/cholesterol cream 1
Critical Clinical Pitfalls
- Avoid prolonged UV exposure: Given the actinic nature of DSAP, patients should minimize sun exposure and avoid phototherapy when possible, as cases have developed following narrowband UVB treatment for other conditions 3
- Monitor immunosuppressed patients closely: Aggressive immunosuppression (such as bone marrow transplant conditioning) can trigger rapid, widespread development of DSAP within days 2
- Surveillance for malignancy: Regular skin examinations are essential given the increased squamous cell carcinoma risk within DSAP lesions 2
Note: DSAP should not be confused with dapsone (a sulfone medication used for dermatitis herpetiformis and leprosy), despite the similar acronym. 5, 6