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Disseminated Superficial Actinic Porokeratosis


BASICS


DESCRIPTION


  • A disorder of keratinization
  • Lesions of the skin with conical papules of a red and brown color surrounding the hair follicle
  • Edges may become raised over time (1).
  • Typically found in 30 to 40 year olds (2)
  • Commonly located on the arms and legs

ETIOLOGY AND PATHOPHYSIOLOGY


Proliferation of atypical keratinocytes from a mutant clonal cell line  
Genetics
  • Genetic studies have reveled mutation of MVK (3).
  • Mutation of keratinocyte proliferation and differentiation (3)
  • Autosomal dominant with variable penetrance (1)

RISK FACTORS


  • UV-light exposure (2)
  • Male sex (1)
  • Immune suppression (1)
  • Infection (1)

GENERAL PREVENTION


Sunscreen and avoidance of UV exposure (2)  

COMMONLY ASSOCIATED CONDITIONS


Skin malignancies in up to 10% of people (1)  

DIAGNOSIS


HISTORY


  • Onset in the 30 to 40s
  • Family history of similar lesions
  • May be pruritic

PHYSICAL EXAM


  • Sun exposed areas with hyperkeratotic papules, patches, or plaques surrounded by a thin-lined elevated border
  • Palms and soles are spared.

DIFFERENTIAL DIAGNOSIS


  • Disseminated actinic keratosis
  • Actinic keratosis
  • Nevoid basal cell syndrome
  • Lichen nitidus
  • Dermal duct nevus

DIAGNOSTIC TESTS & INTERPRETATION


Initial Tests (lab, imaging)
  • Biopsy with confirmation by histology
  • Cornoid lamella in the epidermis with absent granular layer under the parakeratotic column

Follow-Up Tests & Special Considerations
Regular skin exams to monitor for malignant changes  

TREATMENT


GENERAL MEASURES


No controlled studies exist as yet to evaluate treatment modality.  

MEDICATION


First Line
  • Diclofenac sodium 3% topical: Apply to lesions twice daily for 60 to 90 days (4)[C].
  • This topical medication is supported by best available case study evidence as compared with other medications.

Second Line
  • 5-florouracil 5% topical: Apply to lesions twice daily for 2 to 6 weeks (4)[C].
  • Topical imiquimod 5% topical cream: Apply to defined lesions 2 times per week at bedtime for 16 weeks; and leave on skin for 8 hours (4)[C].
  • Tretinoin (50 years and younger): Apply 0.05% cream to lesions once daily at bedtime (4)[C]
  • Vitamin D3 analogs (Tacalcitol) ointment 0.0002-0.0004% once or twice a day for up to 4 weeks (4)[C]
  • Second-line treatment options are separated based on a lack of high quality evidence.

ADDITIONAL THERAPIES


Cosmetic cover-up with tinted creams or spray tans.  

SURGERY/OTHER PROCEDURES


  • Photodynamic therapy (4)[C]
  • Cryotherapy (4)[C]
  • Laser therapy (4)[C]

ONGOING CARE


FOLLOW-UP RECOMMENDATIONS


Yearly dermatologic examination  

PATIENT EDUCATION


Avoidance of UV exposure  

PROGNOSIS


Excellent  

REFERENCES


11 Torkamani  N, Sinclair  R. Disseminated superficial actinic porokeratosis accentuated by spray tan. Int J Dermatol.  2015;54(12):e545-e546.22 Rouhani  P, Fischer  M, Meehan  S, et al. Disseminated superficial actinic porokeratosis. Dermatology Online J..  2012;18(12):24. http://escholarship.org/uc/item/66j9q5jh.33 Zhou  MS, Xie  HF, Chen  ML, et al. A novel mutation for disseminated superficial actinic porokeratosis in the MVK gene. Br J Dermatol.  2014;171(2):427-429.44 Skupsky  H, Skupsky  J, Goldenberg  G. Disseminated superficial actinic porokeratosis: a treatment review. J Dermatolog Treat.  2012;23(1):52-56.

CODES


ICD10


Q82.8 Other specified congenital malformations of skin  

ICD9


692.75 Disseminated superficial actinic porokeratosis (DSAP)  

SNOMED


Disseminated superficial actinic porokeratosis (disorder)  

CLINICAL PEARLS


Age- and UV-related nonmalignant skin condition  
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