Disseminated Superficial Actinic Porokeratosis


Article Author:
Cuong Le


Article Editor:
Paul Bedocs


Editors In Chief:
Sherri Murrell


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Avais Raja
Orawan Chaigasame
Khalid Alsayouri
Kyle Blair
Radia Jamil
Erin Hughes
Patrick Le
Anoosh Zafar Gondal
Saad Nazir
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Hassam Zulfiqar
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Mark Pellegrini
James Hughes
Beenish Sohail
Hajira Basit
Phillip Hynes
Sandeep Sekhon


Updated:
11/13/2019 9:13:56 PM

Introduction

Disseminated superficial actinic porokeratosis (DSAP) is a disease of disordered keratinization. Disseminated superficial actinic porokeratosis is one of six variants of porokeratosis. It has more extensive involvement than most other variants. These other variants include linear porokeratosis, porokeratosis of Mibelli, punctate porokeratosis, porokeratosis palmaris et plantaris disseminata, and disseminated superficial porokeratosis. Porokeratosis ptychotropica, facial porokeratosis, giant porokeratosis, hypertrophic verrucous porokeratosis, reticulate porokeratosis, and eruptive pruritic papular porokeratosis are other rare variants. The eruptive form of porokeratosis is associated with malignancy, immunosuppression, and a proinflammatory state. The lesions appear all over the body. A feature that is seen in all of these variants is the cornoid lamella. It is seen on histology as a column of parakeratotic cells and is characterized by a raised ridge circumscribing the porokeratotic lesions. Risk factors for porokeratosis include genetics, immunosuppression, and ultraviolet light. The lesions in disseminated superficial actinic porokeratosis start as pink to brown papules and macules with a raised border in sun-exposed areas that can be asymptomatic or be slightly pruritic. There are many options for the treatment of disseminated superficial actinic porokeratosis, including topical diclofenac, photodynamic therapy (PDT), 5-fluorouracil (5-FU), imiquimod, vitamin D analogs, retinoids, and lasers. These lesions are considered precancerous. There is a 7.5% to 10% risk of malignant transformation to squamous cell carcinoma or basal cell carcinoma.[1][2]

Etiology

Genetics, ultraviolet radiation, trauma, infection, immunosuppression (including posttransplant) are causes of porokeratosis. There is a form of disseminated superficial actinic porokeratosis that is familial. Familial DSAP has an autosomal dominant inheritance pattern with incomplete penetrance. Mutations in the mevalonate kinase gene (MVK) on chromosome 12q24 were seen in patients with disseminated superficial actinic porokeratosis. MVK gene codes for mevalonate kinase, an enzyme that is part of the cholesterol synthesis pathway, that offers protection against ultraviolet light-induced cell death. Disseminated superficial actinic porokeratosis has a high incidence and is the most common form of porokeratosis. The possibility for malignant transformation is due to overexpression of p53 and is seen more commonly in lesions that have been present for a long time, larger lesions, lesions in the elderly, or lesions in patients that are immunocompromised.[3][4]

Epidemiology

There is a slight female predominance. It is seen more commonly in the 30s and 40s.

Histopathology

Skin biopsy should be done to include the border of the lesion. A column of parakeratotic cells is seen correlating to the raised border. This column is called the cornoid lamella. The granular layer underneath this column can be thin or not present. Spongiosis can be present.

History and Physical

Lesions appear as asymptomatic or pruritic erythematous or brown circular macules, papules, or plaques with a raised hyperkeratotic border surrounding the lesions and an atrophic and hypopigmented center. It occurs bilaterally and symmetrically. Lesions most commonly present in the third or fourth decade and occur in areas exposed to sunlight. The legs, forearms, shoulders, and back are the areas most commonly affected. The face can rarely be involved. The palms and soles are spared. Disseminated superficial actinic porokeratosis usually worsens when it is exposed to sunlight, and pruritus can intensify.[5]

Evaluation

Disseminated superficial actinic porokeratosis can be diagnosed clinically due to the characteristic appearance of the lesions. A skin biopsy can be performed if there is doubt. Dermoscopy can be a useful tool for evaluating disseminated superficial actinic porokeratosis. 

Nicola et al. proposed dermoscopic features seen in porokeratotic lesions.

  • A white border circumscribing the lesion
  • Homogenous central white scar-like area
  • Brownish globules or dots
  • Vascular structures: pinpoint vessels or irregular linear vessels crossing the lesion [6]

Treatment / Management

Treatment options include the following.

Topical diclofenac

Diclofenac is an NSAID that inhibits COX-2. The medication was approved for actinic keratosis and has been used to treat disseminated superficial actinic porokeratosis with variable results. It has a good safety profile.

Ingenol mebutate

Ingenol mebutate is used in the treatment of actinic keratosis and can be used for disseminated superficial actinic porokeratosis lesions. It can help with the hyperkeratosis but not the atrophy or hypopigmentation.

Topical vitamin D analog

Vitamin D3 analogs have shown good responses after being used for 6 to 8 weeks. Vitamin D3 analogs are known to induce the transcription of genes that are responsible for the differentiation and proliferation of the keratinocytes.

5-fluorouracil

5-FU inhibits thymidylate synthase and stops DNA synthesis, therefore, inhibiting fast proliferating cells. Its use results in a very severe and robust inflammatory reaction in some patients, including erythema, ulcerations, and dermatitis. The clinical response is usually temporary.

Imiquimod

Imiquimod works by recruiting the body's immune cells through activation of toll-like receptors 7 and 8 and the induction of cytokines. It has been used mostly for porokeratosis of Mibelli and porokeratosis palmaris et plantaris. It can induce an inflammatory response similar to 5-FU.

Photodynamic therapy

Photodynamic therapy has been used to treat actinic keratosis, basal cell carcinoma, and squamous cell carcinoma in situ. A photosensitizer is applied that gets uptaken by atypical keratinocytes. The photosensitizers used are 5-aminolevulinic acid (ALA) and methyl aminolevulinate (MAL). The atypical keratinocytes are destroyed when light is applied because the photosensitizers generate reactive oxygen species. Some studies show that MAL might be better than ALA because it is more lipophilic.

Retinoids

Retinoids are vitamin A derivatives and are used in disorders where there is abnormal keratinocyte proliferation. Topical retinoids are preferred over systemic retinoids. Systemic retinoids have more side effects and are teratogenic. Relapse is common.

Cryotherapy and other

Cryotherapy, excision, curettage, and dermabrasion have been shown to have some good responses. It is, however, limited and is not used for extensive disease. Cryotherapy leaves a scar, and recurrence is common. 

Lasers

Carbon dioxide (CO2), Q-switched ruby (QSRL), Neodymium:yttrium-aluminum-garnet (Nd:YAG), fractional photothermolysis (FP) lasers, and Grenz ray have been used to treat Disseminated superficial actinic porokeratosis. The carbon dioxide laser uses pulsed or scattered infrared light with wavelengths between 9.4 micrometers and 10.6 micrometers and works on the water inside cells. Vaporization of the liquid causes tissue destruction. Its use can leave hyperpigmentation. QSRL uses melanin as its target. It reduces hyperpigmentation but does not destroy the cornoid lamella. It has greater penetration than Nd:YAG. Nd:YAG laser is used at 532 nm for pigmented lesions. It removes the superficial papillary dermis. It was shown to decrease the hyperpigmentation and obliteration of the cornoid lamella. FP induces small zones of thermal necrosis that doesn't create too much damage, redness, or pain and allows for faster healing. Grenz ray uses electromagnetic radiation like x-rays. It inhibits proliferation by inhibiting DNA synthesis.[7]

Immunosuppressive agents

Medications that suppress the immune response, such as topical corticosteroids, are not usually effective because disseminated superficial actinic porokeratosis is not an inflammatory disease, but using these medications can help with the associated pruritus.[8][9][10][11][12]

Differential Diagnosis

  • Actinic keratoses
  • Granuloma annulare
  • Lichen planus
  • Seborrheic keratoses
  • Tinea corporis 
  • Flat warts

Enhancing Healthcare Team Outcomes

An interprofessional team best manages superficial actinic porokeratosis. Primary care providers should refer patients to dermatologists if the diagnosis is in doubt or for some treatments. Oncologists and transplant physicians should be vigilant and have a high level of suspicion for this diagnosis. Clinicians should be aware that superficial actinic porokeratosis is a premalignant lesion, and patients should be advised to avoid the sun and tanning spas. Anytime there is a change in lesion characteristics, a biopsy is recommended. Dermatology nurses and nurse practitioners provide patient education and ensure follow up. Pharmacists educate patients about medications, check for drug-drug interactions, and monitor for compliance. [Level 5]


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Disseminated Superficial Actinic Porokeratosis - Questions

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What is the most common porokeratosis?



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What is commonly seen on histopathology for disseminated superficial actinic porokeratosis and porokeratosis that correlates clinically to the raised border on these lesions?



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A 69-year-old male presents to the clinic with a rash on his lower legs. It has been present for years. It started as one spot and has spread. He complains of mild pruritus but denies any pain. The patient appears to be a well-developed, well-nourished male with a ruddy complexion. He has scattered hyperkeratotic erythematous papules on his scalp and forehead. He has red erythematous annular lesions with a raised border on his bilateral lower legs. They range in size from 3 mm to 1 cm. Some of the lesions are more hyperkeratotic than others. Which of the following is the next best step in the management of this patient?



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A 50-year-old man presents with a complaint of lesions on his lower extremity. He complains of mild itch and that the spots are spreading. On physical exam, numerous annular lesions varying in size from 3 mm to 8 mm with a raised scaly border and flat smooth center without trailing scale are seen. They are located on his lower legs bilateral. A topical medication is prescribed that can cause redness and irritation with its use. What is the mechanism of action of this medication?



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A 65-year-old man presents to the clinic complaining of a growing and bleeding lesion on his right lower leg. The lesion appeared two months ago and has been steadily growing and started bleeding a week ago, which prompted the appointment. On physical exam, a 1.2 cm hyperkeratotic nodule with hemorrhagic crust on the right anterior shin is noted along with numerous 3-5 mm annular lesions with a raised border on the bilateral legs. After a biopsy is performed. Which of the following is the most accurate statement regarding the patient's condition?



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Disseminated Superficial Actinic Porokeratosis - References

References

Disseminated superficial actinic porokeratosis: a treatment review., Skupsky H,Skupsky J,Goldenberg G,, The Journal of dermatological treatment, 2012 Feb     [PubMed]
Clinical analysis and etiology of porokeratosis., Gu CY,Zhang CF,Chen LJ,Xiang LH,Zheng ZZ,, Experimental and therapeutic medicine, 2014 Sep     [PubMed]
Treatment of Porokeratosis: A Systematic Review., Weidner T,Illing T,Miguel D,Elsner P,, American journal of clinical dermatology, 2017 Aug     [PubMed]
Dermoscopy of Disseminated Superficial Actinic Porokeratosis., Nicola A,Magliano J,, Actas dermo-sifiliograficas, 2017 Jun     [PubMed]
Porokeratosis arising in a burn scar., Nova MP,Goldberg LJ,Mattison T,Halperin A,, Journal of the American Academy of Dermatology, 1991 Aug     [PubMed]
Disseminated superficial actinic porokeratosis improved with fractional 1927-nm laser treatments., Ross NA,Rosenbaum LE,Saedi N,Arndt KA,Dover JS,, Journal of cosmetic and laser therapy : official publication of the European Society for Laser Dermatology, 2016     [PubMed]
Eruptive disseminated porokeratosis: a new classification system., Shoimer I,Robertson LH,Storwick G,Haber RM,, Journal of the American Academy of Dermatology, 2014 Aug     [PubMed]
Porokeratoses: an update of clinical, aetiopathogenic and therapeutic features., Kanitakis J,, European journal of dermatology : EJD, 2014 Sep-Oct     [PubMed]
Successful Use of Grenz Rays for Disseminated Superficial Actinic Porokeratosis: Report of 8 Cases., Ramelyte E,Bylaite-Bucinskiene M,Dummer R,Imhof L,, Dermatology (Basel, Switzerland), 2017 Aug 18     [PubMed]
Disorder of the mevalonate pathway inhibits calcium-induced differentiation of keratinocytes., Jin R,Luo X,Luan K,Liu L,Sun LD,Yang S,Zhang SQ,Zhang XJ,, Molecular medicine reports, 2017 Aug 1     [PubMed]
Light and laser treatment modalities for disseminated superficial actinic porokeratosis: a systematic review., Aird GA,Sitenga JL,Nguyen AH,Vaudreuil A,Huerter CJ,, Lasers in medical science, 2017 May     [PubMed]
[Analysis of clinical and genetic features of nine patients with disseminated superfacial actinic porokeratosis]., Li X,Zhou Q,Zhu L,Zhao Z,Wang P,Zhang L,Zhang G,Wang X,, Zhonghua yi xue yi chuan xue za zhi = Zhonghua yixue yichuanxue zazhi = Chinese journal of medical genetics, 2017 Aug 10     [PubMed]

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