(Last Updated On: December 13, 2018)
Authors: Lily Lyralin Laconico-Tumalad, MD, DPDS; Eileen Liesl Abesamis-Cubillan, MD, FPDS

Abstract

Disseminated superficial porokeratosis (DSP) is a rare keratinization disorder of the epidermis. Treatment is poorly standardized and often difficult. A 60-year-old Filipino female presented with multiple brown, well-defined, annular keratotic papules and plaques with thread-like elevated borders on her trunk, extremities, neck and face which revealed cornoid lamellae on biopsy. A diagnosis of DSP was made. Due to the widespread distribution of lesions, she was started on oral isotretinoin 20mg/day (0.3mkd). 5-fluorouracil (5-FU) 4% cream once daily application was added after two weeks to enhance treatment efficacy, but resulted in intense erythema and painful erosions prompting its discontinuation after two weeks. She was then maintained on oral isotretinoin 30mg/day (0.5mkd) together with emollients and sunscreen. Three months after, there was a decrease in hyperpigmentation, elevation, roughness and thickness of the lesions. In our case, oral isotretinoin notably produced clinical improvement in DSP. This underlies that when treatment is warranted, oral isotretinoin may be an attractive option for managing control of DSP. However, in our case, maximum therapeutic outcome and recurrence rate were not assessed due to our limited treatment and follow up duration. Further studies with longer treatment period are still needed to confirm its efficacy and safety in the management of DSP.

 

Citation

Laconico-Tumalad, LL & Abesamis-Cubillan, EL. (2017). Disseminated superficial porokeratosis in an adult Filipino female treated with oral isotretinoin. Journal of the Philippine Dermatological Society, 26(1), 79-81.

Keywords

Disseminated superficial porokeratosis,  keratinization, disorder, epidermis

  1. 1 Kim SW, Min SU, Won CH, Cho S. Disseminated superficial porokeratosis in a patient
    with gastric cancer. Annals of Dermatology. 2008; 20(4):193-196.
  2. Cao HM, Wang ZY, Zhang GW, Liu CF, Pan CM, Zhao SXet al. Identification of a locus
    (DSP2) for disseminated superficial porokeratosis at chromosome 12q21.2-24.21.
    Clin Exp Dermatol. 2012; 37(6):672-676.
  3. Philippine Dermatological Society Health Information Systems. Philippine
    Dermatological Society. c2011 [updated November 2, 2016; cited January 13, 2017].
  4. O’Regan GM, Irvine AD. Porokeratosis. In: Wolff K, Goldsmith L, Katz S, Gilchrest B,
    Paller A, Lefell D, eds. Fitzpatrick’s Dermatology in General medicine. 7th ed.USA:
    McGraw-Hill Companies; 2008. pp. 442-445.
  5. Wang J, Liu Y,Liu F, Huang C, Han S, Lv Y etal. Loss-of-function Mutation in PMVK
    causes autosomal dominant disseminated superficial porokeratosis. Sci.Rep. 2016;
    6:24226.
  6. Kanzaki T, Miwa N, Kobayashi T, Ogawa S. Eruptive pruritic papular porokeratosis.
    J Dermatol. 1992; 19:109-112.
  7. Taru G, Ramchander, Varghese B, Barara M, Nangia A. Generalized linear
    porokeratosis: a rare entity with excellent response to acitretin. Dermatology
    Online Journal. 2011; 17(5):3.
  8. Requena L, Requena C, Cockerell CJ. Benign epidermal tumors and proliferations.
    In: Bolognia JL, Jorizzo JL, Schaffer JV. Dermatology. 3rd Edition. USA: Elsevier; 2012.
    pp.1801-1803.
  9. Abe M, Yokoyama Y, Ishikawa O. Successful treatment of disseminated superficial
    actinic porokeratosis with tacalcitol lotion. J Dermatol. 2010; 37: 913-915.
  10. Prak AH, Dela Rosa KM. Porokeratosis: Treatment and Management. Medscape.
    2016 May [cited 2016 May 08];Available from: http://emedicine.medscape.com/
    article/1059123-treatment.
  11. Weidner T, Illing T, Miguel D, Elsner P. Treatment of porokeratosis: a systematic
    review. Am J Clin Dermatol. 2017. Epub 2017 Mar 10.
  12. Mortazavi H, Aghazadeh N, Ghiasi M, Lajevardi V. A review of three systemic
    retinoids in dermatology: acitretin, isotretinoin and bexarotene. Iran J Dermatol.
    2013; 16:144-158.
  13. Fritsch P. Retinoids in psoriasis and disorders of keratinization. J Am Acad Dermatol.
    1992; 27 Suppl 6 (Part2):S8-S14.
  14. Gutierrez E,Galarza C,Ramos W, Tello M, Chávez de Paz P, Bobbio L etal. Facial
    porokeratosis: A series of six patients. Australas J Dermatol. 2010; 51:191-194.
  15. Sander CA, Pfeiffer C, Kligman AM, Plewig G. Chemotherapy for disseminated
    actinic keratoses with 5-fluorouracil and isotretinoin. J Am Acad Dermatol. 1997;
    36(2):236-238.
  16. Pinedo HM, Peters GFJ. Fluorouracil: biochemistry and pharmacology. J Clin Oncol.
    1988; 6:1653-64.
  17.  Craft N, Fox LP, Goldsmith LA, Papier A, Birnbaum R, Mercurio MG. VisualDx
    Essential Adult Dermatology. USA: Lippincott Williams and Wilkins; 2010. p.255.
  18. Tallon B, Bluemental G, Bhawan J. Porokeratosis ptychotropica: a lesser-known
    variant. Clin Exp Dermatol. 2009; 34:e895-e897.
  19. Enk A, Bork K, Hoede N, Knop J. Atypical facial porokeratosis of Mibelli. Br. J.
    Dermatol. 1991; 125:596-598.
  20. Danno K, Yamamoto M, Yokoo T, Ohta M, Ohno S. Etretinate Treatment in
    disseminated porokeratosis. J Dermatol. 1988; 15:440-444.
  21. Bundino S, Zina AM. Disseminated porokeratosis of Mibelli treated with RO 10-
    9359 A study of two cases with ultrastructural remarks. Dermatologica. 1980;
    160:328-336.
  22. Borroni RG, Poddighe D, Zecca M, Brazzelli V. Efficacy of acitretin for porokeratosis
    in a child with chronic cutaneous graft versus host disease. Ped Dermatol. 2013;
    30(1):148-150.

More Articles

A randomized, double-blind, comparative study on the safety and efficacy of virgin coconut (Cocos nucifera l.) oil against 1% hydrocortisone lotion as an anti-inflammatory and antipruritic preparation for mosquito reactions

  Authors: Uy, Veronica S, MD; Gracia B. Teodosio, MD, FPDS; Ma. Teresita G. Gabriel, MD, FPDS; Mary Catherine T. Galang, MD; Mohammad Yoga A. Waskito, MD; Johannes F. Dayrit, MD, FPDS     Abstract Background: Virgin coconut oil (VCO) has been reported...

read more