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Published in: American Journal of Clinical Dermatology 4/2017

01-08-2017 | Systematic Review

Treatment of Porokeratosis: A Systematic Review

Authors: Till Weidner, Tanja Illing, Diana Miguel, Peter Elsner

Published in: American Journal of Clinical Dermatology | Issue 4/2017

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Abstract

Background

Porokeratosis (PK) is a rare skin disease of unknown etiology. It consists of a keratinization disorder, which may appear in several clinical forms and can undergo malignant transformation. The histopathological hallmark of PK is the cornoid lamella. While many topical, systemic, and surgical treatment modalities for PK have been described, no randomized controlled trials have been performed yet. Because of a lack of treatment standards for PK, European and international guidelines cannot be created.

Objective

The aim of this systematic review is to outline options for treating PK.

Methods

We performed a systematic literature search in an electronic database for published literature. A total of 88 articles fulfilling our inclusion criteria were found.

Results

There were no randomized controlled trials on the treatment of PK, but mainly case reports and case series. Porokeratosis of Mibelli showed the best outcomes after treatment with imiquimod cream and linear PK responded well to topical or systemic retinoids. Topical vitamin D acid derivatives may be the best therapeutic option for disseminated PK. Surgical interventions and cryotherapy may be preferred in areas where the use of topical agents is difficult or contraindicated.

Conclusion

To offer patients with PK an evidence-based high-quality standardized therapy, randomized controlled trials are needed.
Literature
1.
go back to reference Allegra F. The man behind the eponym. Vittorio Mibelli and the tale of “porokeratosis”. Am J Dermatopathol. 1986;8(2):169–72.PubMedCrossRef Allegra F. The man behind the eponym. Vittorio Mibelli and the tale of “porokeratosis”. Am J Dermatopathol. 1986;8(2):169–72.PubMedCrossRef
5.
go back to reference Wade TR, Ackerman AB. Cornoid lamellation: a histologic reaction pattern. Am J Dermatopathol. 1980;2(1):5–15.PubMedCrossRef Wade TR, Ackerman AB. Cornoid lamellation: a histologic reaction pattern. Am J Dermatopathol. 1980;2(1):5–15.PubMedCrossRef
6.
go back to reference Sasson M, Krain AD. Porokeratosis and cutaneous malignancy: a review. Dermatol Surg. 1996;22(4):339–42.PubMedCrossRef Sasson M, Krain AD. Porokeratosis and cutaneous malignancy: a review. Dermatol Surg. 1996;22(4):339–42.PubMedCrossRef
7.
go back to reference Lederman JS, Sober AJ, Lederman GS. Immunosuppression: a cause of porokeratosis? J Am Acad Dermatol. 1985;13(1):75–9.PubMedCrossRef Lederman JS, Sober AJ, Lederman GS. Immunosuppression: a cause of porokeratosis? J Am Acad Dermatol. 1985;13(1):75–9.PubMedCrossRef
8.
go back to reference Vire G, Latour D, King LE Jr. Porokeratosis and immunosuppression. J Am Acad Dermatol. 1986;14(4):683–4.PubMedCrossRef Vire G, Latour D, King LE Jr. Porokeratosis and immunosuppression. J Am Acad Dermatol. 1986;14(4):683–4.PubMedCrossRef
9.
go back to reference Kanitakis J, Euvrard S, Faure M, Claudy A. Porokeratosis and immunosuppression. Eur J Dermatol. 1998;8(7):459–65.PubMed Kanitakis J, Euvrard S, Faure M, Claudy A. Porokeratosis and immunosuppression. Eur J Dermatol. 1998;8(7):459–65.PubMed
11.
go back to reference Zhou Y, Liu J, Fu X, et al. Identification of three novel frameshift mutations of the MVK gene in four Chinese families with disseminated superficial actinic porokeratosis. Br J Dermatol. 2013;169(1):193–5. doi:10.1111/bjd.12224.PubMedCrossRef Zhou Y, Liu J, Fu X, et al. Identification of three novel frameshift mutations of the MVK gene in four Chinese families with disseminated superficial actinic porokeratosis. Br J Dermatol. 2013;169(1):193–5. doi:10.​1111/​bjd.​12224.PubMedCrossRef
12.
go back to reference Neumann RA, Knobler RM, Jurecka W, Gebhart W. Disseminated superficial actinic porokeratosis: experimental induction and exacerbation of skin lesions. J Am Acad Dermatol. 1989;21(6):1182–8.PubMedCrossRef Neumann RA, Knobler RM, Jurecka W, Gebhart W. Disseminated superficial actinic porokeratosis: experimental induction and exacerbation of skin lesions. J Am Acad Dermatol. 1989;21(6):1182–8.PubMedCrossRef
14.
go back to reference Parish JL. Topical vitamin D3 analogues: unapproved uses, dosages, or indications. Clin Dermatol. 2002;20(5):558–62.PubMedCrossRef Parish JL. Topical vitamin D3 analogues: unapproved uses, dosages, or indications. Clin Dermatol. 2002;20(5):558–62.PubMedCrossRef
18.
go back to reference Asawanonda P, Noppakun N, Huiprasert P. Seborrheic keratosis-like porokeratosis: a case report. Dermatol Online J. 2005;11(1):18.PubMed Asawanonda P, Noppakun N, Huiprasert P. Seborrheic keratosis-like porokeratosis: a case report. Dermatol Online J. 2005;11(1):18.PubMed
19.
go back to reference Bohm M, Luger TA, Bonsmann G. Disseminated superficial actinic porokeratosis: treatment with topical tacalcitol. J Am Acad Dermatol. 1999;40(3):479–80.PubMedCrossRef Bohm M, Luger TA, Bonsmann G. Disseminated superficial actinic porokeratosis: treatment with topical tacalcitol. J Am Acad Dermatol. 1999;40(3):479–80.PubMedCrossRef
20.
go back to reference Harrison PV, Stollery N. Disseminated superficial actinic porokeratosis responding to calcipotriol. Clin Exp Dermatol. 1994;19(1):95.PubMedCrossRef Harrison PV, Stollery N. Disseminated superficial actinic porokeratosis responding to calcipotriol. Clin Exp Dermatol. 1994;19(1):95.PubMedCrossRef
21.
go back to reference Tchernev G, Chokoeva AA, Ivanova B, et al. Disseminated superficial actinic porokeratosis (DSAP): significant improvement after local administration of calcipotriol/betamethasone gel? Wien Med Wochenschr. 2016;. doi:10.1007/s10354-016-0484-8 (Epub ahead of print). Tchernev G, Chokoeva AA, Ivanova B, et al. Disseminated superficial actinic porokeratosis (DSAP): significant improvement after local administration of calcipotriol/betamethasone gel? Wien Med Wochenschr. 2016;. doi:10.​1007/​s10354-016-0484-8 (Epub ahead of print).
24.
go back to reference Fritsch PO. Retinoids in psoriasis and disorders of keratinization. J Am Acad Dermatol. 1992;27(6 Pt 2):S8–14.PubMedCrossRef Fritsch PO. Retinoids in psoriasis and disorders of keratinization. J Am Acad Dermatol. 1992;27(6 Pt 2):S8–14.PubMedCrossRef
25.
go back to reference Garg T, Ramchander Varghese B, et al. Generalized linear porokeratosis: a rare entity with excellent response to acitretin. Dermatol Online J. 2011;17(5):3.PubMed Garg T, Ramchander Varghese B, et al. Generalized linear porokeratosis: a rare entity with excellent response to acitretin. Dermatol Online J. 2011;17(5):3.PubMed
28.
go back to reference Grover C, Goel A, Nanda S, et al. A case of extensive linear porokeratosis with evaluation of topical tretinoin versus 5-flourouracil as treatment modalities. J Dermatol. 2005;32(12):1000–4.PubMedCrossRef Grover C, Goel A, Nanda S, et al. A case of extensive linear porokeratosis with evaluation of topical tretinoin versus 5-flourouracil as treatment modalities. J Dermatol. 2005;32(12):1000–4.PubMedCrossRef
29.
go back to reference Goldman GD, Milstone LM. Generalized linear porokeratosis treated with etretinate. Arch Dermatol. 1995;131(4):496–7.PubMedCrossRef Goldman GD, Milstone LM. Generalized linear porokeratosis treated with etretinate. Arch Dermatol. 1995;131(4):496–7.PubMedCrossRef
32.
go back to reference Hartman R, Mandal R, Sanchez M, Stein JA. Porokeratosis plantaris, palmaris, et disseminata. Dermatol Online J. 2010;16(11):22.PubMed Hartman R, Mandal R, Sanchez M, Stein JA. Porokeratosis plantaris, palmaris, et disseminata. Dermatol Online J. 2010;16(11):22.PubMed
33.
37.
go back to reference Gotz A, Kopera D, Wach F, et al. Porokeratosis Mibelli gigantea: case report and literature review]. [Article in German. Hautarzt. 1999;50(6):435–8.PubMedCrossRef Gotz A, Kopera D, Wach F, et al. Porokeratosis Mibelli gigantea: case report and literature review]. [Article in German. Hautarzt. 1999;50(6):435–8.PubMedCrossRef
38.
go back to reference Agrawal SK, Gandhi V, Madan V, Bhattacharya SN. Topical tretinoin in Indian male with zosteriform porokeratosis. Int J Dermatol. 2003;42(11):919–20.PubMedCrossRef Agrawal SK, Gandhi V, Madan V, Bhattacharya SN. Topical tretinoin in Indian male with zosteriform porokeratosis. Int J Dermatol. 2003;42(11):919–20.PubMedCrossRef
40.
go back to reference Suarez-Amor O, Pereiro-Ferreiros M, Ginarte M, et al. Coexistence of linear porokeratosis and disseminated superficial actinic porokeratosis: a type 2 segmental manifestation. Acta Derm Venereol. 2007;87(4):363–4. doi:10.2340/00015555-0215.PubMedCrossRef Suarez-Amor O, Pereiro-Ferreiros M, Ginarte M, et al. Coexistence of linear porokeratosis and disseminated superficial actinic porokeratosis: a type 2 segmental manifestation. Acta Derm Venereol. 2007;87(4):363–4. doi:10.​2340/​00015555-0215.PubMedCrossRef
41.
go back to reference Sauder DN. Immunomodulatory and pharmacologic properties of imiquimod. J Am Acad Dermatol. 2000;43(1 Pt 2):S6–11.PubMedCrossRef Sauder DN. Immunomodulatory and pharmacologic properties of imiquimod. J Am Acad Dermatol. 2000;43(1 Pt 2):S6–11.PubMedCrossRef
45.
go back to reference Agarwal S, Berth-Jones J. Porokeratosis of Mibelli: successful treatment with 5% imiquimod cream. Br J Dermatol. 2002;146(2):338–9.PubMedCrossRef Agarwal S, Berth-Jones J. Porokeratosis of Mibelli: successful treatment with 5% imiquimod cream. Br J Dermatol. 2002;146(2):338–9.PubMedCrossRef
47.
go back to reference Gracia-Cazana T, Vera-Alvarez J, Garcia-Patos V, Gilaberte Y. Imiquimod and photodynamic therapy are useful in the treatment of porokeratosis in children with bone marrow transplantation. Pediatr Dermatol. 2015;32(6):e291–3. doi:10.1111/pde.12654.PubMedCrossRef Gracia-Cazana T, Vera-Alvarez J, Garcia-Patos V, Gilaberte Y. Imiquimod and photodynamic therapy are useful in the treatment of porokeratosis in children with bone marrow transplantation. Pediatr Dermatol. 2015;32(6):e291–3. doi:10.​1111/​pde.​12654.PubMedCrossRef
48.
go back to reference Erbagci Z, Tuncel AA, Erbagci I. Successful treatment of porokeratosis with topical imiquimod in 2 immunosuppressed cases. J Drugs Dermatol. 2006;5(7):668–71.PubMed Erbagci Z, Tuncel AA, Erbagci I. Successful treatment of porokeratosis with topical imiquimod in 2 immunosuppressed cases. J Drugs Dermatol. 2006;5(7):668–71.PubMed
53.
go back to reference Harrison S, Sinclair R. Porokeratosis of Mibelli: successful treatment with topical 5% imiquimod cream. Aust J Dermatol. 2003;44(4):281–3.CrossRef Harrison S, Sinclair R. Porokeratosis of Mibelli: successful treatment with topical 5% imiquimod cream. Aust J Dermatol. 2003;44(4):281–3.CrossRef
54.
go back to reference Chowdhury MM, Inaloz HS, Holt PJ. A scaly macule on the bridge of the nose of a 15-year-old boy. Pediatr Dermatol. 2000;17(2):149–50.PubMedCrossRef Chowdhury MM, Inaloz HS, Holt PJ. A scaly macule on the bridge of the nose of a 15-year-old boy. Pediatr Dermatol. 2000;17(2):149–50.PubMedCrossRef
55.
go back to reference Danby W. Treatment of porokeratosis with fluorouracil and salicylic acid under occlusion. Dermatol Online J. 2003;9(5):33.PubMed Danby W. Treatment of porokeratosis with fluorouracil and salicylic acid under occlusion. Dermatol Online J. 2003;9(5):33.PubMed
56.
go back to reference Venkatarajan S, LeLeux TM, Yang D, et al. Porokeratosis of Mibelli: successful treatment with 5 percent topical imiquimod and topical 5 percent 5-fluorouracil. Dermatol Online J. 2010;16(12):10.PubMed Venkatarajan S, LeLeux TM, Yang D, et al. Porokeratosis of Mibelli: successful treatment with 5 percent topical imiquimod and topical 5 percent 5-fluorouracil. Dermatol Online J. 2010;16(12):10.PubMed
57.
go back to reference Teixeira SP, de Nascimento MM, Bagatin E, et al. The use of fluor-hydroxy pulse peel in actinic porokeratosis. Dermatol Surg. 2005;31(9 Pt 1):1145–8.PubMedCrossRef Teixeira SP, de Nascimento MM, Bagatin E, et al. The use of fluor-hydroxy pulse peel in actinic porokeratosis. Dermatol Surg. 2005;31(9 Pt 1):1145–8.PubMedCrossRef
58.
go back to reference Zhan H, Zheng H. The role of topical cyclo-oxygenase-2 inhibitors in skin cancer: treatment and prevention. Am J Clin Derm. 2007;8(4):195–200.CrossRef Zhan H, Zheng H. The role of topical cyclo-oxygenase-2 inhibitors in skin cancer: treatment and prevention. Am J Clin Derm. 2007;8(4):195–200.CrossRef
63.
go back to reference Verma KK, Singh OP. Dexamethasone pulse treatment in disseminated porokeratosis of Mibelli. J Dermatol Sci. 1994;7(1):71–2.PubMedCrossRef Verma KK, Singh OP. Dexamethasone pulse treatment in disseminated porokeratosis of Mibelli. J Dermatol Sci. 1994;7(1):71–2.PubMedCrossRef
67.
go back to reference Simpson D, Noble S. Tacrolimus ointment: a review of its use in atopic dermatitis and its clinical potential in other inflammatory skin conditions. Drugs. 2005;65(6):827–58.PubMedCrossRef Simpson D, Noble S. Tacrolimus ointment: a review of its use in atopic dermatitis and its clinical potential in other inflammatory skin conditions. Drugs. 2005;65(6):827–58.PubMedCrossRef
69.
go back to reference Rabbin PE, Baldwin HE. Treatment of porokeratosis of Mibelli with CO2 laser vaporization versus surgical excision with split-thickness skin graft: a comparison. J Dermatol Surg Oncol. 1993;19(3):199–202.PubMedCrossRef Rabbin PE, Baldwin HE. Treatment of porokeratosis of Mibelli with CO2 laser vaporization versus surgical excision with split-thickness skin graft: a comparison. J Dermatol Surg Oncol. 1993;19(3):199–202.PubMedCrossRef
70.
go back to reference Merkle T, Hohenleutner U, Braun-Falco O, Landthaler M. Reticulate porokeratosis: successful treatment with CO2-laser vaporization. Clin Exp Dermatol. 1992;17(3):178–81.PubMedCrossRef Merkle T, Hohenleutner U, Braun-Falco O, Landthaler M. Reticulate porokeratosis: successful treatment with CO2-laser vaporization. Clin Exp Dermatol. 1992;17(3):178–81.PubMedCrossRef
73.
go back to reference McCullough TL, Lesher JL Jr. Porokeratosis of Mibelli: rapid recurrence of a large lesion after carbon dioxide laser treatment. Pediatr Dermatol. 1994;11(3):267–70.PubMedCrossRef McCullough TL, Lesher JL Jr. Porokeratosis of Mibelli: rapid recurrence of a large lesion after carbon dioxide laser treatment. Pediatr Dermatol. 1994;11(3):267–70.PubMedCrossRef
78.
go back to reference Rosenblum J, Roenigk HH Jr. Erbium laser for the treatment of disseminated superficial actinic porokeratosis: a case report. Dermatol Surg. 2013;39(10):1543–5. doi:10.1111/dsu.12261.PubMed Rosenblum J, Roenigk HH Jr. Erbium laser for the treatment of disseminated superficial actinic porokeratosis: a case report. Dermatol Surg. 2013;39(10):1543–5. doi:10.​1111/​dsu.​12261.PubMed
79.
go back to reference Liu HT. Treatment of lichen amyloidosis (LA) and disseminated superficial porokeratosis (DSP) with frequency-doubled Q-switched Nd:YAG laser. Dermatol Surg. 2000;26(10):958–62.PubMedCrossRef Liu HT. Treatment of lichen amyloidosis (LA) and disseminated superficial porokeratosis (DSP) with frequency-doubled Q-switched Nd:YAG laser. Dermatol Surg. 2000;26(10):958–62.PubMedCrossRef
81.
go back to reference Alster TS, Nanni CA. Successful treatment of porokeratosis with 585 nm pulsed dye laser irradiation. Cutis. 1999;63(5):265–6.PubMed Alster TS, Nanni CA. Successful treatment of porokeratosis with 585 nm pulsed dye laser irradiation. Cutis. 1999;63(5):265–6.PubMed
82.
go back to reference Ricci C, Rosset A, Panizzon RG. Bullous and pruritic variant of disseminated superficial actinic porokeratosis: successful treatment with grenz rays. Dermatology. 1999;199(4):328–31. doi:10.1159/000018284.PubMedCrossRef Ricci C, Rosset A, Panizzon RG. Bullous and pruritic variant of disseminated superficial actinic porokeratosis: successful treatment with grenz rays. Dermatology. 1999;199(4):328–31. doi:10.​1159/​000018284.PubMedCrossRef
83.
go back to reference Wolf P. Photodynamic therapy in dermatology: state of the art. J Eur Acad Dermatol Venereol. 2001;15(6):508–9.PubMedCrossRef Wolf P. Photodynamic therapy in dermatology: state of the art. J Eur Acad Dermatol Venereol. 2001;15(6):508–9.PubMedCrossRef
85.
86.
go back to reference Calzavara-Pinton PG, Rossi MT, Italian Group For Photodynamic T, et al. A retrospective analysis of real-life practice of off-label photodynamic therapy using methyl aminolevulinate (MAL-PDT) in 20 Italian dermatology departments. Part 1: inflammatory and aesthetic indications. Photochem Photobiol Sci. 2013;12(1):148–57. doi:10.1039/c2pp25124h.PubMedCrossRef Calzavara-Pinton PG, Rossi MT, Italian Group For Photodynamic T, et al. A retrospective analysis of real-life practice of off-label photodynamic therapy using methyl aminolevulinate (MAL-PDT) in 20 Italian dermatology departments. Part 1: inflammatory and aesthetic indications. Photochem Photobiol Sci. 2013;12(1):148–57. doi:10.​1039/​c2pp25124h.PubMedCrossRef
89.
93.
go back to reference Nayeemuddin FA, Wong M, Yell J, Rhodes LE. Topical photodynamic therapy in disseminated superficial actinic porokeratosis. Clin Exp Dermatol. 2002;27(8):703–6.PubMedCrossRef Nayeemuddin FA, Wong M, Yell J, Rhodes LE. Topical photodynamic therapy in disseminated superficial actinic porokeratosis. Clin Exp Dermatol. 2002;27(8):703–6.PubMedCrossRef
95.
go back to reference Salas T, Hernandez-Gil J, Lopez A, et al. Two cases of disseminated superficial actinic porokeratosis treated with daylight-mediated photodynamic therapy. Dermatol Ther. 2016;29(6):484–5. doi:10.1111/dth.12389.PubMedCrossRef Salas T, Hernandez-Gil J, Lopez A, et al. Two cases of disseminated superficial actinic porokeratosis treated with daylight-mediated photodynamic therapy. Dermatol Ther. 2016;29(6):484–5. doi:10.​1111/​dth.​12389.PubMedCrossRef
97.
go back to reference Spencer JM, Katz BE. Successful treatment of porokeratosis of Mibelli with diamond fraise dermabrasion. Arch Dermatol. 1992;128(9):1187–8.PubMedCrossRef Spencer JM, Katz BE. Successful treatment of porokeratosis of Mibelli with diamond fraise dermabrasion. Arch Dermatol. 1992;128(9):1187–8.PubMedCrossRef
98.
go back to reference Cohen PR, Held JL, Katz BE. Linear porokeratosis: successful treatment with diamond fraise dermabrasion. J Am Acad Dermatol. 1990;23(5 Pt 2):975–7.PubMedCrossRef Cohen PR, Held JL, Katz BE. Linear porokeratosis: successful treatment with diamond fraise dermabrasion. J Am Acad Dermatol. 1990;23(5 Pt 2):975–7.PubMedCrossRef
100.
go back to reference Otani Y, Katagiri K, Takeuchi Y, et al. A case of giant porokeratosis with vestiges of a cornoid lamella. J Dermatol. 2005;32(11):894–8.PubMedCrossRef Otani Y, Katagiri K, Takeuchi Y, et al. A case of giant porokeratosis with vestiges of a cornoid lamella. J Dermatol. 2005;32(11):894–8.PubMedCrossRef
102.
go back to reference Robinson JB, Im DD, Jockle G, Rosenshein NB. Vulvar porokeratosis: case report and review of the literature. Int J Gynecol Pathol. 1999;18(2):169–73.PubMedCrossRef Robinson JB, Im DD, Jockle G, Rosenshein NB. Vulvar porokeratosis: case report and review of the literature. Int J Gynecol Pathol. 1999;18(2):169–73.PubMedCrossRef
104.
go back to reference Levell NJ, Bewley AP, Levene GM. Porokeratosis of Mibelli on the penis, scrotum and natal cleft. Clin Exp Dermatol. 1994;19(1):77–8.PubMedCrossRef Levell NJ, Bewley AP, Levene GM. Porokeratosis of Mibelli on the penis, scrotum and natal cleft. Clin Exp Dermatol. 1994;19(1):77–8.PubMedCrossRef
105.
go back to reference Bhushan M, Craven NM, Beck MH, Chalmers RJ. Linear porokeratosis of Mibelli: successful treatment with cryotherapy. Br J Dermatol. 1999;141(2):389.PubMedCrossRef Bhushan M, Craven NM, Beck MH, Chalmers RJ. Linear porokeratosis of Mibelli: successful treatment with cryotherapy. Br J Dermatol. 1999;141(2):389.PubMedCrossRef
106.
go back to reference Vergara G, Banuls J, Botella R, et al. Porokeratosis of the lower lip. Eur J Dermatol. 2002;12(5):500–2.PubMed Vergara G, Banuls J, Botella R, et al. Porokeratosis of the lower lip. Eur J Dermatol. 2002;12(5):500–2.PubMed
107.
go back to reference Goncalves SV, Valente NY, Oliveira Jrunior JV, Paiva DL. Case for diagnosis. An Braz Dermatol. 2012;87(3):489–90.CrossRef Goncalves SV, Valente NY, Oliveira Jrunior JV, Paiva DL. Case for diagnosis. An Braz Dermatol. 2012;87(3):489–90.CrossRef
108.
go back to reference Dereli T, Ozyurt S, Ozturk G. Porokeratosis of Mibelli: successful treatment with cryosurgery. J Dermatol. 2004;31(3):223–7.PubMedCrossRef Dereli T, Ozyurt S, Ozturk G. Porokeratosis of Mibelli: successful treatment with cryosurgery. J Dermatol. 2004;31(3):223–7.PubMedCrossRef
Metadata
Title
Treatment of Porokeratosis: A Systematic Review
Authors
Till Weidner
Tanja Illing
Diana Miguel
Peter Elsner
Publication date
01-08-2017
Publisher
Springer International Publishing
Published in
American Journal of Clinical Dermatology / Issue 4/2017
Print ISSN: 1175-0561
Electronic ISSN: 1179-1888
DOI
https://doi.org/10.1007/s40257-017-0271-3

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