bs_bs_banner
Australasian Journal of Dermatology (2015) 56, e63–e65
doi: 10.1111/ajd.12232
BRIEF REPORT
Successful treatment of Dowling-Degos disease using intense pulsed light Aakriti Gupta and Shyamala C Huilgol Adelaide Skin and Eye Centre, University of Adelaide and Royal Adelaide Hospital, Adelaide, South Australia, Australia
ABSTRACT Dowling-Degos disease (DDD) is a rare inherited pigmentary disorder characterised by reticulate pigmentation at flexural sites. No treatment modality has yet been established as a definitive method for the effective elimination of lentigines in DDD. We present a case of a 24-year old woman with DDD treated successfully using intense pulsed light as a novel management strategy. Key words: Dowling-Degos disease, intense pulsed light.
yttrium aluminium garnet (Er:YAG) laser.3,4 Concerns remain, however, about the potential risks of ablative laser treatment including post-laser hyperpigmentation, long recovery periods and scarring. Yun and colleagues then described the use of fractional Er:YAG in a woman with dark, Asian skin, with the aim of reducing the risk of postinflammatory hyperpigmentation.5 While this was effective in reducing postoperative inflammation, the authors acknowledged that this latter method could not produce the therapeutic results of an ablative approach. Optimising the treatment of lentigines in DDD while minimising the associated risks thus remains a therapeutic challenge. Here, we describe our experience of a young woman with DDD who was successfully treated with IPL as a novel management strategy.
CASE REPORT INTRODUCTION Intense pulsed light (IPL) is a promising and readily accessible therapeutic option for Dowling-Degos disease (DDD), a rare pigmentary disorder characterised by reticulate pigmentation at flexural sites. This condition has an autosomal dominant mode of inheritance and is due to disturbed epidermal proliferation, linked to mutations in the keratin 5 gene.1,2 No treatment modality has yet been established as a definitive method for the effective elimination of lentigines in DDD. Medical therapy has been unsuccessful, including the use of topical corticosteroids, depigmentating agents such as hydroquinone, topical and systemic retinoids, and adapalene.1,3 There have been case reports using laser treatment to remove the abnormal epidermal tissue in DDD. Wenzel and colleagues reported a case of DDD that was treated successfully using an ablative erbium-doped
Correspondence: Associate Professor Shyamala C Huilgol, Adelaide Skin and Eye Centre, 89 King William St, Kent Town, SA 5067, Australia. Email: [email protected] Aakriti Gupta. Shyamala C Huilgol, FACD. Conflict of interest: none Submitted 8 April 2014; accepted 15 June 2014. © 2014 The Australasian College of Dermatologists
A 24-year old woman presented with numerous lentigines of childhood onset. These appeared initially in the popliteal and antecubital fossae, progressing to involve the axillae, anterolateral neck and décolletage (Fig. 1). There were no other cutaneous lesions, in particular no café au lait patches or cutaneous neurofibromas. Her past medical history was significant only for a small ventricular septal defect noted in the neonatal period that closed spontaneously. There was no family history of a similar skin condition. A subsequent histological examination of a lesional skin biopsy revealed elongated focally branching rete ridges with hyperpigmentation at the rete tips. In addition, perivascular lymphohistiocytic infiltration was observed. These histological features in combination with the clinical picture led to the diagnosis of DDD. Test treatment with IPL was successfully and safely carried out in the antecubital and popliteal fossae with a 15 × 30-mm spot size with 515-nm and 560-nm filters, fluences of 8 and 10 J/cm2 with each filter and a 10 msec
Abbreviations: DDD Er:YAG IPL
Dowling-Degos disease erbium-doped yttrium aluminium garnet intense pulsed light
e64
A Gupta and SC Huilgol
Figure 1 Reticulate pigmentation of the popliteal fossa before treatment.
duration. Two complete treatment sessions to the popliteal fossae were performed over 4 months using a single pass with the 15 × 30-mm spot, 560-nm filter, fluences ranging from 6 to 10 J/cm2, and a pulse duration of 10 msec. The greatest response was seen with higher fluences. She was also advised on sun protection measures, as exposure to UV radiation prior to the procedure would decrease treatment efficacy and result in re-pigmentation following treatment. Photographs were taken using a standard digital camera at baseline and after each treatment session to assess the clinical effects of IPL on the appearance of the lentigines. There was an excellent clinical response to treatment, with a marked reduction in the pigmentation of lentigines present after two treatment sessions with IPL (Fig. 2). No adverse effects were noted in the 7-month follow-up period. In particular, there was no observed scarring, postinflammatory hyperpigmentation or hypopigmentation. A sustained response to treatment was demonstrated, with no recurrence of treated lentigines. The subsequent treatment of other affected areas is planned for the future.
DISCUSSION The ideal treatment strategy to effectively and safely ameliorate reticulate pigmentation in DDD is yet to be established. In the case presented, IPL was used as an innovative approach to successfully treat lentigines without any adverse effects. Previously, a number of medical therapies have been trialed with minimal or temporary effect.1,3 Subsequently, © 2014 The Australasian College of Dermatologists
Figure 2 Marked reduction in the pigmentation of lentigines after two complete treatment sessions with intense pulsed light.
with the increasingly widespread application of laser technology, ablative laser treatment was proposed as a therapeutic strategy.3 By removing epidermal abnormalities in DDD, laser treatment enables the reformation of new epidermal tissue from unaffected follicular epithelium. More recently, fractional Er:YAG has been used to treat DDD and reduce the risk of adverse effects associated with ablative laser. While a fractional technique allows rapid re-epithelialisation and reduces inflammation, the sparing of tissue around microthermal treatment zones limits its efficacy.5 We chose to trial IPL to treat the lentigines in this patient with DDD due to its excellent safety profile and established efficacy in the treatment of pigmented lesions. The good therapeutic results and minimal adverse effects seen here after treatment with IPL are likely to be due to selective photothermolysis. This means that thermally mediated radiation damage in IPL can be confined to the pigmented epidermal targets, sparing surrounding tissue and thus minimising nonspecific widespread thermal injury. Applying these findings, one would expect that pigment lasers treating other forms of lentigines, including frequencydoubled neodymium-doped YAG (either standard mode or Q-switched) and the pulsed dye laser with a compression handpiece might be just as effective as IPL treatment. Although the results in our patient have been sustained, it is likely that exposure to UV radiation would result in re-pigmentation and patients need to be advised of this before undertaking treatment.
CONCLUSION The case presented here suggests that IPL may be an effective and safe therapeutic strategy for the treatment of DDD.
Treatment of DDD using IPL Prospective studies with larger patient cohorts of this rare pigmentary disorder are required, however, to confirm our observations.
3.
4.
REFERENCES 1.
2.
Horner ME, Parkinson KE, Kaye V et al. Dowling-Degos disease involving the vulva and back: case report and review of the literature. Dermatol. Online J. 2011; 17: 1. Batycka-Baran A, Baran W, Hryncewicz-Gwozdz A et al. Dowling-Degos disease: case report and review of the literature. Dermatology 2010; 220: 254–8.
5.
e65
Wenzel G, Petrow W, Tappe K et al. Treatment of DowlingDegos disease with Er:YAG-laser: results after 2.5 years. Dermatol. Surg. 2003; 29: 1161–2. Wenzel J, Tappe K, Gerdsen R et al. Successful treatment of Dowling–Degos disease with Er:YAG laser. Dermatol. Surg. 2002; 28: 748–50. Yun JH, Kim JH, Choi JS et al. Treatment of Dowling-Degos disease with fractional Er:YAG laser. J. Cosmet. Laser Ther. 2013; 15: 336–9.
© 2014 The Australasian College of Dermatologists
Australasian Journal of Dermatology (2015) 56, e63–e65
doi: 10.1111/ajd.12232
BRIEF REPORT
Successful treatment of Dowling-Degos disease using intense pulsed light Aakriti Gupta and Shyamala C Huilgol Adelaide Skin and Eye Centre, University of Adelaide and Royal Adelaide Hospital, Adelaide, South Australia, Australia
ABSTRACT Dowling-Degos disease (DDD) is a rare inherited pigmentary disorder characterised by reticulate pigmentation at flexural sites. No treatment modality has yet been established as a definitive method for the effective elimination of lentigines in DDD. We present a case of a 24-year old woman with DDD treated successfully using intense pulsed light as a novel management strategy. Key words: Dowling-Degos disease, intense pulsed light.
yttrium aluminium garnet (Er:YAG) laser.3,4 Concerns remain, however, about the potential risks of ablative laser treatment including post-laser hyperpigmentation, long recovery periods and scarring. Yun and colleagues then described the use of fractional Er:YAG in a woman with dark, Asian skin, with the aim of reducing the risk of postinflammatory hyperpigmentation.5 While this was effective in reducing postoperative inflammation, the authors acknowledged that this latter method could not produce the therapeutic results of an ablative approach. Optimising the treatment of lentigines in DDD while minimising the associated risks thus remains a therapeutic challenge. Here, we describe our experience of a young woman with DDD who was successfully treated with IPL as a novel management strategy.
CASE REPORT INTRODUCTION Intense pulsed light (IPL) is a promising and readily accessible therapeutic option for Dowling-Degos disease (DDD), a rare pigmentary disorder characterised by reticulate pigmentation at flexural sites. This condition has an autosomal dominant mode of inheritance and is due to disturbed epidermal proliferation, linked to mutations in the keratin 5 gene.1,2 No treatment modality has yet been established as a definitive method for the effective elimination of lentigines in DDD. Medical therapy has been unsuccessful, including the use of topical corticosteroids, depigmentating agents such as hydroquinone, topical and systemic retinoids, and adapalene.1,3 There have been case reports using laser treatment to remove the abnormal epidermal tissue in DDD. Wenzel and colleagues reported a case of DDD that was treated successfully using an ablative erbium-doped
Correspondence: Associate Professor Shyamala C Huilgol, Adelaide Skin and Eye Centre, 89 King William St, Kent Town, SA 5067, Australia. Email: [email protected] Aakriti Gupta. Shyamala C Huilgol, FACD. Conflict of interest: none Submitted 8 April 2014; accepted 15 June 2014. © 2014 The Australasian College of Dermatologists
A 24-year old woman presented with numerous lentigines of childhood onset. These appeared initially in the popliteal and antecubital fossae, progressing to involve the axillae, anterolateral neck and décolletage (Fig. 1). There were no other cutaneous lesions, in particular no café au lait patches or cutaneous neurofibromas. Her past medical history was significant only for a small ventricular septal defect noted in the neonatal period that closed spontaneously. There was no family history of a similar skin condition. A subsequent histological examination of a lesional skin biopsy revealed elongated focally branching rete ridges with hyperpigmentation at the rete tips. In addition, perivascular lymphohistiocytic infiltration was observed. These histological features in combination with the clinical picture led to the diagnosis of DDD. Test treatment with IPL was successfully and safely carried out in the antecubital and popliteal fossae with a 15 × 30-mm spot size with 515-nm and 560-nm filters, fluences of 8 and 10 J/cm2 with each filter and a 10 msec
Abbreviations: DDD Er:YAG IPL
Dowling-Degos disease erbium-doped yttrium aluminium garnet intense pulsed light
e64
A Gupta and SC Huilgol
Figure 1 Reticulate pigmentation of the popliteal fossa before treatment.
duration. Two complete treatment sessions to the popliteal fossae were performed over 4 months using a single pass with the 15 × 30-mm spot, 560-nm filter, fluences ranging from 6 to 10 J/cm2, and a pulse duration of 10 msec. The greatest response was seen with higher fluences. She was also advised on sun protection measures, as exposure to UV radiation prior to the procedure would decrease treatment efficacy and result in re-pigmentation following treatment. Photographs were taken using a standard digital camera at baseline and after each treatment session to assess the clinical effects of IPL on the appearance of the lentigines. There was an excellent clinical response to treatment, with a marked reduction in the pigmentation of lentigines present after two treatment sessions with IPL (Fig. 2). No adverse effects were noted in the 7-month follow-up period. In particular, there was no observed scarring, postinflammatory hyperpigmentation or hypopigmentation. A sustained response to treatment was demonstrated, with no recurrence of treated lentigines. The subsequent treatment of other affected areas is planned for the future.
DISCUSSION The ideal treatment strategy to effectively and safely ameliorate reticulate pigmentation in DDD is yet to be established. In the case presented, IPL was used as an innovative approach to successfully treat lentigines without any adverse effects. Previously, a number of medical therapies have been trialed with minimal or temporary effect.1,3 Subsequently, © 2014 The Australasian College of Dermatologists
Figure 2 Marked reduction in the pigmentation of lentigines after two complete treatment sessions with intense pulsed light.
with the increasingly widespread application of laser technology, ablative laser treatment was proposed as a therapeutic strategy.3 By removing epidermal abnormalities in DDD, laser treatment enables the reformation of new epidermal tissue from unaffected follicular epithelium. More recently, fractional Er:YAG has been used to treat DDD and reduce the risk of adverse effects associated with ablative laser. While a fractional technique allows rapid re-epithelialisation and reduces inflammation, the sparing of tissue around microthermal treatment zones limits its efficacy.5 We chose to trial IPL to treat the lentigines in this patient with DDD due to its excellent safety profile and established efficacy in the treatment of pigmented lesions. The good therapeutic results and minimal adverse effects seen here after treatment with IPL are likely to be due to selective photothermolysis. This means that thermally mediated radiation damage in IPL can be confined to the pigmented epidermal targets, sparing surrounding tissue and thus minimising nonspecific widespread thermal injury. Applying these findings, one would expect that pigment lasers treating other forms of lentigines, including frequencydoubled neodymium-doped YAG (either standard mode or Q-switched) and the pulsed dye laser with a compression handpiece might be just as effective as IPL treatment. Although the results in our patient have been sustained, it is likely that exposure to UV radiation would result in re-pigmentation and patients need to be advised of this before undertaking treatment.
CONCLUSION The case presented here suggests that IPL may be an effective and safe therapeutic strategy for the treatment of DDD.
Treatment of DDD using IPL Prospective studies with larger patient cohorts of this rare pigmentary disorder are required, however, to confirm our observations.
3.
4.
REFERENCES 1.
2.
Horner ME, Parkinson KE, Kaye V et al. Dowling-Degos disease involving the vulva and back: case report and review of the literature. Dermatol. Online J. 2011; 17: 1. Batycka-Baran A, Baran W, Hryncewicz-Gwozdz A et al. Dowling-Degos disease: case report and review of the literature. Dermatology 2010; 220: 254–8.
5.
e65
Wenzel G, Petrow W, Tappe K et al. Treatment of DowlingDegos disease with Er:YAG-laser: results after 2.5 years. Dermatol. Surg. 2003; 29: 1161–2. Wenzel J, Tappe K, Gerdsen R et al. Successful treatment of Dowling–Degos disease with Er:YAG laser. Dermatol. Surg. 2002; 28: 748–50. Yun JH, Kim JH, Choi JS et al. Treatment of Dowling-Degos disease with fractional Er:YAG laser. J. Cosmet. Laser Ther. 2013; 15: 336–9.
© 2014 The Australasian College of Dermatologists
