Journal of Dermatology 2015; 42: 1053–1057
doi: 10.1111/1346-8138.13002
ORIGINAL ARTICLE
Clinical and histological aspect of erythema dyschromicum perstans in Korea: A review of 68 cases Sung Eun CHANG,1,* Hyun Woo KIM,2,* Jae Min SHIN,2 Ji Hyun LEE,1 Jung Im NA,3 Mi Ryung ROH,4 Jong Hee LEE,5 Ga Young LEE,6 Joo Yeon KO2 1
Department of Dermatology, Asan Medical Center, Ulsan University College of Medicine, 2Department of Dermatology, Hanyang University College of Medicine, Seoul, 3Department of Dermatology, Seoul National University Bundang Hospital, Seongnam, 4 Department of Dermatology, Gangnam Severance Hospital, Yonsei University College of Medicine, 5Department of Dermatology, Samsung Medical Center, Sungkyunkwan University School of Medicine, 6Department of Dermatology, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul, Korea
ABSTRACT Erythema dyschromicum perstans (EDP) is a hypermelanotic disorder of the idiopathic variety characterized by blue-gray macules in healthy individuals. It has been described mainly in patients from tropical areas of Central and South America. Our aim was to evaluate EDP in Korea through the analysis of retrospective case series with EDP and to describe the clinical and histopathological features. It was a retrospective study (2002–2012) of EDP confirmed by review of clinical photographs and biopsy specimens. The files of 68 patients with EDP from six tertiary medical centers in Korea were included in this study. Of the 68 patients, 29 were male and 39 female. The age of patients ranged 3–76 years (mean, 33.9). Clinically, the majority of patients had lesions on the trunk (n = 47, 69.1%); the neck was affected in 27 cases, the upper extremities in 26, the face in 22 and the lower extremities in 16. Peripheral erythematous borders were observed in 12 patients (17.6%) and 11 patients had itching sensation at the lesion. Histopathologically, dermal melanophages and pigment incontinence were the most common findings. A follow up of more than 1 year was obtained in 51 patients. Of these, only one patient experienced complete clearance without recurrence. Our study illustrates the clinical and histopathological findings of EDP in Koreans. Our multicenter data may contribute to the understanding of EDP.
Key words: Korean.
ashy dermatosis, clinical feature, erythema dyschromicum perstans, histopathological feature,
INTRODUCTION Erythema dyschromicum perstans (EDP) was first described by Ramirez in 1957.1 EDP, which is also known as ashy dermatosis, is a hypermelanotic disorder of healthy individuals clinically characterized by blue-gray macules. EDP occurs most frequently in Central and South America and the South Central USA, although cases have been described from different parts of the world.2 It mostly occurs in the second decade of life and commonly affects the face, arms, neck and trunk with symmetrical distribution; however, unilateral presentation has also been observed.3 The salient histopathological features were vacuolar degeneration of the basal cell layer with dermal melanosis and a perivascular infiltration. As time passes, the inflammatory infiltrate is usually diminished but prominent melanin incontinence exists with abundant melanophages. Although the features of EDP are somewhat investigated, the data on Asian EDP patients is limited. The aim of this study
was to assess the clinical and histopathological features of EDP in Korean patients through the analysis of a retrospective case series.
METHODS This study was conducted at the six university hospitals in Korea. We retrospectively included all patients with EDP referred to the hospitals during the period between 2002 and 2012. The diagnosis of EDP was made using the following clinical and histopathological 4-item criteria: (i) eruption of multiple blue-gray macules or patches that sometimes have erythematous and slightly elevated borders; (ii) histopathologically compatible with EDP; (iii) absence of pigmentationrelated underlying diseases and drug history; and (iv) no previous history of contact dermatitis confirmed by patch test. Mucosal involvement was not seen and the lesions were usually asymptomatic in the patients. After review of the clinical
Correspondence: Joo Yeon Ko, M.D., Ph.D., Department of Dermatology, Hanyang University Hospital, Seoul 133-792, South Korea. Email: [email protected] *These authors contributed equally to this work and should be considered as joint first authors. Received 25 March 2015; accepted 16 May 2015.
© 2015 Japanese Dermatological Association
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S.E. Chang et al.
Table 1. Demographic features of EDP
Age, years 0–9 10–19 20–29 30–39 40–49 50–59 ≥60 Sex Male Female Fitzpatrick skin type III IV V Location Trunk Neck Upper extremity Face Lower extremity Erythematous border Itching sensation
(a)
n
%
5 10 12 14 9 9 9
7.3 14.7 17.6 20.6 13.2 13.2 13.2
28 38
41.1 55.9
7 56 5
10.3 82.4 7.4
47 27 26 22 16 12 11
69.1 39.7 38.2 32.4 23.5 17.6 16.2
(b)
(c)
and histopathological findings, EDP was identified in 68 patients.
RESULTS Of the 68 patients, 29 were male and 39 were female. The age of onset of the lesion ranged 3–76 years. The mean age was 33.9 years. The majority of patients were of Fitzpatrick skin type IV (82.4%), followed by III (10.3%) and V (7.4%). Peripheral erythematous borders were observed in 12 patients (17.6%) and 11 patients had itching sensation and inflammation signs at the lesion on initiation of the lesions (16.1%) (Table 1). Clinically, the majority of patients had lesions on the trunk with 47 cases (69.1%); the neck was affected in 26 cases (38.2%), the upper limbs in 27 (39.7%), the face in 22 (32.4%) and the lower limbs in 16 (23.5%) (Fig. 1). Although the causal relationship between medication and EDP is uncertain, drug medications (clomiphene, oral estrogen pill and herbal medicine) were suspected as preceding causes of EDP in three patients. Histopathological evaluation of specimens from the 68 patients showed dermal melanophages and pigment incontinence in 57 cases (83.8%), mild to severe lymphocytic infiltration in 50 (73.5%), increased epidermal melanin in 40 (58.8%), vacuolar degeneration of the basal layer in 33 (48.5%), colloid
(g)
(h)
(d)
(e)
(i) (f)
Figure 1. Clinical photographs of (a,b) female patient aged 31 years, (c) female patient aged 15, (d) male patient aged 76, (e) female patient aged 15 and (f) male patient aged 68. Erythematous skin lesions were observed in (g,h) male patient aged 36 and (i) female patient aged 34.
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© 2015 Japanese Dermatological Association
Erythema dyschromicum perstans in Korean
bodies in 18 (26.5%), band-like lymphocytic infiltration in 13 (19.1%) and papillary dermal edema in 12 (17.6%) (Figs 2,3). Topical steroid was the most commonly used agent (29 cases, 42.6%), followed by triple combination cream (fluocinolone acetonide 0.01%, hydroquinone 4%, tretinoin 0.05%) (14 cases, 20.6%), topical hydroquinone (12 cases, 17.6%), topical calcineurin inhibitor (10 cases, 14.7%) and topical tretinoin (five cases, 7.4%). In 16 patients, they received systemic treatment including dapsone (four cases, 5.9%), minocycline (three cases, 4.4%), tranexamic acid and vitamins (three cases, 4.4%), clofazimine (two cases, 2.9%), pentoxifylline (two cases, 2.9%) and macrolide (two cases, 2.9%). A follow up of more than 1 year (range, 1–10 years; mean follow-up period, 17.9 months) was obtained in 51 patients (Table 2). Of these, only one patient (2.0%), a 3-year-old female, had shown clearing after 33 months. Twenty-two patients (43.1%) showed slight to fair improvement. They reflected that the lesions grad-
n 60
57 50
50 40 40
33
30 18
20
13
12
10 0 Dermal Lymphohismelanophages ocyc & pigment infiltraon inconnence
Increased epidermal melanin
Vacuolar degeneraon of basal layer
Colloid body
Band-like Papillary infiltraon dermal edema
Figure 2. Histopathological analysis of erythema dyschromicum perstans.
ually diminished in 3–5 years. In three patients (5.9%), lesions aggravated during the follow-up period, and the rest (49.0%) did not show any significant change. One patient, a 35-yearold male, had shown aggravation of the lesions after Q-switched 1064-nm neodymium:yttrium–aluminum–garnet laser treatment.
DISCUSSION Erythema dyschromicum perstans is an acquired, disfiguring skin disease that occurs most commonly in Latin American and Asian populations. Despite differences in ethnic background and etiology, clinical features of EDP are clinically similar in most patients. The disease spreads centrifugally and has characteristic blue-gray oval macules and patches that range in diameter 0.5–3 cm in all ethnic groups.2 In certain cases, some of the lesions had an easily observable, non-elevated, erythematous border which cleared after a variable period of time.4,5 However, it is difficult to identify the active phase and the erythematous border is infrequently observed despite the nomenclature of the disease.6,7 With regard to skin color, darkcolored individuals (Fitzpatrick types IV–V) are most commonly affected. In this study, the majority patients were of skin type IV (82.4%) and this result was consistent with previous studies. However, in contrast with EDP commonly affecting patients under 30 years old, the peak age of our patients was fourth decade followed by third decade (mean age, 33.9 years). This difference may be due to racial difference or diagnostic delay. The most common locations are on the trunk, arms and face. Although there is no clear sexual predilection, some authors report that EDP may be more common in females and
(a)
(b)
(c)
(d)
Figure 3. Histopathological findings of erythema dyschromicum perstans. (a) Dermal pigmentation, (b) lymphohistiocytic infiltration, (c) vacuolar degeneration and (d) papillary dermal edema (hematoxylin–eosin, original magnification 9200).
© 2015 Japanese Dermatological Association
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S.E. Chang et al.
Table 2. Follow-up period
10 years Total
n
%
17 21 13 8 4 2 0 3 68
25.0 30.9 19.1 11.8 5.9 2.9 0 4.4 100
our study shows female predominance.8,9 Except for occasional slight itching, EDP lesions remain symptomless. Because the lesion is symptomless, patients regard it as a cosmetic problem. We assumed that the sexual difference of concerns about appearance may have led this result. Histopathologically, EDP can be subdivided into active (or early) and inactive (or late) lesions. The active lesion shows basal vacuolar degeneration and edematous papillary dermis accompanied by lymphocytic infiltration. The inactive lesion shows melanophages and pigment incontinence in the dermis without inflammation in general. Although an erythematous border is known as a characteristic feature of EDP, it was only found in 17.6% (12/68) of our patients. Also, histopathological analysis of lesions showed basal layer vacuolization in 48.5% (33/68) of our patients, which is lower than the 85% reported by Vega et al.10 and 58% reported by Vasquez-Ochaoa et al.11 Tschen et al.12 and Leonforte and Pelaez13 described the presence of an active margin in patients with EDP of short duration. Probably, Korean EDP patients visited hospital beyond the initial stage of the disease or there was a referral bias due to tertiary medical center. To date, no effective standard treatment is available, although some good results with clofazimine13,14 and dapsone15,16 have been reported in small case series. Based on the fact that inflammation and pigmentation can be treated with topical steroids and depigmenting agents, these agents were most frequently used in this study. However, the lesion disappeared in only one patient. This implies that EDP is a chronic disease, as indicated by its name. Erythema dyschromicum perstans is a controversial entity and is sometimes considered as lichen planus pigmentosus (LPP)9,17,18 or idiopathic eruptive macular pigmentation (IEMP).19 Sometimes, EDP, LPP and IEMP are considered as the same disease.20 Many patients with LPP may show residual hyperpigmentation resembling that of EDP, which shows histopathological lichenoid features. The following list of items may be helpful to differentiate LP: (i) except for occasional slight itching, they remain symptomless; (ii) some of the lesions have an easily observable, non-elevated, erythematous border; (iii) the lesions appear either on exposed or unexposed areas alike; (iv) EDP does not involve mucosa; and (v) the lesion rarely improves. IEMP is a rare disorder of pigmentation first described by Degos et al. The following list of items are the
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criteria for diagnosis of IEMP: (i) eruption of brownish, nonconfluent, asymptomatic macules involving the trunk, neck and proximal extremities in children and adolescents; (ii) absence of preceding inflammatory lesions; (iii) no previous drug exposure; (iv) basal layer hyperpigmentation of the epidermis and prominent dermal melanophages without visible layer damage or lichenoid inflammatory infiltrate; and (v) normal mast cell count. However, these criteria cannot differentiate between EDP and IEMP in all cases. The main limitations of the present study are a retrospective approach, small number of study patients and referral bias. Despite this, it adds clinical characteristics of EDP in an Asian population from Korea. Our study could contribute to the understanding of the clinicohistopathological features and clinical course of EDP. Furthermore, we expect that leading diagnostic criteria will be established in the near future.
ACKNOWLEDGMENT:
We thank to Shin Ok Kim, AESTURA, for collection of data. Author contribution as follows. All authors have contributed significantly and are in agreement with the content of the manuscript.
CONFLICT OF INTEREST:
None.
REFERENCES 1 Ramirez CO. Los cenescientos: problema clinic. Proceedings of the first central American congress of dermatology. San Salvador: El Salvador, 1957; 122–130. 2 Silverberg NB, Herz J, Wagner AL, Paller AS. Erythema dyschromicum perstans in prepubertal children. Pediatr Dermatol 2003; 20: 398–403. 3 Imanishi H, Tsuruta D, Kobayashi H, Ishii M, Nakagawa K. Two cases of unilateral ashy dermatosis. Case Rep Dermatol 2011; 3: 1– 4. 4 Zaynoun S, Rubeiz N, Kibbi AG. Ashy dermatoses – a critical review of the literature and a proposed simplified clinical classification. Int J Dermatol 2008; 47: 542–544. 5 Lapeere H, Boone B, Schepper SD et al. Hypomelanosis and hypermelanosis. In: Wolff K, Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ, eds. Fitzpatricks Dermatology in General Medicine, 8th edn. New York: McGraw-Hill, 2012; 824– 825. 6 Ono S, Miyachi Y, Kabashima K. Ashy dermatosis with prior pruritic and scaling skin lesions. J Dermatol 2012; 39: 1103–1104. 7 Schwartz RA. Erythema dyschromium perstans: the continuing enigma of Cinderella or ashy dermatosis. Int J Dermatol 2004; 43: 230–232. 8 Ramirez O, Lopez Lino DG. Current status of ashy dermatosis. Synonym–erythema dyschromicum perstans. Med Cutan Ibero Lat Am 1984; 12: 11–18. 9 Pandya AG, Guevara IL. Disorders of hyperpigmentation. Dermatol Clin 2000; 18: 91–98. 10 Vega ME, Waxtein L, Arenas R, Hojyo T, Dominguez-Soto L. Ashy dermatosis and lichen planus pigmentosus: a clinicopathologic study of 31 cases. Int J Dermatol 1992b; 31: 90–94. squez-Ochoa LA, Isaza-Guzma n DM, Orozco-Mora B, Restrepo11 Va Molina R, Trujillo-Perez J, Tapia FJ. Immunopathologic study of erythema dyschromicum perstans (ashy dermatosis). Int J Dermatol 2006; 45: 937–941.
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Erythema dyschromicum perstans in Korean
12 Tschen JA, Tschen EA, McGavran MH. Erythema dyschromicum perstans. J Am Acad Dermatol 1980; 2: 295–302. rez-Alfonzo R, Abrusci V et al. Clinical trial with 13 Piquero-Martın J, Pe clofazimine for treating erythema dyschromicum perstans. Evaluation of cell-mediated immunity. Int J Dermatol 1989; 28: 198–200. 14 Baranda L, Torres-Alvarez B, Cortes-Franco R, Moncada B, Portelas-Perez DP, Gonzalez-Amaro R. Involvement of cell adhesion and activation molecules in the pathogenesis of erythema dyschromicum perstans (ashy dermatitis). The effect of clofazimine therapy. Arch Dermatol 1997; 133: 325–329. 15 Bahadir S, Cobanoglu U, Cimsit G, Yayli S, Alpay K. Erythema dyschromicum perstans: response to dapsone therapy. Int J Dermatol 2004; 43: 220–222. 16 Kontochristopoulos G, Stavropoulos P, Panteleos D, Aroni K. Erythema dyschromicum perstans: response to dapsone therapy. Int J Dermatol 1998; 37: 796–798.
© 2015 Japanese Dermatological Association
17 Gadenne BM, Camisa C. Lichenoid dermatitides (lichen planus, keratosis lichenoides and erythema dischromicum perstans). In: Arndt K, Leboit P, Robinson J, eds. Cutaneous Medicine and Surgery, 1st edn. Philadelphia: W.B.Saunders, 1996; 241. ez de di Bari O. Erythema dyschromicum perstans 18 Leonforte JL, Pela versus lichen planus. Med Cutan Ibero Lat Am 1987; 15: 89–92. 19 Torrelo A, Zaballos P, Colmenero I, Mediero IG, de Prada I, Zambrano A. Erythema dyschromicum perstans in children: a report of 14 cases. J Eur Acad Dermatol Venereol 2005; 19: 422–426. 20 Oiso N, Tsuruta D, Imanishi H, Kobayashi H, Kawada A. Erythema dyschromicum perstans in a Japanese child. Pediatr Dermatol 2012; 29: 637–640.
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doi: 10.1111/1346-8138.13002
ORIGINAL ARTICLE
Clinical and histological aspect of erythema dyschromicum perstans in Korea: A review of 68 cases Sung Eun CHANG,1,* Hyun Woo KIM,2,* Jae Min SHIN,2 Ji Hyun LEE,1 Jung Im NA,3 Mi Ryung ROH,4 Jong Hee LEE,5 Ga Young LEE,6 Joo Yeon KO2 1
Department of Dermatology, Asan Medical Center, Ulsan University College of Medicine, 2Department of Dermatology, Hanyang University College of Medicine, Seoul, 3Department of Dermatology, Seoul National University Bundang Hospital, Seongnam, 4 Department of Dermatology, Gangnam Severance Hospital, Yonsei University College of Medicine, 5Department of Dermatology, Samsung Medical Center, Sungkyunkwan University School of Medicine, 6Department of Dermatology, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul, Korea
ABSTRACT Erythema dyschromicum perstans (EDP) is a hypermelanotic disorder of the idiopathic variety characterized by blue-gray macules in healthy individuals. It has been described mainly in patients from tropical areas of Central and South America. Our aim was to evaluate EDP in Korea through the analysis of retrospective case series with EDP and to describe the clinical and histopathological features. It was a retrospective study (2002–2012) of EDP confirmed by review of clinical photographs and biopsy specimens. The files of 68 patients with EDP from six tertiary medical centers in Korea were included in this study. Of the 68 patients, 29 were male and 39 female. The age of patients ranged 3–76 years (mean, 33.9). Clinically, the majority of patients had lesions on the trunk (n = 47, 69.1%); the neck was affected in 27 cases, the upper extremities in 26, the face in 22 and the lower extremities in 16. Peripheral erythematous borders were observed in 12 patients (17.6%) and 11 patients had itching sensation at the lesion. Histopathologically, dermal melanophages and pigment incontinence were the most common findings. A follow up of more than 1 year was obtained in 51 patients. Of these, only one patient experienced complete clearance without recurrence. Our study illustrates the clinical and histopathological findings of EDP in Koreans. Our multicenter data may contribute to the understanding of EDP.
Key words: Korean.
ashy dermatosis, clinical feature, erythema dyschromicum perstans, histopathological feature,
INTRODUCTION Erythema dyschromicum perstans (EDP) was first described by Ramirez in 1957.1 EDP, which is also known as ashy dermatosis, is a hypermelanotic disorder of healthy individuals clinically characterized by blue-gray macules. EDP occurs most frequently in Central and South America and the South Central USA, although cases have been described from different parts of the world.2 It mostly occurs in the second decade of life and commonly affects the face, arms, neck and trunk with symmetrical distribution; however, unilateral presentation has also been observed.3 The salient histopathological features were vacuolar degeneration of the basal cell layer with dermal melanosis and a perivascular infiltration. As time passes, the inflammatory infiltrate is usually diminished but prominent melanin incontinence exists with abundant melanophages. Although the features of EDP are somewhat investigated, the data on Asian EDP patients is limited. The aim of this study
was to assess the clinical and histopathological features of EDP in Korean patients through the analysis of a retrospective case series.
METHODS This study was conducted at the six university hospitals in Korea. We retrospectively included all patients with EDP referred to the hospitals during the period between 2002 and 2012. The diagnosis of EDP was made using the following clinical and histopathological 4-item criteria: (i) eruption of multiple blue-gray macules or patches that sometimes have erythematous and slightly elevated borders; (ii) histopathologically compatible with EDP; (iii) absence of pigmentationrelated underlying diseases and drug history; and (iv) no previous history of contact dermatitis confirmed by patch test. Mucosal involvement was not seen and the lesions were usually asymptomatic in the patients. After review of the clinical
Correspondence: Joo Yeon Ko, M.D., Ph.D., Department of Dermatology, Hanyang University Hospital, Seoul 133-792, South Korea. Email: [email protected] *These authors contributed equally to this work and should be considered as joint first authors. Received 25 March 2015; accepted 16 May 2015.
© 2015 Japanese Dermatological Association
1053
S.E. Chang et al.
Table 1. Demographic features of EDP
Age, years 0–9 10–19 20–29 30–39 40–49 50–59 ≥60 Sex Male Female Fitzpatrick skin type III IV V Location Trunk Neck Upper extremity Face Lower extremity Erythematous border Itching sensation
(a)
n
%
5 10 12 14 9 9 9
7.3 14.7 17.6 20.6 13.2 13.2 13.2
28 38
41.1 55.9
7 56 5
10.3 82.4 7.4
47 27 26 22 16 12 11
69.1 39.7 38.2 32.4 23.5 17.6 16.2
(b)
(c)
and histopathological findings, EDP was identified in 68 patients.
RESULTS Of the 68 patients, 29 were male and 39 were female. The age of onset of the lesion ranged 3–76 years. The mean age was 33.9 years. The majority of patients were of Fitzpatrick skin type IV (82.4%), followed by III (10.3%) and V (7.4%). Peripheral erythematous borders were observed in 12 patients (17.6%) and 11 patients had itching sensation and inflammation signs at the lesion on initiation of the lesions (16.1%) (Table 1). Clinically, the majority of patients had lesions on the trunk with 47 cases (69.1%); the neck was affected in 26 cases (38.2%), the upper limbs in 27 (39.7%), the face in 22 (32.4%) and the lower limbs in 16 (23.5%) (Fig. 1). Although the causal relationship between medication and EDP is uncertain, drug medications (clomiphene, oral estrogen pill and herbal medicine) were suspected as preceding causes of EDP in three patients. Histopathological evaluation of specimens from the 68 patients showed dermal melanophages and pigment incontinence in 57 cases (83.8%), mild to severe lymphocytic infiltration in 50 (73.5%), increased epidermal melanin in 40 (58.8%), vacuolar degeneration of the basal layer in 33 (48.5%), colloid
(g)
(h)
(d)
(e)
(i) (f)
Figure 1. Clinical photographs of (a,b) female patient aged 31 years, (c) female patient aged 15, (d) male patient aged 76, (e) female patient aged 15 and (f) male patient aged 68. Erythematous skin lesions were observed in (g,h) male patient aged 36 and (i) female patient aged 34.
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© 2015 Japanese Dermatological Association
Erythema dyschromicum perstans in Korean
bodies in 18 (26.5%), band-like lymphocytic infiltration in 13 (19.1%) and papillary dermal edema in 12 (17.6%) (Figs 2,3). Topical steroid was the most commonly used agent (29 cases, 42.6%), followed by triple combination cream (fluocinolone acetonide 0.01%, hydroquinone 4%, tretinoin 0.05%) (14 cases, 20.6%), topical hydroquinone (12 cases, 17.6%), topical calcineurin inhibitor (10 cases, 14.7%) and topical tretinoin (five cases, 7.4%). In 16 patients, they received systemic treatment including dapsone (four cases, 5.9%), minocycline (three cases, 4.4%), tranexamic acid and vitamins (three cases, 4.4%), clofazimine (two cases, 2.9%), pentoxifylline (two cases, 2.9%) and macrolide (two cases, 2.9%). A follow up of more than 1 year (range, 1–10 years; mean follow-up period, 17.9 months) was obtained in 51 patients (Table 2). Of these, only one patient (2.0%), a 3-year-old female, had shown clearing after 33 months. Twenty-two patients (43.1%) showed slight to fair improvement. They reflected that the lesions grad-
n 60
57 50
50 40 40
33
30 18
20
13
12
10 0 Dermal Lymphohismelanophages ocyc & pigment infiltraon inconnence
Increased epidermal melanin
Vacuolar degeneraon of basal layer
Colloid body
Band-like Papillary infiltraon dermal edema
Figure 2. Histopathological analysis of erythema dyschromicum perstans.
ually diminished in 3–5 years. In three patients (5.9%), lesions aggravated during the follow-up period, and the rest (49.0%) did not show any significant change. One patient, a 35-yearold male, had shown aggravation of the lesions after Q-switched 1064-nm neodymium:yttrium–aluminum–garnet laser treatment.
DISCUSSION Erythema dyschromicum perstans is an acquired, disfiguring skin disease that occurs most commonly in Latin American and Asian populations. Despite differences in ethnic background and etiology, clinical features of EDP are clinically similar in most patients. The disease spreads centrifugally and has characteristic blue-gray oval macules and patches that range in diameter 0.5–3 cm in all ethnic groups.2 In certain cases, some of the lesions had an easily observable, non-elevated, erythematous border which cleared after a variable period of time.4,5 However, it is difficult to identify the active phase and the erythematous border is infrequently observed despite the nomenclature of the disease.6,7 With regard to skin color, darkcolored individuals (Fitzpatrick types IV–V) are most commonly affected. In this study, the majority patients were of skin type IV (82.4%) and this result was consistent with previous studies. However, in contrast with EDP commonly affecting patients under 30 years old, the peak age of our patients was fourth decade followed by third decade (mean age, 33.9 years). This difference may be due to racial difference or diagnostic delay. The most common locations are on the trunk, arms and face. Although there is no clear sexual predilection, some authors report that EDP may be more common in females and
(a)
(b)
(c)
(d)
Figure 3. Histopathological findings of erythema dyschromicum perstans. (a) Dermal pigmentation, (b) lymphohistiocytic infiltration, (c) vacuolar degeneration and (d) papillary dermal edema (hematoxylin–eosin, original magnification 9200).
© 2015 Japanese Dermatological Association
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S.E. Chang et al.
Table 2. Follow-up period
10 years Total
n
%
17 21 13 8 4 2 0 3 68
25.0 30.9 19.1 11.8 5.9 2.9 0 4.4 100
our study shows female predominance.8,9 Except for occasional slight itching, EDP lesions remain symptomless. Because the lesion is symptomless, patients regard it as a cosmetic problem. We assumed that the sexual difference of concerns about appearance may have led this result. Histopathologically, EDP can be subdivided into active (or early) and inactive (or late) lesions. The active lesion shows basal vacuolar degeneration and edematous papillary dermis accompanied by lymphocytic infiltration. The inactive lesion shows melanophages and pigment incontinence in the dermis without inflammation in general. Although an erythematous border is known as a characteristic feature of EDP, it was only found in 17.6% (12/68) of our patients. Also, histopathological analysis of lesions showed basal layer vacuolization in 48.5% (33/68) of our patients, which is lower than the 85% reported by Vega et al.10 and 58% reported by Vasquez-Ochaoa et al.11 Tschen et al.12 and Leonforte and Pelaez13 described the presence of an active margin in patients with EDP of short duration. Probably, Korean EDP patients visited hospital beyond the initial stage of the disease or there was a referral bias due to tertiary medical center. To date, no effective standard treatment is available, although some good results with clofazimine13,14 and dapsone15,16 have been reported in small case series. Based on the fact that inflammation and pigmentation can be treated with topical steroids and depigmenting agents, these agents were most frequently used in this study. However, the lesion disappeared in only one patient. This implies that EDP is a chronic disease, as indicated by its name. Erythema dyschromicum perstans is a controversial entity and is sometimes considered as lichen planus pigmentosus (LPP)9,17,18 or idiopathic eruptive macular pigmentation (IEMP).19 Sometimes, EDP, LPP and IEMP are considered as the same disease.20 Many patients with LPP may show residual hyperpigmentation resembling that of EDP, which shows histopathological lichenoid features. The following list of items may be helpful to differentiate LP: (i) except for occasional slight itching, they remain symptomless; (ii) some of the lesions have an easily observable, non-elevated, erythematous border; (iii) the lesions appear either on exposed or unexposed areas alike; (iv) EDP does not involve mucosa; and (v) the lesion rarely improves. IEMP is a rare disorder of pigmentation first described by Degos et al. The following list of items are the
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criteria for diagnosis of IEMP: (i) eruption of brownish, nonconfluent, asymptomatic macules involving the trunk, neck and proximal extremities in children and adolescents; (ii) absence of preceding inflammatory lesions; (iii) no previous drug exposure; (iv) basal layer hyperpigmentation of the epidermis and prominent dermal melanophages without visible layer damage or lichenoid inflammatory infiltrate; and (v) normal mast cell count. However, these criteria cannot differentiate between EDP and IEMP in all cases. The main limitations of the present study are a retrospective approach, small number of study patients and referral bias. Despite this, it adds clinical characteristics of EDP in an Asian population from Korea. Our study could contribute to the understanding of the clinicohistopathological features and clinical course of EDP. Furthermore, we expect that leading diagnostic criteria will be established in the near future.
ACKNOWLEDGMENT:
We thank to Shin Ok Kim, AESTURA, for collection of data. Author contribution as follows. All authors have contributed significantly and are in agreement with the content of the manuscript.
CONFLICT OF INTEREST:
None.
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Erythema dyschromicum perstans in Korean
12 Tschen JA, Tschen EA, McGavran MH. Erythema dyschromicum perstans. J Am Acad Dermatol 1980; 2: 295–302. rez-Alfonzo R, Abrusci V et al. Clinical trial with 13 Piquero-Martın J, Pe clofazimine for treating erythema dyschromicum perstans. Evaluation of cell-mediated immunity. Int J Dermatol 1989; 28: 198–200. 14 Baranda L, Torres-Alvarez B, Cortes-Franco R, Moncada B, Portelas-Perez DP, Gonzalez-Amaro R. Involvement of cell adhesion and activation molecules in the pathogenesis of erythema dyschromicum perstans (ashy dermatitis). The effect of clofazimine therapy. Arch Dermatol 1997; 133: 325–329. 15 Bahadir S, Cobanoglu U, Cimsit G, Yayli S, Alpay K. Erythema dyschromicum perstans: response to dapsone therapy. Int J Dermatol 2004; 43: 220–222. 16 Kontochristopoulos G, Stavropoulos P, Panteleos D, Aroni K. Erythema dyschromicum perstans: response to dapsone therapy. Int J Dermatol 1998; 37: 796–798.
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17 Gadenne BM, Camisa C. Lichenoid dermatitides (lichen planus, keratosis lichenoides and erythema dischromicum perstans). In: Arndt K, Leboit P, Robinson J, eds. Cutaneous Medicine and Surgery, 1st edn. Philadelphia: W.B.Saunders, 1996; 241. ez de di Bari O. Erythema dyschromicum perstans 18 Leonforte JL, Pela versus lichen planus. Med Cutan Ibero Lat Am 1987; 15: 89–92. 19 Torrelo A, Zaballos P, Colmenero I, Mediero IG, de Prada I, Zambrano A. Erythema dyschromicum perstans in children: a report of 14 cases. J Eur Acad Dermatol Venereol 2005; 19: 422–426. 20 Oiso N, Tsuruta D, Imanishi H, Kobayashi H, Kawada A. Erythema dyschromicum perstans in a Japanese child. Pediatr Dermatol 2012; 29: 637–640.
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