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2 Sarin KY, McNiff JM, Kwok S, Kim J, Khavari PA. Activating HRAS mutation in nevus spilus. J Invest Dermatol 2014; 134: 1766–1768. 3 Schaffer JV, Orlow SJ, Lazova R, Bolognia JL. Speckled lentiginous nevus–classic congenital melanocytic nevus hybrid not the result of “collision”. Arch Dermatol 2001; 137: 1655. 4 Haenssle HA, Kaune KM, Buhl T et al. Melanoma arising in segmental nevus spilus: detection by sequential digital dermatoscopy. J Am Acad Dermatol 2009; 61: 337–341. 5 Kinsler VA, Krengel S, Riviere JB et al. Next-generation sequencing of nevus spilus-type congenital melanocytic nevus: exquisite genotype-phenotype correlation in mosaic RASopathies. J Invest Dermatol 2014; 134: 2658–2660. 6 Kinsler VA, Thomas AC, Ishida M et al. Multiple congenital melanocytic nevi and neurocutaneous melanosis are caused by postzygotic mutations in codon 61 of NRAS. J Invest Dermatol 2013; 133: 2229–2236. DOI: 10.1111/jdv.13173
Perforating granuloma annulare: a case report and literature review Editor Perforating granuloma annulare (PGA), which was first described in 1954 by Calnan1 and formally named in 1971 by Owens and Freeman2, is a rare subtype of granuloma annulare with unknown aetiology and perforation mechanism.3 Here, we presented one Chinese PGA case and reviewed 78 cases reported including 13 Chinese cases. A 34-year-old Chinese man presented with an 11-year history of gradually progressive papular lesions scattered over his upper extremities and trunk. These asymptomatic papules initially started on the dorsa of his hands and fingers. Individual papule developed a central umbilication with scale or crust, and then regressed over the next 1–2 months, usually leaving a white punctate scar. Some papules with a central pin point-sized, black horny plug were observed. Gradually, lesions increased and involved bilateral forearms, right arm and trunk. There was no history of trauma, insect bites or excess solar exposure. Physical
(a)
examination revealed multiple 3–6 mm, annular or arciform, flesh-coloured firm papules with central umbilicated crust or scale mainly involving the right arm and back (Fig. 1a). There were several 1–2 mm atrophic hypopigmented scars on the dorsa of his hands. A biopsy from the umbilicated papule on the back showed findings compatible with PGA (Fig. 1b). Laboratory investigations including complete blood cell count, blood biochemistry, urinalysis and chest X-ray were normal. Those lesions resolved with treatments of liquid nitrogen, and topical steroids within 3 months. The eruptions of PGA were classified into papular perforating type (P-type) and ulcerative perforating type (U-type).4 The localization of lesions were divided into localized PGA (LPGA) and generalized PGA (GPGA), and cases with extensive lesions over extremities only were regarded as disseminated.3 The comparison of clinical features of GPGA and LPGA variants and P- and U-type variants of 78 cases reviewed were summarized in Table 1. The entire group included 78 patients (49 female, 29 male), and data regarding age of onset and evolution time of PGA were not available in five cases. The mean age of onset was 32 years (range, 53 days to 72 years). Patients classified as P-type had a lower mean age of onset than U-type (29 vs. 48 years, P < 0.001). Furthermore, it was significantly lower in female than that in male (28 vs. 39 years, P < 0.05). The mean evolution time of PGA was 3 years (range, 1 month to 30 years), and it was significantly shorter in LPGA than GPGA (2.3 vs. 4.6 years, P < 0.05). The most common clinical presentation of PGA was flesh to redcoloured, 1–7 mm papules with central crust or scale, and/or umbilication. One third of patients had umbilicated lesions exuding a thick, creamy or clear, viscous material. Eruptions were usually asymptomatic, whereas pruritus5 (22%, 12/54) and pain3 (9%, 5/54) were also reported. No Koebner phenomenon was noted. Essentially all areas of the body could be affected by PGA, even on the ears,5 soles,6 palms,7 and in the genital region,8 whereas the extensor surfaces of both extremities and the dorsa of the hands and fingers were involved in around half of the patients. About 60% of PGA cases were disseminated, while single lesion9 only presented in 7 (9%) cases, which was the same as that reported by Penas et al.3 Nine PGA
(b)
Figure 1 Close view of umbilicated papules with scale on the upper back in a 34-year-old Chinese man with perforating granuloma annulare (a), and its histology showing upper dermal palisading granuloma and perforation of the epidermis that exuded necrobiotic material (H&E, 409) (b).
JEADV 2016, 30, 1195–1252
© 2015 European Academy of Dermatology and Venereology
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Table 1 Clinical features of perforating granuloma andulare (PGA) and generalized (GPGA) and localized (LPGA) variants, and papular (P-type) and ulcerative (U-type) variants PGA N(Female) Female : Male
78 (49) 1.7 : 1
GPGA 23 (13) 1.3 : 1
LPGA
P-type
55 (36)
32 (20.3)
36 (22.9)
31 (19.1)
Mean evolution time, years (SD)
3.0 (4.7)
4.6 (6.9)
2.3 (3.3)
9/50 (18%)
5/19 (26%)
4/31 (13%)
Papules (%)
85
94
81
Umbilicated (%)
61
72
56
Scaled or Crusted (%)
78
83
76
Pustular-like lesions (%)
27
33
24
Scars (%)
28
42
22
U-type
63 (39)
1.9 : 1
Mean age of onset, years (SD) DM (%)
P*
1.6 : 1 0.046
P†
15 (10) 2:1
29 (18.7)
48 (20.0)
3.1 (4.9)
2.3 (4.0)
3/40 (7.5%)
6/10 (60%)
0.0008 0.000
Skin lesions
Annular lesions (%)
22
17
24
Plaques (%)
17
17
18
Nodules (%)
16
11
18
*Between GPGA and LPGA variants. †Between papular and ulcerative variants. SD, standard deviation; DM, diabetes mellitus.
cases (18%) were diagnosed with DM.4,10 U-type had a stronger relationship with DM (P < 0.001) than P-type. Furthermore, DM patients had significantly higher mean age of onset than non-DM patients (51 vs. 33 years, P < 0.01). Remission of PGA lesions was found in approximately 60% of patients regardless of treatments options. Spontaneous remission was seen in 10 of the 13 patients without any treatment.10 No specific difference was found in Chinese PGA cases.
8 Madan V, Gangopadhyay M, Dawn G. Multiple asymptomatic scrotal nodules. Clin Exp Dermatol 2009; 34: 433–434. 9 Kai Y, Xuyan Z, Xianhua J. Perforating Granuloma Annulare. Chin J Lepr Skin Dis 2013; 29: 787–788. 10 Santos R, Afonso A, Cunha F et al. Generalized Perforating Granuloma Annulare. J Eur Acad Dermatol Venereol 1999; 13: 62–63. DOI: 10.1111/jdv.13174
W. Zhong,1,2 Y. Shao,1 T. Ye,1 J. Li,3 B. Yu,1,*,† X. Dou1,*,† 1 Department of Dermatology, Peking University Shenzhen Hospital, Shenzhen, Guangdong 518036, China, 2Shantou University Medical College, Shantou, Guangdong 515041, China, 3Department of Pathology, Peking University Shenzhen Hospital, Shenzhen, Guangdong 518036, China *Correspondence: X. Dou and B. Yu. E-mails: [email protected]; [email protected] † These authors contributed equally to this work.
References 1 Calnan CD. Granuloma annulare. Case history (Summary). Br J Dermatol 1954; 66: 254. 2 Owens DW, Freeman RG. Perforating granuloma annulare. Arch Dermatol 1971; 103: 64–67. 3 Penas PF, Jones-Caballero M, Fraga J, Sanchez-Perez J, Garcıa-Dıez A. Perforating granuloma annulare. Int J Dermatol 1997; 36: 340–348. 4 Shimizu H, Harada T, Baba E, Kuramochi M. Perforating granuloma annulare. Int J Dermatol 1985; 24: 581–583. 5 Farrar CW, Bell HK, Dobson CM, Sharpe GR. Perforating granuloma annulare presenting on the ears. Br J Dermatol 2002; 147: 1026–1028. 6 Jacyk WK, Birecka I. Generalized perforating granuloma annulare. Arch Dermatol 1974; 110: 809. 7 Gamo Villegas R, Sopena Barona J, Guerra Tapia A, Vergara Sanchez A, Rodrıguez Peralto JL, Iglesias DIez L. Pustular generalized perforating granuloma annulare. Br J Dermatol 2003; 149: 866–868.
JEADV 2016, 30, 1195–1252
Bimatoprost in periorbital vitiligo: a ray of hope or dilemma Editor A 12-year-old girl presented to the dermatology OPD with depigmented macule predominantly on the periorbital region (Fig. 1). The lesion was stable and the patient gave a history of non-progressive nature of the lesion since one and a half year. The patient also had history of prolonged application of tacrolimus 0.03% ointment. The patient was given bimatoprost 0.03%
Figure 1 Periorbital vitiligo before treatment.
© 2015 European Academy of Dermatology and Venereology
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2 Sarin KY, McNiff JM, Kwok S, Kim J, Khavari PA. Activating HRAS mutation in nevus spilus. J Invest Dermatol 2014; 134: 1766–1768. 3 Schaffer JV, Orlow SJ, Lazova R, Bolognia JL. Speckled lentiginous nevus–classic congenital melanocytic nevus hybrid not the result of “collision”. Arch Dermatol 2001; 137: 1655. 4 Haenssle HA, Kaune KM, Buhl T et al. Melanoma arising in segmental nevus spilus: detection by sequential digital dermatoscopy. J Am Acad Dermatol 2009; 61: 337–341. 5 Kinsler VA, Krengel S, Riviere JB et al. Next-generation sequencing of nevus spilus-type congenital melanocytic nevus: exquisite genotype-phenotype correlation in mosaic RASopathies. J Invest Dermatol 2014; 134: 2658–2660. 6 Kinsler VA, Thomas AC, Ishida M et al. Multiple congenital melanocytic nevi and neurocutaneous melanosis are caused by postzygotic mutations in codon 61 of NRAS. J Invest Dermatol 2013; 133: 2229–2236. DOI: 10.1111/jdv.13173
Perforating granuloma annulare: a case report and literature review Editor Perforating granuloma annulare (PGA), which was first described in 1954 by Calnan1 and formally named in 1971 by Owens and Freeman2, is a rare subtype of granuloma annulare with unknown aetiology and perforation mechanism.3 Here, we presented one Chinese PGA case and reviewed 78 cases reported including 13 Chinese cases. A 34-year-old Chinese man presented with an 11-year history of gradually progressive papular lesions scattered over his upper extremities and trunk. These asymptomatic papules initially started on the dorsa of his hands and fingers. Individual papule developed a central umbilication with scale or crust, and then regressed over the next 1–2 months, usually leaving a white punctate scar. Some papules with a central pin point-sized, black horny plug were observed. Gradually, lesions increased and involved bilateral forearms, right arm and trunk. There was no history of trauma, insect bites or excess solar exposure. Physical
(a)
examination revealed multiple 3–6 mm, annular or arciform, flesh-coloured firm papules with central umbilicated crust or scale mainly involving the right arm and back (Fig. 1a). There were several 1–2 mm atrophic hypopigmented scars on the dorsa of his hands. A biopsy from the umbilicated papule on the back showed findings compatible with PGA (Fig. 1b). Laboratory investigations including complete blood cell count, blood biochemistry, urinalysis and chest X-ray were normal. Those lesions resolved with treatments of liquid nitrogen, and topical steroids within 3 months. The eruptions of PGA were classified into papular perforating type (P-type) and ulcerative perforating type (U-type).4 The localization of lesions were divided into localized PGA (LPGA) and generalized PGA (GPGA), and cases with extensive lesions over extremities only were regarded as disseminated.3 The comparison of clinical features of GPGA and LPGA variants and P- and U-type variants of 78 cases reviewed were summarized in Table 1. The entire group included 78 patients (49 female, 29 male), and data regarding age of onset and evolution time of PGA were not available in five cases. The mean age of onset was 32 years (range, 53 days to 72 years). Patients classified as P-type had a lower mean age of onset than U-type (29 vs. 48 years, P < 0.001). Furthermore, it was significantly lower in female than that in male (28 vs. 39 years, P < 0.05). The mean evolution time of PGA was 3 years (range, 1 month to 30 years), and it was significantly shorter in LPGA than GPGA (2.3 vs. 4.6 years, P < 0.05). The most common clinical presentation of PGA was flesh to redcoloured, 1–7 mm papules with central crust or scale, and/or umbilication. One third of patients had umbilicated lesions exuding a thick, creamy or clear, viscous material. Eruptions were usually asymptomatic, whereas pruritus5 (22%, 12/54) and pain3 (9%, 5/54) were also reported. No Koebner phenomenon was noted. Essentially all areas of the body could be affected by PGA, even on the ears,5 soles,6 palms,7 and in the genital region,8 whereas the extensor surfaces of both extremities and the dorsa of the hands and fingers were involved in around half of the patients. About 60% of PGA cases were disseminated, while single lesion9 only presented in 7 (9%) cases, which was the same as that reported by Penas et al.3 Nine PGA
(b)
Figure 1 Close view of umbilicated papules with scale on the upper back in a 34-year-old Chinese man with perforating granuloma annulare (a), and its histology showing upper dermal palisading granuloma and perforation of the epidermis that exuded necrobiotic material (H&E, 409) (b).
JEADV 2016, 30, 1195–1252
© 2015 European Academy of Dermatology and Venereology
Letters to the Editor
1247
Table 1 Clinical features of perforating granuloma andulare (PGA) and generalized (GPGA) and localized (LPGA) variants, and papular (P-type) and ulcerative (U-type) variants PGA N(Female) Female : Male
78 (49) 1.7 : 1
GPGA 23 (13) 1.3 : 1
LPGA
P-type
55 (36)
32 (20.3)
36 (22.9)
31 (19.1)
Mean evolution time, years (SD)
3.0 (4.7)
4.6 (6.9)
2.3 (3.3)
9/50 (18%)
5/19 (26%)
4/31 (13%)
Papules (%)
85
94
81
Umbilicated (%)
61
72
56
Scaled or Crusted (%)
78
83
76
Pustular-like lesions (%)
27
33
24
Scars (%)
28
42
22
U-type
63 (39)
1.9 : 1
Mean age of onset, years (SD) DM (%)
P*
1.6 : 1 0.046
P†
15 (10) 2:1
29 (18.7)
48 (20.0)
3.1 (4.9)
2.3 (4.0)
3/40 (7.5%)
6/10 (60%)
0.0008 0.000
Skin lesions
Annular lesions (%)
22
17
24
Plaques (%)
17
17
18
Nodules (%)
16
11
18
*Between GPGA and LPGA variants. †Between papular and ulcerative variants. SD, standard deviation; DM, diabetes mellitus.
cases (18%) were diagnosed with DM.4,10 U-type had a stronger relationship with DM (P < 0.001) than P-type. Furthermore, DM patients had significantly higher mean age of onset than non-DM patients (51 vs. 33 years, P < 0.01). Remission of PGA lesions was found in approximately 60% of patients regardless of treatments options. Spontaneous remission was seen in 10 of the 13 patients without any treatment.10 No specific difference was found in Chinese PGA cases.
8 Madan V, Gangopadhyay M, Dawn G. Multiple asymptomatic scrotal nodules. Clin Exp Dermatol 2009; 34: 433–434. 9 Kai Y, Xuyan Z, Xianhua J. Perforating Granuloma Annulare. Chin J Lepr Skin Dis 2013; 29: 787–788. 10 Santos R, Afonso A, Cunha F et al. Generalized Perforating Granuloma Annulare. J Eur Acad Dermatol Venereol 1999; 13: 62–63. DOI: 10.1111/jdv.13174
W. Zhong,1,2 Y. Shao,1 T. Ye,1 J. Li,3 B. Yu,1,*,† X. Dou1,*,† 1 Department of Dermatology, Peking University Shenzhen Hospital, Shenzhen, Guangdong 518036, China, 2Shantou University Medical College, Shantou, Guangdong 515041, China, 3Department of Pathology, Peking University Shenzhen Hospital, Shenzhen, Guangdong 518036, China *Correspondence: X. Dou and B. Yu. E-mails: [email protected]; [email protected] † These authors contributed equally to this work.
References 1 Calnan CD. Granuloma annulare. Case history (Summary). Br J Dermatol 1954; 66: 254. 2 Owens DW, Freeman RG. Perforating granuloma annulare. Arch Dermatol 1971; 103: 64–67. 3 Penas PF, Jones-Caballero M, Fraga J, Sanchez-Perez J, Garcıa-Dıez A. Perforating granuloma annulare. Int J Dermatol 1997; 36: 340–348. 4 Shimizu H, Harada T, Baba E, Kuramochi M. Perforating granuloma annulare. Int J Dermatol 1985; 24: 581–583. 5 Farrar CW, Bell HK, Dobson CM, Sharpe GR. Perforating granuloma annulare presenting on the ears. Br J Dermatol 2002; 147: 1026–1028. 6 Jacyk WK, Birecka I. Generalized perforating granuloma annulare. Arch Dermatol 1974; 110: 809. 7 Gamo Villegas R, Sopena Barona J, Guerra Tapia A, Vergara Sanchez A, Rodrıguez Peralto JL, Iglesias DIez L. Pustular generalized perforating granuloma annulare. Br J Dermatol 2003; 149: 866–868.
JEADV 2016, 30, 1195–1252
Bimatoprost in periorbital vitiligo: a ray of hope or dilemma Editor A 12-year-old girl presented to the dermatology OPD with depigmented macule predominantly on the periorbital region (Fig. 1). The lesion was stable and the patient gave a history of non-progressive nature of the lesion since one and a half year. The patient also had history of prolonged application of tacrolimus 0.03% ointment. The patient was given bimatoprost 0.03%
Figure 1 Periorbital vitiligo before treatment.
© 2015 European Academy of Dermatology and Venereology
