Letters to the Editor
Pustular pyoderma gangrenosum: Report of two cases Dear Editor, In our case 1, a 23-year-old woman, who was otherwise healthy, was admitted to our hospital, complaining of multiple ulcers on the lower legs with a sudden onset 1 month prior to presentation. Physical examination revealed deep, coalesced ulcers with small satellite ulcerations and pustules on the bilateral lower legs (Fig. 1a,b). Laboratory examination showed an increased number of leukocytes (10 900/mm3 with 77% neutrophils), C-reactive protein (CRP; 14.55 mg/dL) and erythrocyte sedimentation rate (44 mm/h), but other data including liver and renal function were normal. The pustule was sterile. A biopsy specimen from the edge of the ulcers revealed prominent red blood cells and diffuse neutrophil infiltration in the upper to mid-dermis. Another biopsy specimen from the small pustule showed neutrophilic abscess in the epidermis and neutrophil infiltration in the upper to mid-dermis (Fig. 1c). Detail investigation did not reveal systemic underlying disorders. She was successfully treated with systemic prednisolone (30 mg/ day). In our case 2, a 52-year-old man suffered from stomachache, diarrhea and bloody stools for some months prior to presentation, and was diagnosed with ulcerative colitis (UC). Almost simultaneously, he developed ulcerative lesions on the lower legs, and also multiple, small pustular lesions on the scalp (Fig. 1d,e). Laboratory examination showed increased levels of CRP (3.12 mg/dL) and erythrocyte sedimentation rate (53 mm/h), and a white blood cell count (8900/mm3) with 70%
(a)
(b)
(c)
neutrophils. The pustule was sterile. Histological examination from the edge of the ulcer showed subepidermal edema and neutrophil and mononuclear cell infiltration in the mid- to lower dermis. After admission, systemic prednisolone (30 mg/day) was administrated for intestinal lesions, which also improved skin lesions. Pustular pyoderma gangrenosum (PG) is rare1–5 and usually seen in association with other types of PG. In both of our cases, ulcerative PG was also seen on the lower limbs. Usually, pustules do not develop into ulceration, however, evolution from pustule to ulcer may occur. Regarding the sites of pustular PG, the extremities are the most frequently involved followed by the upper trunk, and additionally, pustules can be rarely seen on the scalp and penis as well.4 In case 2, multiple sterile pustules were on the scalp. Ulcerative PG occasionally associates with several systemic diseases including rheumatoid arthritis, inflammatory bowel disease, hematological malignancy and Takayasu’s arteritis, whereas pustular PG frequently associates with inflammatory bowel disease, and bullous PG mostly associates with hematological malignancies. Pediatric cases of pustular PG were reported associated with UC.3 Case 2 had UC, whereas case 1 had no accompanying systemic disorders. In both cases, development of pustular lesions was related to the activity of PG, and responded well to oral prednisolone. Although systemic prednisolone is the first choice, there are also cases successfully treated with topical betamethasone5 or tacrolimus.4
(d)
(e)
Figure 1. (a) Multiple ulcerative and pustular lesions on the bilateral lower legs (case 1). (b) Higher magnification showing small pustules surrounded with erythema. (c) Histological features showing numerous neutrophil infiltration in the upper to mid-dermis (hematoxylin–eosin, original magnification 9100). (d) Deep ulceration covered with bloody necrotizing tissues surrounded by edematous borders (case 2). (e) Multiple pustular lesions on the scalp.
Correspondence: Nobuyuki Kikuchi, M.D., Department of Dermatology, Fukushima Medical University, 1 Hikarigaoka, Fukushima 960-1295, Japan. Email: [email protected]
542
© 2015 Japanese Dermatological Association
Letters to the Editor
Although arthritides did not occur either of our cases, a case of pustular PG was reported in association with bowel-associated arthritis–dermatitis syndrome,5 suggesting that skin manifestation of bowel-bypass syndrome shows an overlap with PG and other neutrophilic disorders. PG may be included in the skin–gut or skin–joint–gut axis disorders mediated by T-helper 17 type cytokines, but further studies are necessary.
CONFLICT OF INTEREST:
None declared.
Nobuyuki KIKUCHI, Yuka HANAMI, Mikio OHTSUKA, Toshiyuki YAMAMOTO Department of Dermatology, Fukushima Medical University, Fukushima, Japan
REFERENCES 1 Nakano S, Oiso N, Kawada A. Coexisting pustular and ulcerative pyoderma gangrenosum. Eur J Dermatol 2012; 22: 153–154. 2 Omiya W, Ujiie H, Akiyama M, Shigematsu K, Onozawa M, Shimuzu H. Coexistence of pustular and vegetative pyoderma gangrenosum in a patient with myelodysplastic syndrome. Eur J Dermatol 2012; 22: 711–712. 3 Barnes L, Lucky AW, Bucuvalas JC, Suchy FJ. Pustular pyoderma gangrenosum associated with ulcerative colitis in childhood. J Am Acad Dermatol 1986; 15: 608–614. 4 Larsen CG, Thyssen JP. Pustular penile pyoderma gangrenosum successfully treated with topical tacrolimus ointment. Acta Derm Venereol 2012; 92: 104–105. 5 Brouard MC, Chavaz P, Borradori L. Acute pustulosis of the legs in diverticulitis with sigmoid stenosis: an overlap between bowl-associated dermatosis-arthritis syndrome and pustular pyoderma gangrenosum. J Eur Acad Dermatol Venereol 2004; 18: 89–92.
doi: 10.1111/1346-8138.12817
Multiple halo nevi subsequent to a short period of sunbathing Dear Editor, The halo nevus is characterized by a depigmented, vitiligolike halo around a melanocytic nevus, commonly affecting children. We herein report a case of a 16-year-old boy with multiple halo nevi, possibly induced by exposure to sunlight. A 16-year-old Japanese boy was referred to us for multiple areas of depigmentation around melanocytic nevi. His medical
(a)
or family history revealed no pigmentary disorders. The depigmentation occurred 1 month after a 2-week period spent swimming at the beach in the summer. Physical examination revealed a total of 18 halo nevi on the patient’s trunk and extremities (Fig. 1a). Among them, seven were located on the frontal trunk, four on the back, three on the right upper arm, one on the face, one on the right upper thigh, one on the right lower thigh and
(b)
(c)
Figure 1. (a) Clinical appearance showing multiple halo nevi (arrows). Nevus is surrounded by depigmentation (insert). (b) Histological view showing dense lymphocyte infiltration among the dissociated nevus nests in the upper dermis (original magnification 9200). (c) A number of CD8-positive T cells infiltrated into the nevoid cells (9200).
Correspondence: Atsuko Kawaguchi, M.D., Department of Dermatology, Tokyo Medical University, 6-7-1 Nishi-shinjuku, Shinjuku-ku, Tokyo 160-0023, Japan. Email: [email protected]
© 2015 Japanese Dermatological Association
543
Pustular pyoderma gangrenosum: Report of two cases Dear Editor, In our case 1, a 23-year-old woman, who was otherwise healthy, was admitted to our hospital, complaining of multiple ulcers on the lower legs with a sudden onset 1 month prior to presentation. Physical examination revealed deep, coalesced ulcers with small satellite ulcerations and pustules on the bilateral lower legs (Fig. 1a,b). Laboratory examination showed an increased number of leukocytes (10 900/mm3 with 77% neutrophils), C-reactive protein (CRP; 14.55 mg/dL) and erythrocyte sedimentation rate (44 mm/h), but other data including liver and renal function were normal. The pustule was sterile. A biopsy specimen from the edge of the ulcers revealed prominent red blood cells and diffuse neutrophil infiltration in the upper to mid-dermis. Another biopsy specimen from the small pustule showed neutrophilic abscess in the epidermis and neutrophil infiltration in the upper to mid-dermis (Fig. 1c). Detail investigation did not reveal systemic underlying disorders. She was successfully treated with systemic prednisolone (30 mg/ day). In our case 2, a 52-year-old man suffered from stomachache, diarrhea and bloody stools for some months prior to presentation, and was diagnosed with ulcerative colitis (UC). Almost simultaneously, he developed ulcerative lesions on the lower legs, and also multiple, small pustular lesions on the scalp (Fig. 1d,e). Laboratory examination showed increased levels of CRP (3.12 mg/dL) and erythrocyte sedimentation rate (53 mm/h), and a white blood cell count (8900/mm3) with 70%
(a)
(b)
(c)
neutrophils. The pustule was sterile. Histological examination from the edge of the ulcer showed subepidermal edema and neutrophil and mononuclear cell infiltration in the mid- to lower dermis. After admission, systemic prednisolone (30 mg/day) was administrated for intestinal lesions, which also improved skin lesions. Pustular pyoderma gangrenosum (PG) is rare1–5 and usually seen in association with other types of PG. In both of our cases, ulcerative PG was also seen on the lower limbs. Usually, pustules do not develop into ulceration, however, evolution from pustule to ulcer may occur. Regarding the sites of pustular PG, the extremities are the most frequently involved followed by the upper trunk, and additionally, pustules can be rarely seen on the scalp and penis as well.4 In case 2, multiple sterile pustules were on the scalp. Ulcerative PG occasionally associates with several systemic diseases including rheumatoid arthritis, inflammatory bowel disease, hematological malignancy and Takayasu’s arteritis, whereas pustular PG frequently associates with inflammatory bowel disease, and bullous PG mostly associates with hematological malignancies. Pediatric cases of pustular PG were reported associated with UC.3 Case 2 had UC, whereas case 1 had no accompanying systemic disorders. In both cases, development of pustular lesions was related to the activity of PG, and responded well to oral prednisolone. Although systemic prednisolone is the first choice, there are also cases successfully treated with topical betamethasone5 or tacrolimus.4
(d)
(e)
Figure 1. (a) Multiple ulcerative and pustular lesions on the bilateral lower legs (case 1). (b) Higher magnification showing small pustules surrounded with erythema. (c) Histological features showing numerous neutrophil infiltration in the upper to mid-dermis (hematoxylin–eosin, original magnification 9100). (d) Deep ulceration covered with bloody necrotizing tissues surrounded by edematous borders (case 2). (e) Multiple pustular lesions on the scalp.
Correspondence: Nobuyuki Kikuchi, M.D., Department of Dermatology, Fukushima Medical University, 1 Hikarigaoka, Fukushima 960-1295, Japan. Email: [email protected]
542
© 2015 Japanese Dermatological Association
Letters to the Editor
Although arthritides did not occur either of our cases, a case of pustular PG was reported in association with bowel-associated arthritis–dermatitis syndrome,5 suggesting that skin manifestation of bowel-bypass syndrome shows an overlap with PG and other neutrophilic disorders. PG may be included in the skin–gut or skin–joint–gut axis disorders mediated by T-helper 17 type cytokines, but further studies are necessary.
CONFLICT OF INTEREST:
None declared.
Nobuyuki KIKUCHI, Yuka HANAMI, Mikio OHTSUKA, Toshiyuki YAMAMOTO Department of Dermatology, Fukushima Medical University, Fukushima, Japan
REFERENCES 1 Nakano S, Oiso N, Kawada A. Coexisting pustular and ulcerative pyoderma gangrenosum. Eur J Dermatol 2012; 22: 153–154. 2 Omiya W, Ujiie H, Akiyama M, Shigematsu K, Onozawa M, Shimuzu H. Coexistence of pustular and vegetative pyoderma gangrenosum in a patient with myelodysplastic syndrome. Eur J Dermatol 2012; 22: 711–712. 3 Barnes L, Lucky AW, Bucuvalas JC, Suchy FJ. Pustular pyoderma gangrenosum associated with ulcerative colitis in childhood. J Am Acad Dermatol 1986; 15: 608–614. 4 Larsen CG, Thyssen JP. Pustular penile pyoderma gangrenosum successfully treated with topical tacrolimus ointment. Acta Derm Venereol 2012; 92: 104–105. 5 Brouard MC, Chavaz P, Borradori L. Acute pustulosis of the legs in diverticulitis with sigmoid stenosis: an overlap between bowl-associated dermatosis-arthritis syndrome and pustular pyoderma gangrenosum. J Eur Acad Dermatol Venereol 2004; 18: 89–92.
doi: 10.1111/1346-8138.12817
Multiple halo nevi subsequent to a short period of sunbathing Dear Editor, The halo nevus is characterized by a depigmented, vitiligolike halo around a melanocytic nevus, commonly affecting children. We herein report a case of a 16-year-old boy with multiple halo nevi, possibly induced by exposure to sunlight. A 16-year-old Japanese boy was referred to us for multiple areas of depigmentation around melanocytic nevi. His medical
(a)
or family history revealed no pigmentary disorders. The depigmentation occurred 1 month after a 2-week period spent swimming at the beach in the summer. Physical examination revealed a total of 18 halo nevi on the patient’s trunk and extremities (Fig. 1a). Among them, seven were located on the frontal trunk, four on the back, three on the right upper arm, one on the face, one on the right upper thigh, one on the right lower thigh and
(b)
(c)
Figure 1. (a) Clinical appearance showing multiple halo nevi (arrows). Nevus is surrounded by depigmentation (insert). (b) Histological view showing dense lymphocyte infiltration among the dissociated nevus nests in the upper dermis (original magnification 9200). (c) A number of CD8-positive T cells infiltrated into the nevoid cells (9200).
Correspondence: Atsuko Kawaguchi, M.D., Department of Dermatology, Tokyo Medical University, 6-7-1 Nishi-shinjuku, Shinjuku-ku, Tokyo 160-0023, Japan. Email: [email protected]
© 2015 Japanese Dermatological Association
543
