J Plast Surg Hand Surg, 2014; 48: 80–83 © 2014 Informa Healthcare ISSN: 2000-656X print / 2000-6764 online DOI: 10.3109/2000656X.2012.748323
CASE REPORT
Effective surgical treatment of progressive nodular histiocytosis Norihisa Nakayashiki1, Hirotaka Akita2, Wakako Mori1, Daisuke Watanabe3, Yasuo Sakai1 & Yohko Yoshimura1 1
Department of Plastic and Reconstructive Surgery, 2Department of Dermatology, Fujita Health University, Aichi and 3Department of Dermatology, Aichi Medical University, Aichi, Japan Abstract Progressive nodular histiocytosis (PNH) is a rare benign self-limiting histiocytic disorder of the skin that is characterised by the progressive appearance of widespread xanthomatous lesions with no spontaneous remission. We operated on a 13-year-old girl with PNH four times with a result that had stabilised the condition at 1 year. Key Words: Progressive nodular histiocytosis, juvenile xanthogranuloma, radical surgical treatment
Introduction Progressive nodular histiocytosis (PNH) was first described as progressive nodular histiocytoma by Taunton et al. [1]. It is a proliferative disorder that mainly involves dermal dendrocytes. Its histological appearance is similar to that of juvenile xanthogranuloma (JXG) [2,3], in which xanthomatous papules and nodules develop on the skin from early infancy. Most lesions are solitary, and may resolve spontaneously, so the role of surgical treatment is strictly limited. However, PNH is characterised by the progressive and widespread appearance of xanthomatous lesions that do not resolve spontaneously and are resistant to treatment. Surgical resection of selected nodules is therefore the only therapeutic option. There have been few reports about PNH, and even fewer reports about surgical treatment. Case report A 13-year-old girl presented with an 8-year history of skin lesions with progressive enlargement, which was diagnosed as PNH [4]. Several treatments were attempted, including chemotherapy, but all efforts were unsuccessful. At the age of 12, she was referred to our hospital. On examination, there were widespread asymptomatic red-brown papules and nodules on her trunk, extremities, lips, palms, and fingers. The nodules were clustered near large skinfolds, such as the axillae. There were flattened nodules on the palms, and most of the lesions on the fingers were located on the lateral aspect over the joints or adjacent to the proximal nailfold [5,6]. The joints of each finger moved normally, but her nails had become deformed adjacent to the nodules. The skin overlying the larger nodules was ulcerated. The maximum diameter of the nodules was >25 mm. Laboratory investigations including complete blood cell count and serum chemistry with serum lipid levels and kidney and liver function tests showed no abnormality. There was no clinical evidence of neurofibromatosis and no internal involvement. With the
consent of the patient, large nodules were excised, mainly for cosmetic reasons. She had a total of four operations. Initially, these were done under local anaesthesia. Wound healing was normal and the histopathological findings resembled those of JXG. There was no postoperative progression or seeding of skin lesions [7]. Clinically, there were features of PNH, but it did not seem to be untreatable. More extensive operation was therefore done under general anaesthesia. Most of the lesions were simply excised with an ellipse of skin along the tension lines, after which the incision was closed easily. However, skin closure was difficult to achieve in the fingers and palms, and at sites with many nodules. In PNH, the nodules are tightly adherent to the surrounding skin, as indicated by the histological finding of epithelial collarettes (Figure 1). Preservation of the overlying skin was therefore difficult, but it was required to achieve closure at some sites. The nodules were therefore divided along the tension lines of the skin or the midlateral line of the finger, after which the epithelial collarette was chipped off and parts of the nodules were peeled off and removed subcutaneously. The preserved skin was then trimmed for accurate closure (Figure 2). This way, a total of about 200 nodules were removed. At the 1-year follow-up, the disease seemed stable (Figure 3). Discussion Although PNH might not be serious, and scars could be an aesthetic problem, the widespread skin nodules were problematic for an adolescent girl [8,9]. Many of this girl’s nodules were ulcerated and seemed likely to leave scars if they regressed in the future, so removal was planned. Specimens of the excised xanthomatous tumours showed well-demarcated nodular lesions growing within the papillary dermis. The overlying epidermis extended deeply downwards at the rete ridges and epithelial collarettes were present. We
Correspondence: Norihisa Nakayashiki, MD, PhD, Department of Plastic and Reconstructive Surgery, Fujita Health University, 1-98 Dengakugakubo, Kutsugake-cho, Toyoake, Aichi 470-1192, Japan. Tel: +81 562 93 9249. Fax: +81 562 93 3083. E-mail: [email protected] (Accepted 27 January 2011)
Treatment of progressive nodular histiocytosis
a
81
b
Figure 1. Haematoxylin-and-eosin-stained specimens of resected nodules. (a) The epidermis showing deep extension of the rete ridge (r) overlying a tumour (T) (original magnification 100). (b) An epithelial collarette (c) at the lateral margins of a tumour (original magnification 40).
a
b
c
d
e
f
g
h
Figure 2. Excision of the tumour from the lateral aspect of a finger. (a) An incision line designed along the midlateral line of the finger. (b) The tumour divided into two parts. (c) The epithelial collarette cut between the epidermis overlying the tumour and the normal dermis. (d) The epidermis peeled off the tumour. (e) The fragile epidermis trimmed. (f) The skin closed. (g) Preoperative view. (h) Postoperative view at 9 months.
82 N. Nakayashiki et al.
a
b
c
d
e
f
Figure 3. Preoperative views of (a, b) the hands and (e) the groin. (c, d, f) postoperative views at 9 months.
therefore adopted the chipped-off technique for excision, particularly on the fingers and palms. The overlying epidermis was fragile in some areas at the rete ridges, but could usually be preserved. Almost all preserved epidermis stayed alive. Our procedure made it easy to remove the nodules efficiently. Although en bloc resection of tumours is usual, this patient had benign disease and complete removal was not required. The postoperative result was satisfactory and the patient was happy to have smoother skin.
In conclusion, this was a rare case of PNH that was treated radically. Although PNH is a benign disease, extensive surgical treatment with the chipped-off technique is an effective treatment. This may also be used for the treatment of other histiocytic diseases. Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.
Treatment of progressive nodular histiocytosis References [1] Taunton OD, Yeshurun D, Jarratt M. Progressive nodular histiocytoma. Arch Dermatol 1978;114:1505–8. [2] Weitzman S, Jaffe R. Uncommon histiocytic disorders: the non-Langerhans cell histiocytoses. Pediatr Blood Cancer 2005;45:256–64. [3] Zelger BW, Sidoroff A, Orchard G, Cerio R. Non-Langerhans cell histiocytoses. A new unifying concept. Am J Dermatopathol 1996;18:490–504. [4] Watanabe T, Watanabe D, Tamada Y, Matsumoto Y. Progressive nodular histiocytosis - a five-year follow up. Eur J Dermatol 2008;18:200–2. [5] Hughes DB, Hanasono MM, Nolan WB III. Juvenile xanthogranuloma of the finger. Pediatr Dermatol 2006;23:53–5.
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[6] Kim EJ, Kim MY, Kim HO, Park YM. Juvenile xanthogranuloma of the finger: an unusual localization. J Dermatol 2007;34: 590–2. [7] Numajiri T, Nishino K, Fujiwara T, Takeda K, Sowa Y. Juvenile xanthogranuloma presenting rapid progression after curettage: a case report with clinicopathological findings. J Plast Reconstr Aesthet Surg 2007;60:1248–51. [8] Vadoud-Seyedi J, Vadoud-Seyedi R, De Dobbeleer G. Progressive nodular histiocytomas. Br J Dermatol 2000;143: 678–9. [9] Gonzalez Ruíz A, Bernal Ruíz AI, Aragoneses Fraile H, Peral Martinez I, García Muñoz M. Progressive nodular histiocytosis accompanied by systemic disorders. Br J Dermatol 2000; 143:628–31.
Copyright of Journal of Plastic Surgery & Hand Surgery is the property of Taylor & Francis Ltd and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
CASE REPORT
Effective surgical treatment of progressive nodular histiocytosis Norihisa Nakayashiki1, Hirotaka Akita2, Wakako Mori1, Daisuke Watanabe3, Yasuo Sakai1 & Yohko Yoshimura1 1
Department of Plastic and Reconstructive Surgery, 2Department of Dermatology, Fujita Health University, Aichi and 3Department of Dermatology, Aichi Medical University, Aichi, Japan Abstract Progressive nodular histiocytosis (PNH) is a rare benign self-limiting histiocytic disorder of the skin that is characterised by the progressive appearance of widespread xanthomatous lesions with no spontaneous remission. We operated on a 13-year-old girl with PNH four times with a result that had stabilised the condition at 1 year. Key Words: Progressive nodular histiocytosis, juvenile xanthogranuloma, radical surgical treatment
Introduction Progressive nodular histiocytosis (PNH) was first described as progressive nodular histiocytoma by Taunton et al. [1]. It is a proliferative disorder that mainly involves dermal dendrocytes. Its histological appearance is similar to that of juvenile xanthogranuloma (JXG) [2,3], in which xanthomatous papules and nodules develop on the skin from early infancy. Most lesions are solitary, and may resolve spontaneously, so the role of surgical treatment is strictly limited. However, PNH is characterised by the progressive and widespread appearance of xanthomatous lesions that do not resolve spontaneously and are resistant to treatment. Surgical resection of selected nodules is therefore the only therapeutic option. There have been few reports about PNH, and even fewer reports about surgical treatment. Case report A 13-year-old girl presented with an 8-year history of skin lesions with progressive enlargement, which was diagnosed as PNH [4]. Several treatments were attempted, including chemotherapy, but all efforts were unsuccessful. At the age of 12, she was referred to our hospital. On examination, there were widespread asymptomatic red-brown papules and nodules on her trunk, extremities, lips, palms, and fingers. The nodules were clustered near large skinfolds, such as the axillae. There were flattened nodules on the palms, and most of the lesions on the fingers were located on the lateral aspect over the joints or adjacent to the proximal nailfold [5,6]. The joints of each finger moved normally, but her nails had become deformed adjacent to the nodules. The skin overlying the larger nodules was ulcerated. The maximum diameter of the nodules was >25 mm. Laboratory investigations including complete blood cell count and serum chemistry with serum lipid levels and kidney and liver function tests showed no abnormality. There was no clinical evidence of neurofibromatosis and no internal involvement. With the
consent of the patient, large nodules were excised, mainly for cosmetic reasons. She had a total of four operations. Initially, these were done under local anaesthesia. Wound healing was normal and the histopathological findings resembled those of JXG. There was no postoperative progression or seeding of skin lesions [7]. Clinically, there were features of PNH, but it did not seem to be untreatable. More extensive operation was therefore done under general anaesthesia. Most of the lesions were simply excised with an ellipse of skin along the tension lines, after which the incision was closed easily. However, skin closure was difficult to achieve in the fingers and palms, and at sites with many nodules. In PNH, the nodules are tightly adherent to the surrounding skin, as indicated by the histological finding of epithelial collarettes (Figure 1). Preservation of the overlying skin was therefore difficult, but it was required to achieve closure at some sites. The nodules were therefore divided along the tension lines of the skin or the midlateral line of the finger, after which the epithelial collarette was chipped off and parts of the nodules were peeled off and removed subcutaneously. The preserved skin was then trimmed for accurate closure (Figure 2). This way, a total of about 200 nodules were removed. At the 1-year follow-up, the disease seemed stable (Figure 3). Discussion Although PNH might not be serious, and scars could be an aesthetic problem, the widespread skin nodules were problematic for an adolescent girl [8,9]. Many of this girl’s nodules were ulcerated and seemed likely to leave scars if they regressed in the future, so removal was planned. Specimens of the excised xanthomatous tumours showed well-demarcated nodular lesions growing within the papillary dermis. The overlying epidermis extended deeply downwards at the rete ridges and epithelial collarettes were present. We
Correspondence: Norihisa Nakayashiki, MD, PhD, Department of Plastic and Reconstructive Surgery, Fujita Health University, 1-98 Dengakugakubo, Kutsugake-cho, Toyoake, Aichi 470-1192, Japan. Tel: +81 562 93 9249. Fax: +81 562 93 3083. E-mail: [email protected] (Accepted 27 January 2011)
Treatment of progressive nodular histiocytosis
a
81
b
Figure 1. Haematoxylin-and-eosin-stained specimens of resected nodules. (a) The epidermis showing deep extension of the rete ridge (r) overlying a tumour (T) (original magnification 100). (b) An epithelial collarette (c) at the lateral margins of a tumour (original magnification 40).
a
b
c
d
e
f
g
h
Figure 2. Excision of the tumour from the lateral aspect of a finger. (a) An incision line designed along the midlateral line of the finger. (b) The tumour divided into two parts. (c) The epithelial collarette cut between the epidermis overlying the tumour and the normal dermis. (d) The epidermis peeled off the tumour. (e) The fragile epidermis trimmed. (f) The skin closed. (g) Preoperative view. (h) Postoperative view at 9 months.
82 N. Nakayashiki et al.
a
b
c
d
e
f
Figure 3. Preoperative views of (a, b) the hands and (e) the groin. (c, d, f) postoperative views at 9 months.
therefore adopted the chipped-off technique for excision, particularly on the fingers and palms. The overlying epidermis was fragile in some areas at the rete ridges, but could usually be preserved. Almost all preserved epidermis stayed alive. Our procedure made it easy to remove the nodules efficiently. Although en bloc resection of tumours is usual, this patient had benign disease and complete removal was not required. The postoperative result was satisfactory and the patient was happy to have smoother skin.
In conclusion, this was a rare case of PNH that was treated radically. Although PNH is a benign disease, extensive surgical treatment with the chipped-off technique is an effective treatment. This may also be used for the treatment of other histiocytic diseases. Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.
Treatment of progressive nodular histiocytosis References [1] Taunton OD, Yeshurun D, Jarratt M. Progressive nodular histiocytoma. Arch Dermatol 1978;114:1505–8. [2] Weitzman S, Jaffe R. Uncommon histiocytic disorders: the non-Langerhans cell histiocytoses. Pediatr Blood Cancer 2005;45:256–64. [3] Zelger BW, Sidoroff A, Orchard G, Cerio R. Non-Langerhans cell histiocytoses. A new unifying concept. Am J Dermatopathol 1996;18:490–504. [4] Watanabe T, Watanabe D, Tamada Y, Matsumoto Y. Progressive nodular histiocytosis - a five-year follow up. Eur J Dermatol 2008;18:200–2. [5] Hughes DB, Hanasono MM, Nolan WB III. Juvenile xanthogranuloma of the finger. Pediatr Dermatol 2006;23:53–5.
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[6] Kim EJ, Kim MY, Kim HO, Park YM. Juvenile xanthogranuloma of the finger: an unusual localization. J Dermatol 2007;34: 590–2. [7] Numajiri T, Nishino K, Fujiwara T, Takeda K, Sowa Y. Juvenile xanthogranuloma presenting rapid progression after curettage: a case report with clinicopathological findings. J Plast Reconstr Aesthet Surg 2007;60:1248–51. [8] Vadoud-Seyedi J, Vadoud-Seyedi R, De Dobbeleer G. Progressive nodular histiocytomas. Br J Dermatol 2000;143: 678–9. [9] Gonzalez Ruíz A, Bernal Ruíz AI, Aragoneses Fraile H, Peral Martinez I, García Muñoz M. Progressive nodular histiocytosis accompanied by systemic disorders. Br J Dermatol 2000; 143:628–31.
Copyright of Journal of Plastic Surgery & Hand Surgery is the property of Taylor & Francis Ltd and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
