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DERMATOLOGICA SINICA 29 (2011) 98e100

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Dermatologica Sinica

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CASE REPORT Progressive nodular : a rare type of xanthogranuloma

Fu-Chen Chuang 1,2, Erick Chern 1,2, Wei-Ming Wu 1,2,*

1 Department of Dermatology, Chang Gung Memorial Hospital-Kaohsiung Medical Center, Chang Gung University, Kaohsiung, Taiwan 2 Chang Gung University, College of Medicine, Kaohsiung, Taiwan

article info abstract

Article history: Progressive nodular histiocytosis is a rare non-Langerhans histiocytosis. It tends to occur in adult with Received: Nov 8, 2010 progressive course that differentiates from other groups of xanthogranuloma. The clinical presentation is Revised: Dec 17, 2010 characteristic and disfiguring of the face is not uncommon. Extracutaneous involvement and metabolic Accepted: Dec 20, 2010 association were reported in a few cases. Treatment is unsatisfactory and follow-up is suggested for possible complication. Herein, we reported a typical case of the disease. Copyright Ó 2011, Taiwanese Dermatological Association. Keywords: Published by Elsevier Taiwan LLC. All rights reserved. Non-Langerhans histiocytosis Progressive nodular histiocytosis Xanthogranuloma

Introduction increased in a progressive fashion without spontaneous regression. The face gradually showed a leonine feature (Figure 1). His respi- Progressive nodular histiocytosis (PNH) is a subgroup of xanthog- ratory pattern is smooth and there are no dysphagia, hearing ranuloma and belongs to the class of non-Langerhans cell histio- impairment, or signs of ocular involvement. There is no family cytosis. It was first described by Taunton et al1 in 1978. It member sharing similar cutaneous presentation. The laboratory predominantly affects the young adult in their 3rde4th decades of survey disclosed to as high as 334 mg/dL and life. The patient usually presents with multiple yellowish-brown, a normal cholesterol level. A skin biopsy taken from the largest variable-sized papulonodules involving mainly the head, neck, nodule on face revealed diffused dermal infiltrate, composed of trunk, and the limbs.2 The lesions tend to progress without spon- spindle cells with variable foamy cytoplasm and multinucleated taneous resolution. It is characterized histologically by diffuse giant cells, especially the Touton type (Figure 2). The spindle cells infiltrate of spindle-shaped histiocytes with whorl-like growth comprised nearly 90% of the cells and showed a storiform pattern, admixed with xanthomatized histiocytes and Touton giant arrangement. There was no cellular atypia and mitotic activity was cells. Herein, we reported a typical case of the disease. absent. The histiocytes stained positive for CD68 and the spindle cells were negative for smooth muscle actin and desmin. For the Case report cosmetic purpose, the patient later underwent surgical excision of the larger nodules on face. A 46-year-old man presented with numerous pedunculated asymptomatic papulonodules over face, trunk, and the upper limbs. Discussion The lesions varied from 0.3 cm to 2 cm and were firm in consis- tency. Telangiectasia on an erythematous to yellowish background PNH is a rare entity and there were only 15 cases reported in the can be easily appreciated. The nodules have developed since 6 years literature to date. The typical histology is proliferation of storiform, ago, beginning from face to trunk. He ever received skin biopsy spindle-shaped histiocytes, confining to the dermis but it may when the lesions were only several and scattered over the involve as deep as the muscle layer. Xanthomatous, vacuolated face. was told. However, the nodules histiocytes or multinucleated giant cells may be abundant. The histiocytes are positive for the macrophage/dendritic cell marker such as CD68 and negative for S100 and CD1a, suggesting the origin of non-Langerhans cell. The histological findings resemble several * Corresponding author. Department of Dermatology, Chang Gung Memorial xanthogranulomatous diseases, such as juvenile xanthogranuloma, Hospital-Kaohsiung Medical Center, Chang Gung University, No. 123, Ta-Pei Road, Niao-Sung County, Kaohsiung, Taiwan. papular , benign cephalic histiocytosis, generalized 3 E-mail address: [email protected] (W.-M. Wu). eruptive histiocytosis, and xanthoma disseminatum. Chu had

1027-8117/$ e see front matter Copyright Ó 2011, Taiwanese Dermatological Association. Published by Elsevier Taiwan LLC. All rights reserved. doi:10.1016/j.dsi.2011.07.005 F.-C. Chuang et al. / Dermatologica Sinica 29 (2011) 98e100 99

Figure 1 (A) Multiple pedunculated papulonodules mainly over face, showing a leonine feature. (B) Also scattered nodules involve the trunk. (C) Close view of the nodule shows yellowish appearance with telangiectasia. suggested a possible continuity of the diseases as a spectrum. cases of xanthogranuloma were reported to occur during adulthood, However, the differential diagnosis is still possible on clinical the lesion was usually solitary.4 Benign cephalic histiocytosis and ground. Juvenile xanthogranuloma usually onsets before the age of 6 generalized eruptive histiocytosis are usually self-limited as well, years and mostly shows a spontaneous resolution. Although several whereas xanthoma disseminatum has a predilection for the flexural

Figure 2 (A)Whorls of spindle-shaped histiocytes admixed with foamy histioctyes and multinucleated giant cells infiltrate the entire dermis [hematoxylin and eosin (H&E); original magnification, Â40]. (B) Touton (arrow) and foreign body (arrow head) giant cells can be easily appreciated (H&E; original magnification, Â200). (C) CD68 stains positive for the histiocytes (original magnification, Â200). 100 F.-C. Chuang et al. / Dermatologica Sinica 29 (2011) 98e100 area with frequent visceral organ involvement.5 The progressive Treatment of PNH is usually unsatisfactory. Surgical excision course and typical pedunculated nodules characterize the PNH. provides temporary relief, but local recurrence is very likely. However, early diagnosis may be difficult when the lesion number is Systemic corticosteroid, cyclophosphamide, radiotherapy, or few and therefore long-term follow up is necessary for the sus- chemotherapy, such as vincristine, tends to be ineffective.8,9 pected cases. Our patient with protracted clinical course and char- However, long-term follow-up is still necessary for early detec- acteristic histological feature is a typical case of PNH. tion to avoid possible complication. PNH is regarded as solely a cutaneous disease without tendency of extracutaneous involvement. Systemic survey is required only References when symptoms are present. However, Glavin et al5 reported a case with a 7-year history of the progressive skin nodularities showing 1. Taunton OD, Yeshurun D, Jarratt M. Progressive nodular histiocytoma. Arch Dermatol 1978;114:1505e8. biopsy-proved extensive laryngeal and pharyngeal involvement 2. Zelger B, Cerio R, Orchard G, Wilson-Jones E, Burgdorf WH. Solitary and that cause dyspnea and dysphagia. Another case report suggests generalized variants of spindle cell xanthogranuloma. Histopathology 1995;27: the hypothalamic involvement with precocious puberty and 11e9. 6 7 3. Chu AC. The confusing state of the histiocytoses. Br J Dermatol 2000;143:475e6. growth failure. Gonzalez Ruíz et al also imply a possible corre- 4. Chang MW. Update on juvenile xanthogranuloma: unusual cutaneous and lation between chronic myeloid leukemia and long-lasting PNH. systemic variants. Semin Cutan Med Surg 1999;18:195e205. The association between lipid metabolism and PNH is obscure. 5. Glavin FL, Chhatwall H, Karimi K. Progressive nodular histiocytosis: a case report fi Although most of the patients with PNH are normolipemic like with literature review, and discussion of differential diagnosis and classi cation. J Cutan Pathol 2009;36:1286e92. those with juvenile xanthogranuloma, coexisting lipid dysfunction 6. Beswick SJ, Kirk JM, Bradshaw K, Sanders DS, Moss C. Progressive nodular was reported. was noted in a 34-year-old histiocytosis in a child with a hypothalamic tumor. Br J Dermatol 2002;146: e woman with PNH for 7 years.5 Gonzalez Ruíz et al7 demonstrated 128 40. 7. Gonzalez Ruíz A, Bernal Ruíz AI, Aragoneses Fraile H, Peral Martinez I, García another patient with PNH for 26 years showing hypothyroidism Muñoz M. Progressive nodular histiocytosis accompanied by systemic disorders. and hypocholesterolemia.7 Our patient presented with hyper- Br J Dermatol 2000;143:628e31. triglyceridemia and a normal serum cholesterol level. Whether 8. Watanabe T, Watanabe D, Tamada Y, Matsumoto Y. Progressive nodular histiocytosis-a five-year follow up. Eur J Dermatol 2008;18:200e2. is a collision phenomenon or does it play a role in 9. Vadoud-Seyedi J, Vadoud-Seyedi R, De Dobbeleer G. Progressive nodular his- developing PNH requires further evidence. tiocytomas. Br J Dermatol 2000;143:678e9.