This article has Open Peer Review reports available.
Langerhans cell histiocytosis on the penis: a case report
© Hagiuda et al; licensee BioMed Central Ltd. 2006
Received: 24 May 2006
Accepted: 11 October 2006
Published: 11 October 2006
Langerhans cell histiocytosis affects mainly young children and features an accumulation of CD1a+ dendritic Langerhans cells in the bone, skin, and other organs. A few cases of Langerhans cell histiocytosis on the penis have been reported in the literature. We present a case of Langerhans cell histiocytosis on the penis and review the similar cases in the literature.
The patient was a 13-year-old boy who had a history of lymph node, femur bone, and pituitary-thalmic axis lesions from Langerhans cell histiocytosis who noticed a painful nodule on the prepuce of his penis. The histological and immunohistochemical examination fulfilled the criteria of Langerhans cell histiocytosis.
We herein describe the case reported of Langerhans cell histiocytosis on the penis.
Langerhans cell histiocytosis (LCH) is characterized by an organ-specific infiltration of cells with many morphological features and immunohistochemical markers of Langerhans cells. Clinically, LCH ranges from self-healing lesions to a multi-system involvement with organ dysfunction resistant to current therapies. The lesions appear in multiple organs, for example in the bones, skin, and lungs, but a lesion localized on the penis is uncommon. We herein report a case of LCH on the penis.
Histiocytosis X, which included Letterer-Siwe's disease, Hand-Shuller-Christian's disease, and eosinophilic granuloma, was renamed Langerhans cell histiocytosis in 1985 by the Histiocytic Society . The diagnosis of LCH has been based on a histopathological pattern in biopsy specimens showing mono- or multinucleated Langerhans cells, histiocytes, and eosinophils. The presence of Birbeck granules on electron microscopic examination or of antigenic markers that react with CD1a glycoprotein and the cytoplasmic protein S100 detected by immunostain is considered diagnostic, as shown in the present case. LCH lesions are common in the bone, lung, skin, liver, spleen, and lymph nodes. In 30.6% of patients, LCH involved more than one body system. Twenty-five percent of patients had skin and/or mucous membrane LCH. The most common mucous membranes involved are the genitalia and oral mucous . In the current literature, we found only 6 cases of a penile lesion, reported by Myers et al. , Caputo et al. , Yokota et al. , Meehan et al. , Seseke F et al. , and Maekawa et al., respectively. Each case was treated by surgical excision, chemotherapy, or steroid ointment, and there are no reports in the literature of recurrence on the penis. Although treatment of vulvar lesions with radiation and surgical excision did not prevent recurrence , penile lesions appear to be too sensitive for this type of treatment. In a prior report, distinct Langerhans cell preponderance is shown in the transformation zone of the cervix and the vulva. Langerhans cells are modulated actively in response to certain chemical stimuli, human papillomavirus infection, and cervical intraepithelial neoplasia . It is not proven that the distribution of Langerhans cells or various stimuli to the penis may be related to the rare nature of penile lesions or the good response to the treatment. In the adult cases, there are sole cutaneous involvements of the penis. These cutaneous lesions in adult life may simply represent a limited form of LCH with an excellent prognosis. It is also possible that such lesions are the initial presentation of one of the multi-system instances of LCH .
Patients with localized LCH may have a good chance of spontaneous remission and a favorable outcome over a period of months to years . The important factors for predicting recurrent disease and poor prognosis include LCH involving both bone and mucocutaneous tissue, LCH involving both osseous and extraosseous tissues, relapse after treatment of patients with osseous LCH and multi-systemic LCH, >3 bones involvement, the presence of mucous membrane LCH, the presence of hepatosplenomegaly in patients <3 years of age at presentation, pituitary-thalamic axis LCH in the presence of multi-systemic LCH, young age (<5 years) at presentation, and LCH involving 3 or more body systems . This patient had presented LCH at 3 years old and had already relapsed after the treatment for 3 lesions when the penile lesion appeared. Because this may indicate poor prognosis, we will continue to follow the patient closely, to watch for the development of any additional lesions.
We thank the patient for giving consent for publication of this case report.
JH designed this case report and drafted the manuscript. SA and IK carried out the operation of this patient and helped to draft the manuscript. MU and ND participated in the design of the study and helped to draft the manuscript. KI carried out the pathological examination. All authors read and approved the final manuscript.
- Writing Group of the Histiocyte Society: Histiocytosis syndromes in children. Lancet. 1987, 1 (8526): 208-9.Google Scholar
- Howarth DM, Gilchrist GS, Mullan BP, Wiseman GA, Edmonson JH, Schomberg PJ: Langerhans cell histiocytosis; diagnosis, natural history, management, and outcome. Cancer. 1999, 85: 2278-90. 10.1002/(SICI)1097-0142(19990515)85:10<2278::AID-CNCR25>3.0.CO;2-U.View ArticlePubMedGoogle Scholar
- Myers DA, Strandjord SE, Marcus RB: Histiocytosis X presenting as a primary penile lesion. J Urol. 1981, 126: 268-9.PubMedGoogle Scholar
- Caputo R, Gianotti R, Monti M: Nodular 'pure' mococutaneous histiocytosis X in an adult. Arch Dermatol. 1987, 123: 1274-1275. 10.1001/archderm.123.10.1274.View ArticlePubMedGoogle Scholar
- Yokota T, Aga Y, Miki H, Oomori M: A case of histiocytosis X of the penis. Jpn J Urol. 1990, 81: 1904-7.View ArticleGoogle Scholar
- Meehan SA, Smoller BR: Cutaneous Langerhans cell histiocytosis of the genitalia in the elderly: A report of three cases. J Cutan Pathol. 1998, 25: 370-374. 10.1111/j.1600-0560.1998.tb01760.x.View ArticlePubMedGoogle Scholar
- Seseke F, Kugler A, Hermanns M, Zoller G, Kallerhoff M, Ringert RH: Langerhans cell histiocytosis of the penis. Urologe. 1999, 38: 42-45. 10.1007/s001200050243.View ArticlePubMedGoogle Scholar
- Maekawa Y, Kogushi H: A case of Langerhans cell histiocytosis on the foreskin and external auditory meatus. J Pediat Dermatol. 2004, 23: 35-38.Google Scholar
- Singh A, Prieto V, Czelusta A, Czelusta A, McClain K, Duvic M: Adult Langerhans cell histiocytosis limited to the skin. Dermatology. 2003, 207: 157-161. 10.1159/000071786.View ArticlePubMedGoogle Scholar
- Velez-Yanguas MC, Warrier RP: Langerhans cell histiocytosis. Orthop Clin North Am. 1996, 27: 615-23.PubMedGoogle Scholar
- The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2490/6/28/prepub
This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.