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Localised vulva Langerhans cell histiocytosis

European Journal of Dermatology. Volume 14, Number 6, 412-4, November-December 2004, Clinical report

Summary

Author(s) : Hikaru ISHIGAKI, Naohito HATTA, Mizuki YAMADA, Hidemitsu ORITO, Kazuhiko TAKEHARA , Department of Dermatology, Kanazawa University School of Medicine, 13-1 Takaramachi, Kanazawa, 920-8641 Japan, Division of Dermatology, Suzu City Hospital, Suzu, 927-1213 Japan.

Summary : A cutaneous lesion as sole manifestation of adult Langerhans cell histiocytosis is rare. We report a 65-year old female who presented with vulvar ulcers. Histological examination of the ulcer showed diffuse proliferation of histiocytic cells with large bright cytoplasm that were positively stained for anti-S-100 protein and anti-CD1a antibodies by immunohistochemistry. Electron microscopy demonstrated Birbeck granules in the cytoplasm of the cells. Since there was no other organ involvement, the patient was treated by complete surgical excision without recurrence or other-organ involvement in the 1-year follow-up period.

Keywords : Langerhans cell histiocytosis, ulcer, vulva

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Auteur(s) :, Hikaru ISHIGAKI¹, Naohito HATTA^1,*, Mizuki YAMADA¹, Hidemitsu ORITO², Kazuhiko TAKEHARA¹

¹Department of Dermatology, Kanazawa University School of Medicine, 13-1 Takaramachi, Kanazawa, 920-8641 Japan
²Division of Dermatology, Suzu City Hospital, Suzu, 927-1213 Japan

accepté le 15 Mars 2004

Langerhans cell histiocytosis (LCH) includes diseases previously called histiocytosis X, eosinophilic granuloma and Lettere-Siwe disease. LCH is characterized by a broad spectrum of clinical presentations which involves one or more body organ systems such as bone, liver, hypothalamus, skin and mucous membrane [1, 2]. These diseases are usually observed in children, however, they sometimes affect elderly patients. Solitary LCH, which is most often seen in adults, has a benign clinical course, while multi-organ LCH, most often seen in children, has an acute, aggressive and sometimes fatal clinical course. We here report an adult case where LCH presented with vulvar ulcers and discuss the literature of similar clinical features.

Case report

A 65-year-old Japanese woman first visited our clinic with a 2-month-history of stinging on urination. Physical examination revealed multiple ulcers, 20-30 mm in diameter, on the vulva and perineum lesion. These lesions were infiltrated and were surrounded with red papules (( Figure 1 )). There was bilateral inguinal lymph node swelling but no hepato-splenomegaly. Laboratory examination results were almost normal. A skeletal radiographic survey showed no evidence of bone involvement. Computed tomography did not show any abnormality suggesting multi-organ involvement, except for swollen inguinal lymph nodes.

A biopsy taken from the ulcer revealed diffuse proliferation of large histiocytic cells with bright cytoplasm in the dermis and subcutaneous lesions, accompanied by scattered eosinophils. The large histiocytic cells had lobulated and grooved ‘coffee bean’ or reniform nuclei (( Figure 2 )) and were positive for S-100 protein, CD1a, CD4 and HLA-DR antigen by immunohistochemistry using specific antibodies (( Figure 3 )). Ultrastructurally, the proliferating cells were found to contain typical Birbeck granules in the cytoplasm (( Figure 4 )). In view of all of these findings, the patient was diagnosed as having LCH.

She underwent complete excision of the genital lesion followed by local skin flap-reconstruction, and bilateral inguinal lymph node biopsy. No tumor cells were found in the lymph nodes. There has been no evidence of local or systemic disease during 12 months of follow up.

Discussion

The cutaneous lesions may be a sole manifestation of LCH, in addition to multi-organ diseases. The seborrheic dermatitis-like lesions on the scalp and the flexeres (groin, perianal, axilla, neck and behind the ear) are typical of the skin involvement of LCH [2]. Although genital lesions are relatively rare for skin involvement of LCH, at least 24 adult cases with genital manifestation have been reported in the English literature [3-12] (Table I( Table I )). All but one patient was female and the median age was 38 years. Vulval ulcers with or without papules or nodules were the most common clinical feature. Five of the 22 (23%) also presented with other extragenital skin involvements such as head and ‘flexer’ lesions. Thus, vulvar ulcers seem to be one of the typical cutaneous manifestations of adult LCH. We must consider the possibility of LCH when we encounter a woman with atypical chronic ulcers or papulo-erythematous lesions on the vulva.

However, the clinical diagnosis of any ulcerative lesion is difficult and a biopsy is usually needed for correct diagnosis. Histologically, the lesion contains proliferating Langerhans-type histiocytes, characterized by eosinophilic cytoplasm and reniform vesicular nuclei. These cells express S-100 protein and exhibit a D-mannosidase activity. Definitive diagnosis requires the findings of Birbeck granules by electron microscopy or positivity for CD1a by immunohistochemistry in lesional cells. The Writing Group of the Histiocyte Society has identified three levels of confidence in the diagnosis of LCH [13]. The present case was diagnosed as “definitive LCH” by these criteria.

Treatment for genital LCH is not well-defined because there have been few reports of cases. In general, the treatment strategy is different for patients with and without systemic involvement. In the patients with genital LCH, 9 of the 21 (43%) were complicated with systemic disease. Systemic LCH may be present at the same time, or develop subsequently. A single systemic disease can be treated with local therapy including surgical excision, radiotherapy, topical corticosteroid, and topical nitrogen mustard. For multisystem LCH, cyclosporin, 2-chlorodeoxyadenisine and etoposide are used [1, 2]. Complete remissions were reported with vincristine and vinblastine in genital LCH [8, 9]. We performed local excision in the present case as there was no systemic sign of LCH. Although there was no evidence of recurrence in the 1-year follow-up period, further observation will be needed for both local recurrence and subsequent multi-organ disease.
Table I Genital Langerhans Cell Histiocytosis

No.	Age	Gender	Cutaneous presentation		Systemic disease	Treatment	Outcome	Ref.
Genital	Ex-genital
1	NA	F	vulvar ulcer	(-)	(-)	SX	IMP	[3]
2	38	F	vulvar ulcer	(-)	(-)	SX	CR	[3]
3	42	F	vulvar ulcer	(-)	(-)	RTX	PR	[3]
4	29	F	vulvar ulcer	(-)	(-)	SX, RTX	UR	[3]
5	33	F	cervical ulcer	(-)	(-)	SX	CR	[3]
6	85	F	vulvar ulcer	(-)	(-)	steroid	UR	[3]
7	36	F	vulvar ulcer	skin eruption	DI, lung,bone	RTX, SX, steroid	UR	[3]
8	36	F	vulvar induration	(-)	lung	SX	CR	[3]
9	38	F	vaginal papules	skin lesion	DI	RTX	CR	[3]
10	20	F	vulvar ulcer	(-)	bone	RTX	CR	[3]
11	35	F	vulvar nodules	(-)	DI	RTX	IMP	[3]
12	22	F	vulvar nodules	(-)	bone	steroid	CR	[3]
13	27	F	vaginal discharge	(-)	bone	CTX, steroid	PR	[3]
14	76	F	vulvar papules/erosion	NA	NA	NA	NA	[4]
15	54	F	vulvar papules	NA	NA	NA	NA	[4]
16	54	M	palules on penis	NA	NA	NA	NA	[4]
17	62	F	vulvar ulcer	(-)	bone	CTX, RTX, SX	CR	[5]
18	65	F	vulvar ulcer	axilla	(-)	RTX	CR	[6]
19	46	F	vulvar ulcer	axilla, scalp, groin	NA	NA	NA	[7]
20	19	F	vulvar lesion	(-)	bone	CTX	CR	[8]
21	40	F	vulvar ulcer	(-)	(-)	CTX, SX	CR	[9]
22	45	F	vulvar ulcer	(-)	(-)	RTX	CR	[10]
23	90	F	perivulvar lesion	perianal, axilla	(-)	nitrogen mustard	CR	[11]
24	36	F	vulvar ulcer	(-)	(-)	RTX	CR	[12]
25	65	F	vulvar ulcer	(-)	(-)	SX	CR	present case

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