Vol. 21, No. 3, 2009
Received November 10, 2008, Accepted for publication December 24,
Reprint request to: Moon Kyun Cho, M.D., Department of Dermatology,
College of Medicine, Soonchunhyang University, 22, Daesagwan-gil,
Yongsan-gu, Seoul 140-743, Korea. Tel: 82-2-709-9368, Fax: 82-2-
709-9374, E-mail: MKCho2001@yahoo.co.kr
Ann DermatolVol. 21, No. 3, 2009
Fig. 1. Three brown lichenoid patches on left dorsal foot.
Langerhans Cell Histiocytosis Presenting as Brown
Hyuk Kwon, M.D., Jang Hyun Lee, M.D.1, Sung Keun Kim, M.D., Young Lip Park, M.D.,
Jong Suk Lee, M.D., Moon Kyun Cho, M.D.
Department of Dermatology, College of Medicine, Soonchunhyang University, Seoul, 1Department of Plastic and Reconstruction Surgery,
Hanyang University Guri Hospital, Guri, Korea
Langerhans cell histiocytosis (LCH) is related diseases
characterized by proliferation of Langerhans cell with
involvement of bone, skin, lung and other organs. LCH
usually occurs in childhood and are presented as multiple
small papules or eczematoid lesion mostly. We report a
50-year-old man with 3 brown lichenoid patches on left
dorsal foot. He was diagnosed pulmonary LCH 5 years ago.
Typical LC cells on skin lesion and CD1 complex positive
staining confirm the diagnosis of LCH. We consider brown
lichenoid patches may be a previously unreported
cutaneous presentation in cutaneous or multisystem LCH.
(Ann Dermatol 21(3) 277∼∼280, 2009)
Langerhans cell histiocytosis
Langerhans cell histiocytosis (LCH) is a rare, clinically
polymorphous group of disorders all having in common
proliferation of Langerhans cells. The recognized clinical
variants of LCH include Letterer-Siwe disease, Hand-
Schuller-Christian disease, eosinophilic granuloma, and
congenital self-healing reticulohistiocytosis1. We report a
50-year-old man with brown lichenoid patches, unusual
cutaneous presentation of LCH, on left dorsal foot.
A 50-year-old man attended Department of Dermatology
with a 2 year duration of brown-colored patches on the
left dorsal foot. Lobular consolidation was shown on chest
X-ray in health screening 5 years ago, but he had no
symptoms. He was diagnosed pulmonary LCH by chest
CT and percutaneous transthoracic needle aspiration. He
is a heavy smoker and has 45 pack-years smoking history.
On physical examination, there were three brown
lichenoid patches on left dorsal foot (Fig. 1). Any regional
lymph node is not palpable. His laboratory findings were
all within normal range. Initial chest CT showed lobular
consolidation at left basal segment of left lower lobe (Fig.
2). Regular enhanced chest CT in every 3 months revealed
no interval change in pulmonary LCH lesion. A biopsy
specimen of cutaneous lesion showed dense infiltrations
in the dermis and dermoepidermal junction. The infil-
trations consist of numerous histiocytes and few lymp-
hocytes. Histiocytes appear as large, round cells with
abundant cytoplasm and indented eccentric nucleus (Fig.
H Kwon, et al
Fig. 3. (A) Dense epidermotropic infiltration of histiocytes (H&E, ×100). (B) Many histiocytes consist of indented eccentric nucleus
and abundant cytoplasm (H&E, ×400). (C) The histiocytes stain with the CD 1a complex (×200). (D) The histiocytes stain with
the S-100 protein (×100).
Fig. 2. Lobular consolidation at left basal segment of left lower
lobe in chest CT.
3A, B). Immunochemistry for CD 1a complex and S-100
protein showed positive staining but CD 68 show negative
(Fig. 3C, D). The patient did not receive treatment but he
visited our Department of Pulmonary Medicine regularly.
To his last visit (1 year later) we can't find any changes on
pulmonary and cutaneous lesions.
LCH encompasses a group of disorders of unknown origin
with widely diverse clinical presentations and outcomes,
characterized by infiltration of the involved tissues by
large numbers of LC. LCH is characterized clinically by
various combinations of systemic and cutaneous manife-
stations. LCH consists of Letterer-Siwe disease, Hand-
Schuller- Christian disease, eosinophilic granuloma, and
congenital self-healing reticulohistiocytosis1.
The definite diagnosis of LCH should fit light microscopy
Langerhans Cell Histiocytosis Presenting as Brown Lichenoid Patches
Vol. 21, No. 3, 2009
Table 1. Cases of adult LCH in Korean literature
Year AuthorsAge/Sex Skin findings
Sang et al.4
Jung et al.5
Chang et al.6
Kim et al.7
Lee et al.8
Table 2. Unusual cutaneous manifestations of LCH
Year AuthorsSkin findings
Modi et al.12
Johno et al.13
Mejia et al.14
Aoki et al.15
Jain et al.16
Lee et al.17
Holme et al.18
Gonul et al.19
Tender swelling of the nail folds
histology and at least 1 of the 2 following factors: 1)
Birbeck’s granules by electron microscopy, 2) labeling of
CD1 antigen on pathological cells2. On light microscopy,
the typical LCH cells are approximately four to five times
larger than lymphocytes and have a vesiculated, reniform
(kidney-shaped) nucleus and abundant, slightly eosino-
philic cytoplasm. Sometimes, indeterminate cell histio-
cytosis (ICH) closely resembles LCH clinically and his-
tologically. The cells in ICH are positive for S-100 protein,
but show variable reactivity for CD1a. Macrophage mar-
kers, such as KP1 (CD68) and Ki-M1p, are often positive.
No Birbeck granules are seen ultrastructurally3. In this
case, we confirmed LCH by his past history, many LC
infiltration in H&E stain and immunochemical positivity
for S-100 protein and CD1a and negativity for macro-
Five cases of adult cutaneous LCH were noted in Korean
Dermatologic literature (Table 1)4-8. Although LCH is com-
monly thought to be confined to children, a small per-
centage of patients are elderly and may have cutaneous
involvement for many years before the onset of visceral
disease9. Adult LCH has difference with childhood LCH in
organ involvement. Bone disease is most common in
childhood LCH, but pulmonary disease is very frequent in
adult LCH. Skin disease and diabetes insipidus are fre-
quent in both type10.
Cutaneous manifestations are common in multi-organ
involved LCH and adult LCH skin lesions are similar that
of childhood. The typical lesion is yellowish or reddish
small multiple papules with ulceration, crusting or a he-
morrhagic change on the trunk and scalp. Especially the
scalp lesions resemble seborrheic dermatitis and other
common finding is an eczematous lesion on intertriginous
On the other hand, unusual cutaneous manifestations of
LCH such as varicelliform eruption, solitary nodule and
nail fold swelling are also reported (Table 2)12-19. In this
case, cutaneous lesions are shown as brown lichenoid
patches on left dorsal foot and we consider this is rare in
Surgery, steroid cream, topical nitrogen mustard, in-
terferon IL, PUVA, isotretinoin, thalidomide can be treat-
ment for skin manifestations of LCH. Single system-pa-
tients should receive prednisone and vinblastine for 6 wk
followed by continuation treatment with 6-mercapto-
purine, prednisone, and vinblastine for 6 months. Patients
with multisystem disease should receive prednisone and
vinblastine for 6 wk followed by continuation treatment
with 6-mercaptopurine, prednisone, and vinblastine for 6
or 12 months, respectively20.
LCH presents a very wide clinical spectrum and variable
course. Prognostic factors are including age of patients at
diagnosis, numbers of organ involved and organ dys-
function. Single system disease is usually associated with a
good prognosis, but multisystem disease may be fatal11.
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