SHORT COMMUNICATION
463
Indeterminate Cell Histiocytosis Presenting with Leonine Facies
Cheng CHEN 1 , Giang Huong NGUYEN 2 and Yue-Ping ZENG 1 *
Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College,
Dongcheng District, Beijing 100730, China, and 2 Department of Dermatology, University of Colorado Anschutz Medical Campus, Aurora, CO,
USA. *E-mail: [email protected]
Accepted Dec 14, 2017, E-pub ahead of print Dec 15, 2017
1
Indeterminate cell histiocytosis (ICH) is a rare disease
characterized by the proliferation of histiocytic cells
derived from indeterminate dermal cells. The clinical
features of ICH include solitary or multiple, asymptoma-
tic, papules and nodules on the trunk and extremities. In
some cases, ICH presents as a generalized eruption. We
report here the case of a patient with ICH who presented
with “leonine facies”.
CASE REPORT
A 31-year-old Chinese man presented with a 4-year history of pro-
gressive, asymptomatic, scattered, skin-coloured and erythematous
papules and nodules. The skin lesions initially appeared on the
left cheek, spreading to his right cheek, trunk, and extremities. He
did not report any preceding illness or associated constitutional
symptoms, such as weight gain/loss, fevers, chills, night sweat,
coughs, or chest pain. He had no personal or family history of skin
rashes. The skin lesions regressed spontaneously over the subse-
quent 42 months without any treatment, leaving atrophic scars.
Two months prior to his initial visit to our clinic, he experienced
a recurrence of his skin lesions.
Dermatological examination revealed skin-coloured and ery
thematous, soft papules and nodules on his face and both auricles
resembling “leonine facies” (Fig. 1A). The lesions ranged from 5
to 40 mm in diameter. There were similar lesions on the patient’s
lower and upper extremities (Fig. 1B), but his back was relatively
spared. A peripheral blood count, urinalysis, serum electrolytes,
antinuclear antigen, rheumatoid factor, and tumour markers in-
cluding carcinoembryonic antigen, and squamous cell carcinoma
were within normal limits. The patient had a modestly elevated
aspartate transaminase, lactate dehydrogenase, triglycerides, and
an erythrocyte sedimentation rate. Abdominal sonography revealed
a fatty liver. A chest X-ray was normal.
A punch biopsy revealed a well-defined Grenz zone with no
evidence of histiocytic epidermotropism. A dense dermal infiltrate
of histiocytes admixed with lymphocytes was present without
eosinophilic infiltration. The histiocytes had abundant eosinophilic
cytoplasm with vesicular nuclei. The nuclei of some histiocytes
appeared coffee-bean or kidney shaped (Fig. 2A). The histiocytic
cells were immunohistochemically reactive for CD1a (Fig. 2B),
CD163, and CD68, and partly positive for S-100 protein; cells
were negative for langerin. Acid-fast and Periodic Acid-Schiff
staining were negative.
The constellation of skin morphology, histology, and immu-
nophenotypic findings were compatible with ICH. Methotrexate
(MTX), 15 mg weekly, was recommended. No improvement was
seen a