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SHORT COMMUNICATION
Langerhans Cell Histiocytosis with Lymph Node Involvement Presenting as Erythroderma
Huiqin ZHU 1 , Yannan MA 2 , Ling SUN 1 , Rongxin ZHANG 1 , Li LV 3 and Aoxue WANG 1 *
Departments of 1 Dermatology and Venereology and 3 Pathology, The Second Hospital of Dalian Medical University, Dalian 116027, and
2
Department of Dermatology and Venereology, Liaocheng People’s Hospital, Liaocheng, China. E-mail: [email protected]
Accepted Sep 4, 2018; Epub ahead of print Sep 5, 2018
Langerhans cell histiocytosis (LCH) represents a group of
rare and clinically heterogeneous conditions of unknown
etiology (1). Although most LCH cases appear in pediatric
patients, the disease does occur de novo in adults, with
an estimated incidence of 1–2 per million. Most adult
LCH cases involve only a single organ system, such as
the bones or lungs. Diagnosis of LCH is generally de-
pendent on biopsy and is made based on the observation
of inflammatory cell infiltration with large numbers of
histiocytic cells and Langerhans cells staining positive
for S100 and CD1a (1). Here we report an unusual case
of LCH involving the lymph nodes that presented with
the clinical features of erythroderma.
CASE REPORT
A 75-year-old Chinese man had a 2-month history of pruritic
papular eruption on his trunk and limbs that began following an
exposure to rain. The rash had rapidly progressed to generalized
exfoliative dermatitis in a 2-week period. The patient had expe-
rienced slight weight loss and fatigue for the previous one week but
denied other systemic complaints including fever, expectoration,
diarrhea, bone pain, and jaundice. Physical examination revealed
enlarged bilateral axillary and inguinal lymph nodes exceeding 1
cm in diameter. Skin examinations revealed generalized uniform
erythematous macules and diffuse scaling on the whole body (Fig.
1A, Fig. S1A 1 ). Blood tests revealed leukocytosis (14.61×10 9
cells/l) with a predominance of eosinophils (21.71%), anemia with
low hemoglobin (119 g/l), and increases in the erythrocyte sedi-
mentation rate, C-reactive protein and IgE (556 IU/ml) above the
normal ranges. Chest X-rays and computed tomography (CT) scans
showed mild interstitial changes. No Sézary cells were found in
the peripheral blood. Routine studies included bacterial and fungal
cultures from the skin and measurement of serum electrolyte,
creatinine, and transaminase levels. Skull and abdominal CT scans
showed normal results, and no bone alterations were observed.
https://www.medicaljournals.se/acta/content/abstract/10.2340/00015555-3030
1
A skin biopsy of the affected area indicated hyperkeratosis,
parakeratosis, acanthosis, and intercellular edema, with capillary
dilation in the upper dermis and inflammatory infiltrate consisting
of lymphocytes and eosinophils, collectively suggesting a case of
superficial dermatitis (Fig. S1B, C 1 ). Histological examination of
the inguinal node revealed destruction of the lymph node struc-
ture as well as tumor cell infiltration. Large cells with grooved
nuclei containing compact chromatin along with eosinophils and
plasma cells also were observed (Fig. 1B, Fig. S1D 1 ). These cells
stained positively for S-100 protein and CD1a upon immunoche-
mical analysis, and thus, a definitive diagnosis of LCH could be
made (Fig. 1C, D).
Upon diagnosis of LCH, the patient was initially treated with
oral methylprednisolone (40 mg/day) and application of a topical
emollient to the affected areas daily. The sizes of the lymph nodes
in the axillary and inguinal regions were significantly reduced
after 2 weeks of these initial treatments, and the skin eruptions
had improved. Improvements in the laboratory results also were
noted. After 2 weeks of treatment, the methylprednisolone dose
was tapered by 5 mg every 2 weeks to a maintenance dose of 5
mg/day. LCH in this patient responded completely to treatment,
and thus, methylprednisolone use was discontinued after 3 months.
No recurrence was observed during the 2-year follow-up.
DISCUSSION
Erythroderma, also known as exfoliative dermatitis, is an
inflammatory reaction of the skin that is often secondary
to a variety of causes (2). The most common causative
factors are previous dermatoses, such as psoriasis and
atopic dermatitis, followed by drug reaction, malignancy,
or idiopathic causes. As this condition has received more
attention, more unusual underlying dermatoses for erythro
derma have been reported. To our knowledge, our case pre-
sented is the third documented adult case of LCH involving
the lymph nodes first manifesting as erythroderma (2, 3).
In general, drug therapy for erythroderma consists of to-
pical and/or systemic medications based on the underlying
etiologies (2). The severe generalized skin inflammation
Fig. 1. Langerhans cell histocytosis. (A) Generalized uniform erythematous macules and diffuse scaling affecting the patient. (B) Higher magnification
images of lymph node showing large cells with grooved nuclei (arrows) and eosinophils. (H&E ×400). (C, D) Immunohistochemical staining revealed that
the tumor cells were positive for CD1a (×200) and S-100 protein (×200).
This is an open access article under the CC BY-NC license. www.medicaljournals.se/acta
Journal Compilation © 2019 Acta Dermato-Venereologica.
doi: 10.2340/00015555-3030
Acta Derm Venereol 2019; 99: 99–100