Langerhans Cell Histiocytosis: A Case Report

Widya Nur Febriani, ...

1
Department of Dermatology and Venereology, Medical Faculty of Diponegoro University, Semarang,
Indonesia
2
Dr. Kariadi General Hospital, Semarang, Indonesia

ABSTRACT

Langerhans cell histiocytosis (LCH) is a heterogenous disease, characterized by accumulation of

dendritic cells with features similar to epidermal Langerhans cells. Clinical presentation of LCH
vary from skeletal, liver, to skin involvement. Cutaneous involvement is observed in 40% of cases
and typically reflects multisystem disease with a 20% mortality. LCH is a rare disease and when
the disease involves the skin, LCH most commonly presents as a seborrheic or eczematous
eruption on the scalp and trunk. Clinical manifestations of LCH may vary from spontaneous
resolution to life-threatening multisystem disease.
A 2-year-old boy was referred to the Kariadi General Hospital for an extensive cutaneous eruption
in the form of erythematous macules and crusts throughout the face, scalp, and trunk. Jaundice and
ascites, were reported. Laboratory testing revealed elevated transaminase enzymes,
hyperbilirubinemia. Abdominal USG showed hepatosplenomegaly. Biopsy showed
epidermotrophism with histiocytes over the superficial dermis. Immunohistochemical staining of
CD68 showed positive result which support diagnose of LCH. The patient had chemotherapy
program and supportive treatment. Patient was consulted to dermatology department and was
treated with topical fusidic acid cream/12h. mometasone furuoat cream/12h, urea cream/12h,
ketoconazole shampoo/24h and zinc syrup 10mg/24h. However, the patient deceased during the
treatment.
The main aim of LCH treatment is to resolve the signs and symptoms of the disease and to prevent
the permanent consequences. LCH as multi-system needs more severe treatments like
chemotherapy and various treatments such as observation, surgical curettage, radiation therapy,
steroid injections, or combined therapy. Three main negative prognostic indicators are: early-
onset, extensive disease, and the presence of organ failure. This patient had poor prognosis and
multiple organ failure is the cause of death

Keyword: histiocytosis, Langerhans cell histiocytosis, multi system histiocytosis,