Langerhans Cell Histiocytosis with Hemophagocytic Syndrome in a 1 year and 8 month old female: a case report

Authors: Jenlan S. Que, MD, Jasmin Jamora, MD, FPDS,

Abstract

Introduction: Langerhans cell histiocytosis(LCH) is a rare clonal disorder of pathologic dendritic cells commonly seen in children ages 1-3 years old. It may affect nearly every organ system, the skin being the second most frequently involved. Etiopathogenesis has been long debated but recent findings favor the neoplastic nature of this condition. Clinical manifestations are diverse and severity ranging from self-limiting to fatal systemic disease. Hemophagocytic lymphohistiocytosis(HLH) has been reported to develop in multisystemic LCH which is associated with poorer prognosis. Although typical lesions are papules, cutaneous manifestations are of various forms that may mimic other skin condition such as seborrheic dermatitis, pyoderma and intertrigo among others. Consequently, diagnosis is usually delayed. The common histologic element of the different lesions is the “LCH cell.” Definitive diagnosis is through histopathologic identification of this cell. Multisystemic disease are treated with chemotherapy.

Case Presentation: Our case is a 1 year and 8 month old, Filipino, female, initially presenting with yellowish scaly plaques on the scalp 1 year prior to admission. She was managed as seborrheic dermatitis with no improvement. These were noted to develop into pyoderma-like lesions which was unresponsive to antibiotics. Symptoms were noted to progress with bleeding as well as purpuric macules and papules on the trunk and back which was later accompanied by fever, pallor, cough, malaise and anorexia. Patient was also noted with anemia and thrombocytopenia. On admission, physical examination showed pale palpebral conjunctiva, peri-orbital edema, crackles on bilateral upper lung fields and multiple well defined reddish brown to purpuric macules, papules and plaques some with yellowish to hemorrhagic crusts. Dengue hemorrhagic fever and pneumonia were considered by main service. Patient was then referred to dermatology department.

Skeletal survey revealed an osteolytic lesion at the occipital region. Abdominal ultrasound showed hepatosplenomegaly. Bone marrow aspiration with findings of increase in macrophages/histiocytes with evidence of hemophagocytosis. Skin punch biopsy confirmed the diagnosis of Langerhans cell histiocytosis.

Conclusion: Langerhans cell histiocytosis is a rare disorder with clinical manifestation of various forms and severity. Though some cases may be self-limiting, systemic LCH may be fatal especially when HLH is present. Diagnosis is usually delayed which may lead to poorer prognosis. When the skin is involved, skin punch biopsy aids in its diagnosis.

 

Citation

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Keywords

langerhans cell histiocytosis, histiocytosis X, histiocytosis, hemophagocytic lymphohistiocytosis, hemophagocytic syndrome

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