Published date: Sep 24 2018

Journal Title: Sudan Journal of Medical Sciences

Issue title: Sudan JMS: Volume 13 (2018), Issue No. 3

Pages: 207–218

DOI: 10.18502/sjms.v13i3.2958

Suad H HDr.suadhassan4@gmail.com

Mona Mohamed Elamin

Gad Allah Modawe

Khalid AbdElmohsin Awad Elseed

Background: Letterer–Siwe Disease (LSD) is one of the variants of Langerhans cell histiocytosis (LCH), which is considered as a rare disease that affects many systems in the body; it is characterized by monoclonal migration and proliferation of specific dendritic cells. The disease affects the bones and skin primarily, but can involve
other organs as well, or appear as a multi-system disease leading to different clinical manifestations and eventually death.

Summary: The authors present a case report of LSD in a two-year-old child from western Sudan, Messeria tribe, who is presented with one and a half-month history of fever, cutaneous ulcers, purprae,
scaly crusted scalp, and pallor. His full blood count showed very low Hb with marked reduction of platelets. TWBC was normal. US showed hepatosplenomegaly with lymphadenopathy. A suspicion of sickle cell anemia and leukemia was suggested. He received treatment in his area in the form of antibiotics, skin care, blood transfusion and platelets aggregate without improvement. Patient was referred to Khartoum for further investigations and management. On presentation, a diagnosis of histiocytosis x was suggested depending on the clinical presentation of a general ill health in a child with purpurae, skin ulcers, and a scaly crusted scalp. A skin biopsy, bone marrow aspirate, and a skull x-ray were requested. Bone marrow aspiration showed hyper cellular BM with marked hemophagocytosis. Patient was admitted in a pediatric ward for further general investigations and blood transfusion, but he passed few days later before starting chemotherapy. Usually this is the prognosis of this rare and fatal aggressive form of histiocytosis x.

Conclusion: A sick child with fever, anemia, hepatosplenomegaly, scaly scalp, and skin lesions should be investigated for LSD.

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