Langerhans Cell Histiocytosis in a Three-Year-Old Girl in Bosnia and Herzegovina

Abstract

Objective. To present a rare disease, and to point out that clinical manifestations treated for a long period of time without an adequate response to therapy may be a manifestation of a rare disease.

Case report. We present the case of a 3-year-old girl who had been drinking a large amount of water for the previous ten days with frequent urination, and who experienced the worsening of symptoms of scalp dermatitis that had been treated for a year without success. Physical examination revealed a maculopapular rash on the scalp, neck and both ear shells, and exophthalmos of the right eye with periorbital edema. Magnetic resonance imaging of the orbits showed extensive lesions of the skull bones. Further diagnostic evaluation revealed similar lesions in other bone structures. Biopsy of the affected region, microscopic and immunohistochemical analysis led to diagnosis of Langerhans cell histiocytosis.

Conclusion. Langerhans cell histiocytosis mostly occurs in the first three years of life. The incidence is 4-5 patients per million children under 15 years of life. The clinical presentation is highly variable, and can range from isolated, self-healing skin and bone lesions to life-threatening multisystem diseases. Due to the diverse clinical picture, that is often unrecognized, these patients are often referred to other specialists, resulting in the treatment of individual symptoms rather than the underlying disease.