Langerhans Cell Histiocytosis: Clinical Case of a Difficult Diagnosis
Objective of the Paper: To present a clinical case of Langerhans cell histiocytosis (LCH) in a newborn girl and describe specific issues in diagnosing this condition.
Key Points: This paper describes a clinical case of LCH in a newborn girl. She presented with petechial and small macular hemorrhagic rash on the skin of the head, face, torso, and extremities, including the feet. The skin in the central portions of some small maculae had the appearance of ruptured pustules. The initial diagnosis was idiopathic thrombocytopenic purpura. In view, however, of both the failure of the skin lesions to improve with treatment and the uncertainty of the initial diagnosis of idiopathic thrombocytopenic purpura, a multidisciplinary team, including a pediatric cancer surgeon, hematologist, medical geneticist, dermatologist, and allergy specialist, decided in favor of performing a biopsy of the skin. The histology and immunohistochemistry confirmed LCH.
Conclusion: LCH is a rather rare disorder with a wide spectrum of non-specific clinical manifestations, creating difficulties for diagnosis within the first days of hospitalization. Skin biopsy is required to confirm the diagnosis. Differential diagnosis is needed, to exclude other disorders manifested by a slowly evolving hemorrhagic rash.
Contribution: Faizullina, R.M. participated in development of the study design, checked critically important content, approved the final version of the manuscript submitted for publication, selected and examined the patient, and contributed to analysis and interpretation of data. Gafurova, R.R. was responsible for collection of clinical information and for analysis and interpretation of data, and contributed to the final manuscript. Shangareeva, Z.A. was involved in reviewing relevant publications, collection of clinical information, data processing, analysis, and interpretation, and writing. Tikhonova, I.V. contributed to the selection and examination of the patient, as well as data analysis and interpretation. Dunaev, Yu.K. participated in the selection and examination of the patient, and in data analysis and interpretation.
Conflict of interes: The authors declare that they do not have any conflict of interests.
R.M. Faizullina — Bashkir State Medical University, Russian Ministry of Health; 3 Lenin Street, Ufa, Russian Federation 450008. eLIBRARY.RU SPIN: 6706-3639. E-mail: email@example.com
R.R. Gafurova — Bashkir State Medical University, Russian Ministry of Health; 3 Lenin Street, Ufa, Russian Federation 450008. eLIBRARY.RU SPIN: 3334-7890. E-mail: firstname.lastname@example.org
Z.A. Shangareeva — Bashkir State Medical University, Russian Ministry of Health; 3 Lenin Street, Ufa, Russian Federation 450008. eLIBRARY.RU SPIN: 8103-5360. E-mail: email@example.com
I.V. Tikhonova — City Pediatric Clinical Hospital No. 17; 29 Svobody Street, Ufa, Russian Federation 450065. E-mail: firstname.lastname@example.org
Yu.K. Dunaev — City Pediatric Clinical Hospital No. 17; 29 Svobody Street, Ufa, Russian Federation 450065. E-mail: email@example.com
Fig. 1. Multiple petechial and small macular hemorrhagic rash in a newborn girl. Photo: Courtesy of R.M. Faizullina
Complete blood count over time
Blood chemistry over time
Fig. 2. Multiple hemorrhagic rash lesions on the skin of the feet (A) and the palm (B). Photo: Courtesy of R.M. Faizullina
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