Self-healing Langerhans Cell Histiocytosis with single organ system involvement of the skin associated with positive double-strand DNA and Perinuclear Anti-Neutrophil Cytoplasmic Antibodies

Abstract We present a unique case of a 6-week-old male, with heterogeneous skin lesions. The internal organ involvement was excluded by normal blood cell count, urinary examination, liver and renal functions, chest and skull radiographs and abdominal and transfontanelar ultrasound examinations. The histopathological examination and laboratory tests revealed typical findings of Langerhans cell histiocytosis and a novel association with positive antibodies related to autoimmunity: positive double-strand DNA antibodies and perinuclear anti-neutrophil cytoplasmic antibodies. Our patient's lesions are in continuous regression during the last months but a much-needed close follow-up continues. Despite significant clinical improvement, positive antibodies related to autoimmunity persist.