ABSTRACT
CASO CLÍNICO: Paciente de 10 años de edad, con inflamación palpebral superior derecha, sin malestar general ni fiebre, de 3 semanas de evolución y sin respuesta a tratamiento antibiótico oral. La tomografía computarizada orbitaria reveló una lesión de tejidos blandos en el margen superior de la órbita extraconal con destrucción ósea del techo orbitario. El estudio anatomopatológico fue compatible con histiocitosis de células de Langerhans. Tras descartar afectación sistémica se realizó curetaje de la lesión orbitaria e infiltración de corticoides intraoperatoriamente con buena respuesta. DISCUSIÓN: La afectación orbitaria de la histiocitosis de células de Langerhans, a pesar de su baja incidencia, debe tenerse en cuenta en el diagnóstico diferencial de un cuadro inflamatorio orbitario. Suele presentarse como una lesión osteolítica y se confirma con examen histológico y técnicas de inmunohistoquímicas positivas para CD1a y S100. Se recomienda un enfoque interdisciplinario para descartar afectación sistémica y desarrollar una estrategia de tratamiento apropiada
CLINICAL CASE: A 10-year-old girl was seen with a 3-week history of right upper lid swelling and with no other symptoms or fever. There was no recent history of sinusitis, trauma, or previous infection involving the periorbital area, or response to oral antibiotic treatment. Orbital computed tomography showed a lesion involving the upper margin of the orbit, and bone destruction at the orbital roof. Biopsy performed revealed the presence of Langerhans cell Histiocytosis. The lesion was surgically debulked and corticosteroids were used intra-operatively. The lesion responded to treatment. DISCUSSION: The orbital involvement of Langerhans cell histiocytosis, despite its low incidence, should be considered in the examination of acute peri-orbital swelling. It usually presents as an osteolytic lesion, and it is confirmed with a histological examination and immunohistochemical techniques for CD1a and S100. An interdisciplinary approach is recommended to rule out multifocal or multisystemic diseases, as well as to develop an appropriate treatment strategy
Subject(s)
Humans , Female , Child , Histiocytosis, Langerhans-Cell/diagnosis , Histiocytosis, Langerhans-Cell/pathology , Orbital Cellulitis/diagnostic imaging , Orbital Cellulitis/drug therapy , Inflammation/diagnosis , Adrenal Cortex Hormones/therapeutic use , Immunohistochemistry , Blepharoptosis/diagnosis , Triamcinolone/therapeutic useABSTRACT
CLINICAL CASE: A 10-year-old girl was seen with a 3-week history of right upper lid swelling and with no other symptoms or fever. There was no recent history of sinusitis, trauma, or previous infection involving the periorbital area, or response to oral antibiotic treatment. Orbital computed tomography showed a lesion involving the upper margin of the orbit, and bone destruction at the orbital roof. Biopsy performed revealed the presence of Langerhans cell Histiocytosis. The lesion was surgically debulked and corticosteroids were used intra-operatively. The lesion responded to treatment. DISCUSSION: The orbital involvement of Langerhans cell histiocytosis, despite its low incidence, should be considered in the examination of acute peri-orbital swelling. It usually presents as an osteolytic lesion, and it is confirmed with a histological examination and immunohistochemical techniques for CD1a and S100. An interdisciplinary approach is recommended to rule out multifocal or multisystemic diseases, as well as to develop an appropriate treatment strategy.
