Respuesta de los autores: Penfigoide y demencia / Author's reply: Pemphigoid and dementias

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Asociación entre demencia y penfigoide ampolloso como paradigma de una atención multidimensional e interdisciplinaria / Association between dementia and pemphigoid bullous as a paradigm of a multidimensional and interdisciplinary attention

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[Xanthomas in a patient with Langerhans cell histiocytosis and liver cirrhosis]. / Xantomas en paciente con histiocitosis de células de Langerhans y cirrosis biliar.

Skin involvement in acute forms of Langerhans cell histiocytosis (LCH) is in the form of erythematous papules, although rare forms of xanthomatous lesions have been described. We present the case of a boy with acute disseminated LCH who, at the age of 16 months, began to experience outbreaks of seborrheic dermatitis-like skin lesions and progressive hepatic dysfunction. The symptoms were complicated by partial central diabetes insipidus and specific pulmonary infiltration by Langerhans cells, which led to fibrosis. During the course of the disease, the patient developed liver cirrhosis, alterations in the lipid profile and disseminated xanthomatous skin lesions, concomitant with the lesions specific to the LCH. Despite successive cycles of chemotherapy, the outcome was the death of the patient after five years, due to his liver disease. Xanthomatous lesions in LCH are typical of the late stages of chronic progressive forms, such as Hand-Schüller-Christian disease. When they appear in acute disseminated forms, there is some controversy over whether they correspond to a progression of the disease towards more chronic forms, or whether they are associated independent lesions, such as in this case.