Scleredema diabeticorum in a patient: un uncommon etiology of restrictive lung pattern Escleredema diabeticorum en un paciente: una etiología infrecuente de patrón restrictivo pulmonar.

Scleredema adultorum of Buschke is a rare skin disease characterized by skin thickening and tightening typically at the neck and the upper part of the body. This thickening results from increased mucin deposition in the reticular dermis. Three variants are recognized. Scleredema diabeticorum is one subtype associated with diabetes mellitus. We report a man with a history of poorly controlled diabetes presenting with extensive scleredema adultorum of the trunk in a "cuirasse" pattern associated with restrictive lung disease. Cutaneous ultrasonography revealed a marked thickening of the dermis as well as various echogenic spots in the dermis. Cutaneous hardness secondary to scleredema may cause limited mobility. Therefore, respiratory manifestations may be associated and assessed, mainly in cases of extensive scleredema involving trunk and shoulders. Moreover, cutaneous ultrasonography may be useful to monitor the evolution of scleredema and confirm the diagnosis.

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

La afectación cutánea en las formas agudas de histiocitosis de células de Langerhans (HCL) es en forma de pápulas eritematosas, aunque se han descrito formas raras de lesiones xantomatosas. Presentamos el caso de un niño con HCL aguda diseminada, que comenzó a los 16 meses de edad con brotes de lesiones cutáneas tipo dermatitis seborreica y disfunción hepática progresiva. El cuadro se complicó con diabetes insípida central parcial e infiltración específica pulmonar por células de Langerhans, que desembocó en fibrosis. En su evolución, el paciente desarrolló cirrosis biliar, alteración del perfil lipídico y lesiones cutáneas xantomatosas diseminadas, concomitantes con aquéllas específicas de su HCL. A pesar de sucesivos ciclos de quimioterapia, el curso fue ominoso, y el paciente falleció al cabo de 5 años a causa de su enfermedad hepática. Las lesiones xantomatosas en los cuadros de HCL son típicas de fases tardías de formas crónicas progresivas, como la enfermedad de Hand-Schüller-Christian. Cuando aparecen en las formas agudas diseminadas existe controversia sobre si corresponden a una progresión de la enfermedad hacia formas más crónicas o si se trata de lesiones independientes asociadas, como en el presente caso

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