A 9 year old boy with juvenile dermatomyositis developed pulmonary alveolar lipoproteinosis.
Uso de lipoid proteinosis en inglés
1
More than 250 cases of this disorder have been described in the literature, but occurrence of lipoidproteinosis in siblings is very rare.
2
Lipoidproteinosis is a rare autosomal recessive condition affecting the majority of organ systems, but predominantly involving skin and mucous membranes.
3
Lipoidproteinosis is presented by varying degrees of skin scars, beaded papules along the eyelid margins, variable signs of hoarseness of voice and respiratory disorders.