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2017, Number 1

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Rev Cub Gen 2017; 11 (1)

Neurocutaneous melanosis associated with humoral immunodeficiency. A case report

Torres RB, Portal MJÁ, Calixto RY, Lantigua CA, Mederos BY, Rodríguez PC, Martínez TG

Language: Spanish
References: 21
Page: 43-47
PDF size: 751.91 Kb.


ABSTRACT

Neurocutaneous melanosis is a rare disease characterized by the presence of giant melanocytic nevi and melanocytic lesions of the central nervous system. Some described cases lack of neurological manifestations, and suggestive images which lesions are only detectable by biopsy of the central nervous system. In this case, a report of a 4-yearold child with a diagnosis of neurocutaneous melanosis and recurrent infectious processes of respiratory system, mainly tonsillitis and rhinitis is described. Immunological studies showed decreased levels of IgA, IgM, IgG2, and CD19 positive cells; as well as normal levels of CD4+ and CD8+ lymphocytes and non-protective levels of anti-diphtheria antibodies. A humoral immunodeficiency associated with this genetic disease was diagnosed. The disturbance of the underlying disease suggest that a dysregulation of the immune system may occurred in thoses cases, as in the case under study, therefore we considered the indication of immunological studies in patients with neurocutaneous melanosis is necessary.


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