RESUMEN
A sixteen year-old male patient with no history of consanguinity in the family, reported with patchy, thickened lichenified plaques over the whole body. Some areas had normal skin while some were Blaschkoid lesions. The child had delayed milestones along with hypogonadism. Digital contracture with palmoplantar keratoderma was present. Histopathology showed characteristic vacuolar degeneration of the upper epidermis and suprabasilar keratinocytes with hyperkeratosis.
Asunto(s)
Adolescente , Contractura/etiología , Dedos/patología , Humanos , Hiperqueratosis Epidermolítica/clasificación , Hipogonadismo/etiología , Queratodermia Palmoplantar/patología , Masculino , Mosaicismo , Piel/patologíaRESUMEN
Systemic lupus erythematosus is a multisystem organ inflammation due to damage of cells and tissues by pathogenic auto-antibodies and immune complexes. The most important factor for the causation of familial systemic lupus erythematosus is genetic on which environmental factor usually coexists. Two brothers of 7 and 3 years of age having childhood systemic lupus erythematosus are reported here because of low incidence and familial occurrence. Both the children fulfilled the American Rheumatology Association (ARA) criterion of systemic lupus erythematosus though anti-dsDNA was negative in both and antinuclear factor was positive only in the younger child. Systemic lupus erythematosus begins in childhood in 20% of adult patients and usually after the age of 8 years but here onset was even at an earlier age. The treatment of the both the siblings included administration of steroids and cyclophosphamide.