| 초록 |
X-linked agammaglobulinemia (XLA) is characterized by markedly reduced number of B lymphocytes, panhypogammaglobulinemia, recurrent bacterial infections in the first few years of life because of genetic defect for Bruton's tyrosine kinase at Xq22 region. Scleredema is a rare autoimmune disease of unknown etiology and characterized by nonpitting induration of skin with occasional erythema. Scelredema may be associated with a history of an antecedent febrile illness, diabetes mellitus, or blood dyscrasia. We present 'a case of scleredema developed on hands, legs and feet in 15-year-boy who had suffer from X-linked agammaglobulinemia (XLA). Histopathologic findings of skin showed normal epidermis with a widening of spaces between collagen bundles by infiltration of acid mucopolysaccharide in dermis. |
