Clinical Study on Acrodermatitis Enteropathica / 대한피부과학회지

ABSTRACT

Clinical studies on five patients with acrodermatitis enteropathica visited during the period from March 1968 to September 1970 to the department of dermatology, Pusan national University hospital were made and the results obtained were summerized as follows; in addition, the literature was reviewed. 1) All of 5 patients aging from 3 months to 3 years, showed characteristic distributions of the cutaneous lesions which ranged in character from vesicobullous to heavily scaled psoriasiform and moniliasis-like lesions. 2) Of these, the nearly full clinical pictures were presented in two cases with the gradual onset in early infancy, dermatitis predominantly involving the periorficial areas and extremities, which followed by recurrent attacks of greenish yellow-colored diarrhea, partial and diffuse loss of the scalp hairs, stomatitis and monilial infections, whereas the rest thtee cases seemed to be "forme fruste" of this disease with the absence of hair and nail abnormalities. 3) There found no speeific histologic findings in two biopsies performed but showed somewhat the pictures of sub-acute dermatoses. 4) Candida albicans were demonstrated from the skin lesions in two cases, 5) Treatment with local application of gentian violet solution, nystatin ointment for the skin lesions and stomatitis were temporarily favorable in all cases and in case 1 and 5, diodoquin, each 300mg and 600mg were administered orally in divided doses for two weeks, with the result of satisfactory responses. However, the latter died, on the 3rd hospitalized day, of acute glomerulonephritis and upper respiratory infection. 6) Though it is generally accepted that there is definite familial occurrence in this disorder but we recognized no evidence of familial incidence in our five cases.