A Case of Acrodermatitis Enteropathica

Abstract

ACRODERMATITIS ENTEROPATHiCA is a rare disease commencing in childhood with cutaneous and gastro-intestinal disturbances. The condition was described and named by Danbolt and Closs (1942). Since that time about 25 to 30 cases have been described. The gastro-intestinal symptoms have been likened to those seen in cceliac disease or other malabsorption states. Dillaha et al. (1953) were the first to describe successful treatment of the disease with diodoquin or 8-hydroxy-5.7 -di-iodo-quinoline.The symptoms, signs, investigations and treatment of a boy of eight years suffering from acrodermatitis enteropathica and believed to be the first case fully reported in Australia will be described. CASE RECORD.R.U., a boy of eight years and two months, was first seen by one of us (R.K.) on September 5th, 1957. His mother offered the story that he had been well and had progressed normally until the age of nine months, when hair fall had been noticed, bald patches appearing in the scalp. Within two weeks all the hair had faHen. At the same time redness appeared on the back of the neck and a rash on the buttocks which was thought to be a napkin rash. He had been breast fed, and about this time was weaned on to cow's milk. The rash on the buttocks persisted and spread to the groins, over the genitalia, and appeared behind the knees. At nine months he had also developed redness and swelling about the left middle finger-nail. All the finger-nails became similarly involved, with loss of the nails between the age of 12 and 18 months. The rash extended about the mouth, eyes and ears, and on the face, and the hands, feet and elbows became involved.Exacerbations and remissions of the rash followed, improvement occurring for periods of two to three months at a time, followed by exacerbation. In an attack he developed soreness of the hands and feet, followed by increased erythema in the affected areas. Blisters would appear, pinhead in size, enlarge and coalesce to form a large blister which would rupture, and crusts with an offensive smell would develop along with raw weeping areas.His mother stated that he had always suffered from looseness of the bowels and on most days the bowels were opened three to four times during the day and once at night. During exacerbations of the disease, the motions were liquid and frequent-up to six or seven during the day and an equal number at night. At these times he frequently complained of abdominal pain, associated with desire to open the bowels. In between he passed motions which were formed, but soft and pale.He did not walk until 23 months, but this had been delayed owing to the severity of the skin lesions. Mental development was normal. He had one sister, aged 10 years, who was weU. Mother and father were healthy and there was no known family history of cutaneous disturbance.

Intestinal malabsorption in childhood.

“…In a previous review of some unusual causes of steatorrhoea, Anderson, Townley, Freeman, and Johansen (1961) stated that in a community of predominantly European origin, the majority of children with persistent steatorrhoea suffered from Temporary malabsorption syndrome common, 3-4 per year in earlier years of study About 2 per year referred as malabsorption syndrome-renal origin unsuspected 1-2 per year referred as malabsorption syndrome-constipation unrecognized 11 cases now seen 6 cases seen 5 cases seen Emotional (maternal rejection) 4 cases seen Malnutrition 3 cases seen Iron-deficiency anaemia Only 3 cases investigated (see also Naiman, Oski, Diamond, Vawter, and Schwachman, 1964) Liver cirrhosis with portal hypertension 3 cases investigated (Astaldi and Strosselli, 1960) Pancreatic achylia with diabetes mellitus 2 cases seen Intestinal lymphangiectasis with protein-losing 2 cases seen (Jarnum and Peterson, 1961) enteropathy Familial dysautonomia 2 cases seen (Burke, 1966) Hypoparathyroidism 2 cases seen (Jackson, 1957;Williams and Wood, 1959) Ganglioneuroma 1 cases seen (Sindhu and Anderson, 1965;Rosenstein and Engelman, 1963) Regional ileitis 1 case seen (Shiner and Drury, 1962) a-D3-lipoproteinaemia 1 case seen (Salt et al, 1960;Anderson et al, 1961;Isselbacher, Scheig, Plotkin, and Caulfield, 1964) Acrodermatitis enteropathica 1 case seen (Kelly and Anderson, 1960;Moynahan, Johnson, and McMinn, 1963) Specific lipase deficiency Not seen (2 cases described by Rey et al, 1966;Sheldon, 1964) Specific bile salt deficiency Not seen (1 case described by Ross, Frazer, French, Gerrard, Sammons, and Smellie, 1955) Whipple's disease Not seen (1 case described by Bruni and Massimo, 1959;no biopsy;Dickinson, Hartog, and Shiner, 1960;adults Hyperparathyroidism Not seen (Davies, Dent, and Willcox, 1956) Agammaglobulinaemia Not seen (Pelkonen et a!., 1963) Neonatal hepatitis and childhood liver cirrhosis (recently recognized to have steatorrhoea) (Burke and Danks, 1966) Shows disorders with proven steatorrhoea referred to Gastroenterological Research Unit, Royal Children's Hospital, …”

Intestinal malabsorption in childhood.

“…In a previous review of some unusual causes of steatorrhoea, Anderson, Townley, Freeman, and Johansen (1961) stated that in a community of predominantly European origin, the majority of children with persistent steatorrhoea suffered from Temporary malabsorption syndrome common, 3-4 per year in earlier years of study About 2 per year referred as malabsorption syndrome-renal origin unsuspected 1-2 per year referred as malabsorption syndrome-constipation unrecognized 11 cases now seen 6 cases seen 5 cases seen Emotional (maternal rejection) 4 cases seen Malnutrition 3 cases seen Iron-deficiency anaemia Only 3 cases investigated (see also Naiman, Oski, Diamond, Vawter, and Schwachman, 1964) Liver cirrhosis with portal hypertension 3 cases investigated (Astaldi and Strosselli, 1960) Pancreatic achylia with diabetes mellitus 2 cases seen Intestinal lymphangiectasis with protein-losing 2 cases seen (Jarnum and Peterson, 1961) enteropathy Familial dysautonomia 2 cases seen (Burke, 1966) Hypoparathyroidism 2 cases seen (Jackson, 1957;Williams and Wood, 1959) Ganglioneuroma 1 cases seen (Sindhu and Anderson, 1965;Rosenstein and Engelman, 1963) Regional ileitis 1 case seen (Shiner and Drury, 1962) a-D3-lipoproteinaemia 1 case seen (Salt et al, 1960;Anderson et al, 1961;Isselbacher, Scheig, Plotkin, and Caulfield, 1964) Acrodermatitis enteropathica 1 case seen (Kelly and Anderson, 1960;Moynahan, Johnson, and McMinn, 1963) Specific lipase deficiency Not seen (2 cases described by Rey et al, 1966;Sheldon, 1964) Specific bile salt deficiency Not seen (1 case described by Ross, Frazer, French, Gerrard, Sammons, and Smellie, 1955) Whipple's disease Not seen (1 case described by Bruni and Massimo, 1959;no biopsy;Dickinson, Hartog, and Shiner, 1960;adults Hyperparathyroidism Not seen (Davies, Dent, and Willcox, 1956) Agammaglobulinaemia Not seen (Pelkonen et a!., 1963) Neonatal hepatitis and childhood liver cirrhosis (recently recognized to have steatorrhoea) (Burke and Danks, 1966) Shows disorders with proven steatorrhoea referred to Gastroenterological Research Unit, Royal Children's Hospital, …”

Intestinal malabsorption in childhood.

“…In a previous review of some unusual causes of steatorrhoea, Anderson, Townley, Freeman, and Johansen (1961) stated that in a community of predominantly European origin, the majority of children with persistent steatorrhoea suffered from Temporary malabsorption syndrome common, 3-4 per year in earlier years of study About 2 per year referred as malabsorption syndrome-renal origin unsuspected 1-2 per year referred as malabsorption syndrome-constipation unrecognized 11 cases now seen 6 cases seen 5 cases seen Emotional (maternal rejection) 4 cases seen Malnutrition 3 cases seen Iron-deficiency anaemia Only 3 cases investigated (see also Naiman, Oski, Diamond, Vawter, and Schwachman, 1964) Liver cirrhosis with portal hypertension 3 cases investigated (Astaldi and Strosselli, 1960) Pancreatic achylia with diabetes mellitus 2 cases seen Intestinal lymphangiectasis with protein-losing 2 cases seen (Jarnum and Peterson, 1961) enteropathy Familial dysautonomia 2 cases seen (Burke, 1966) Hypoparathyroidism 2 cases seen (Jackson, 1957;Williams and Wood, 1959) Ganglioneuroma 1 cases seen (Sindhu and Anderson, 1965;Rosenstein and Engelman, 1963) Regional ileitis 1 case seen (Shiner and Drury, 1962) a-D3-lipoproteinaemia 1 case seen (Salt et al, 1960;Anderson et al, 1961;Isselbacher, Scheig, Plotkin, and Caulfield, 1964) Acrodermatitis enteropathica 1 case seen (Kelly and Anderson, 1960;Moynahan, Johnson, and McMinn, 1963) Specific lipase deficiency Not seen (2 cases described by Rey et al, 1966;Sheldon, 1964) Specific bile salt deficiency Not seen (1 case described by Ross, Frazer, French, Gerrard, Sammons, and Smellie, 1955) Whipple's disease Not seen (1 case described by Bruni and Massimo, 1959;no biopsy;Dickinson, Hartog, and Shiner, 1960;adults Hyperparathyroidism Not seen (Davies, Dent, and Willcox, 1956) Agammaglobulinaemia Not seen (Pelkonen et a!., 1963) Neonatal hepatitis and childhood liver cirrhosis (recently recognized to have steatorrhoea) (Burke and Danks, 1966) Shows disorders with proven steatorrhoea referred to Gastroenterological Research Unit, Royal Children's Hospital, …”

Intestinal malabsorption in childhood.

“…In a previous review of some unusual causes of steatorrhoea, Anderson, Townley, Freeman, and Johansen (1961) stated that in a community of predominantly European origin, the majority of children with persistent steatorrhoea suffered from Temporary malabsorption syndrome common, 3-4 per year in earlier years of study About 2 per year referred as malabsorption syndrome-renal origin unsuspected 1-2 per year referred as malabsorption syndrome-constipation unrecognized 11 cases now seen 6 cases seen 5 cases seen Emotional (maternal rejection) 4 cases seen Malnutrition 3 cases seen Iron-deficiency anaemia Only 3 cases investigated (see also Naiman, Oski, Diamond, Vawter, and Schwachman, 1964) Liver cirrhosis with portal hypertension 3 cases investigated (Astaldi and Strosselli, 1960) Pancreatic achylia with diabetes mellitus 2 cases seen Intestinal lymphangiectasis with protein-losing 2 cases seen (Jarnum and Peterson, 1961) enteropathy Familial dysautonomia 2 cases seen (Burke, 1966) Hypoparathyroidism 2 cases seen (Jackson, 1957;Williams and Wood, 1959) Ganglioneuroma 1 cases seen (Sindhu and Anderson, 1965;Rosenstein and Engelman, 1963) Regional ileitis 1 case seen (Shiner and Drury, 1962) a-D3-lipoproteinaemia 1 case seen (Salt et al, 1960;Anderson et al, 1961;Isselbacher, Scheig, Plotkin, and Caulfield, 1964) Acrodermatitis enteropathica 1 case seen (Kelly and Anderson, 1960;Moynahan, Johnson, and McMinn, 1963) Specific lipase deficiency Not seen (2 cases described by Rey et al, 1966;Sheldon, 1964) Specific bile salt deficiency Not seen (1 case described by Ross, Frazer, French, Gerrard, Sammons, and Smellie, 1955) Whipple's disease Not seen (1 case described by Bruni and Massimo, 1959;no biopsy;Dickinson, Hartog, and Shiner, 1960;adults Hyperparathyroidism Not seen (Davies, Dent, and Willcox, 1956) Agammaglobulinaemia Not seen (Pelkonen et a!., 1963) Neonatal hepatitis and childhood liver cirrhosis (recently recognized to have steatorrhoea) (Burke and Danks, 1966) Shows disorders with proven steatorrhoea referred to Gastroenterological Research Unit, Royal Children's Hospital, …”