Acrodermatitis enteropathica-like eruption in an infant with nonketotic hyperglycinemia.

Acrodermatitis enteropathica is a rare inherited disorder characterized by zinc deficiency and a triad of dermatitis, diarrhea, and alopecia. It is an autosomal recessive condition thought to be due to the inability to absorb zinc from the gastrointestinal tract. Acquired zinc deficiency due to a variety of etiologies may produce a similar clinical picture. These causes include inadequate supply, malabsorption, and low zinc stores. In addition to zinc, deficiencies of other nutrients such as branched chain amino acids have induced an acrodermatitis enteropathica-like eruption. We describe a case of a 26-month-old boy with a rare inborn error of metabolism known as nonketotic hyperglycinemia who developed an acrodermatitis enteropathica-like eruption. In addition to zinc deficiency, the patient was deficient in branched chain amino acids due to a low protein diet instituted to reduce his elevated glycine levels. The rash did not respond to zinc replacement alone, and therefore is most likely a combination of amino acid and zinc deficiency. Acrodermatitis enteropathica-like eruptions have been described in other conditions that cause decreased serum amino acids, such as maple syrup urine disease and organic acidurias. This is the first case describing an association between acrodermatitis enteropathica and nonketotic hyperglycinemia.

Dermatologic correlates of selected metabolic events.

Various metabolic events may lead to dermatologic pathology. Three illustrative examples are glucagonoma syndrome, uremic pruritus, and zinc deficiency. The glucagonoma syndrome, resulting from a glucagon secreting-tumor, is characterized by a distinctive dermatitis, necrolytic migratory erythema. This skin rash is a generalized, pruritic eruption which first appears as erythematous patches, then progresses to form superficial vesicles and bullae. Uremic pruritus is a clinical phenomenon seen in patients with chronic renal failure; it provokes vigorous scratching and may lead to numerous cutaneous lesions including extensive excoriations, lichen simplex chronicus, prurigo nodularis, keratotic papules, or secondary impetigo. Zinc deficiency may be evident as an inherited disease called acrodermatitis enteropathica, which presents clinically with a predominately acral and periorificial rash of sharply demarcated erythematous, exfoliative, and exudative patches. It may also result from an acquired defect with similar clinical findings. A brief review concerning the etiology, clinical manifestations, diagnosis, and treatment are given for glucagonoma syndrome, uremic pruritus, and zinc deficiency. Any pathophysiologic dysfunction that results in a loss of metabolic control of homeostasis in the body may demonstrate cutaneous manifestations. These skin findings may be of great clinical significance and may aid in the diagnosis or even be the first sign of a disease process. The glucagonoma syndrome, uremic pruritus, and zinc deficiency are three examples of dermatologic correlates of metabolic events.

[Some clinical symptoms and allergens on asthma-prurigo syndrome]. / Niektóre objawy kliniczne oraz alergeny w zespole asthma-prurigo.

The group of 146 patients suffering from asthma-prurigo syndrome (85 adults and 64 children) have been inquired in many various clinical centers. It was established that in 79.6% of the patients the first symptoms of illness appeared already in infancy and only 28.2% of the patients had negative familiar anamnesis on the allergy. In 73.2% of the patients with asthma-prurigo syndrome the symptoms of atopic dermatitis persisted longer than asthma symptoms and in 89.9% of them asthma-prurigo symptoms accompanied other form of allergic diseases. The most important causal allergens provoking asthma-prurigo symptoms were: house dust (in 64.4% of the patients), chocolate (in 42.2%), cat epithelia (in 40.2%) and cow milk proteins (in 29.5% of the patients).

[Changeability of respiratory tract and skin symptoms in patients with asthma-prurigo syndrome under the influence of emotional and environmental factors]. / Zmiennosc nasilenia objawów ze strony dróg oddechowych i skóry u chorych z zespolem asthma-prurigo pod wplywem czynników emocjonalnych i srodowiskowych.

In a group of 228 patients suffering from asthma-prurigo syndrome the influence of emotional state, permanent residence and season time on dynamic of the clinical symptoms were studied. The evaluation of emotional state in 80 adult patients was carried-out by means of Eysenck Personality Inventory. The emotional state had less negative influence on the exacerbation of the symptoms upon children then in adults (especially on their skin symptoms). It was confirmed that the patients with high level of neurotic symptoms revealed easier the exacerbation of asthma and skin disorders under the influence of emotional stress. The climatic treatment on the sea-side was more efficient for them than any mountain climatic cure, especially in the treatment of airways symptoms. Similarly, the summer season brought the relief of their symptoms from both the respiratory and skin symptoms.

Zinc deficiency in exclusively breast-fed infants.

Zinc deficiency in breast-fed infants is an important disorder. Unlike acrodermatitis enteropathica, it is transient and stops when nursing ends. We report two cases of premature infants, breast-fed only, who showed skin lesions resembling acrodermatitis enteropathica. Results of laboratory investigations revealed a lowered zinc level in the infants' serum and in the mothers' milk and a normal level of zinc in the mothers' serum. After the infants were given zinc supplements and their diets diversified, the skin lesions regressed and their serum zinc levels became normal.

[Skin reactivity in subjects sensitive to different concentrations of nickel, chromium and cobalt]. / Reaktywnosc skóry osób uczulonych na rózne stezenia niklu, chromu i kobaltu.

Patch tests with serial dilutions of nickel sulphate, potassium dichromate and cobalt chloride in petrolatum were performed on 124 nickel-sensitive, 64 chromium-sensitive, and 72 cobalt-sensitive subjects. The lowest eliciting patch test concentrations were as follows: nickel sulphate--0.005, potassium dichromate--0.0025, and cobalt chloride--0.005.

[Recurrent skull neuropathy during the course of diabetes mellitus--case report]. / Nawracajaca neuropatia czaszkowa w przebiegu cukrzycy--opis przypadku.

This study presents a case of a 73 year old woman who has suffered from diabetes mellitus for 8 years and has had 6 isolated cranial nerves palsies within a period of 7 years. The last are-because of which she was treated in Department of Neurology in July 1992--concerned left oculomotor nerve. The coincidence of metabolic and vascular disorders provoke authors to take a wide diagnostics and discussion on diabetic ophthalmoplegia problem.

[Clinical characterization of "asthma-prurigo" syndrome with emphasis on risk factors]. / Charakterystyka kliniczna zespolu "asthma-prurigo" z uwzglednieniem czynników przyczynowych.

In a group of 228 patients with Asthma-Prurigo syndrome in 82.5% of patients the onset of skin lesions was earlier than the onset of asthma. It was found that the early onset of atopic dermatitis suggests the greater probability of subsequent asthma. In 88.6% of patients also other allergic diseases were diagnosed, and rhinitis allergica was observed most frequently. In the investigated group of patients urticaria chronica idiopathica or physical urticarias were not observed. Among 228 patients with Asthma-Prurigo syndrome, 74.6% of patients pointed to house dust as one of the most frequent causative factors. The house dust similarly to other inhalant allergens, somewhat more frequently had influence on the course of atopic dermatitis than on the course of asthma. Contrary to this observation according to patients food allergens (milk, eggs, fish, chocolate) aggravated or induced the relapses of skin lesions much more frequently than asthma. It was shown how often patients noticed adverse reactions due to penicillin and sulphonamides.

[Diagnostic difficulties in the case of 57-year-old woman suffering from encephalitis lethargica]. / Trudnosci rozpoznawcze w przypadku spiaczkowego zapalenia mózgu u 57-letniej kobiety.

The case of encephalitis lethargica in 57 years woman was described. The basal ganglia was already damaged after 7 months from illness onset, and control examination after 15 months showed Parkinsonian syndrome. Serological and CSF viral titres and cultures were negative.

[Nonspecific skin changes as early manifestation of Hodgkin's disease]. / Nieswoiste zmiany skórne jako wczesna manifestacja choroby Hodgkina.

The paper presents a case of Hodgkin disease with unspecific skin changes as the early manifestation of this disease, incomplete clinical picture which with the rapidly deteriorating patient's general condition clearly illustrates the differential diagnostic problems connected with the disease. The problem is also discussed of chemotherapy introduction in severe clinical condition and impossibility of obtaining of a histological proof. Specific and unspecific skin changes which may appear in the course of Hodgkin disease are shortly discussed.