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Two groups of 70 newborns each, all from diabetic mothers with and without nystatin prophylaxis, were compared, during the first three weeks of age, for incidence of mucocutaneous Candida albicans contamination (17 or 30 per cent) and for moniliasis (17 per cent in the group without prophylaxis and 71 per cent with Candida albicans contamination). Reference is made to modes of birth for comparison between pathogenesis and incidence of moniliasis and the need for prophylaxis of newborns delivered by diabetic mothers or by women with intact metabolism.

Five children (aged 11 to 19 years) with lifelong chronic mucocutaneous candidiasis had 12 episodes of esophageal and/or laryngeal candidiasis documented by endoscopy. Symptoms included hoarseness (8/12), dysphagia (6/12), and hemoptysis (1/12). There was poor correlation between oral lesions and esophageal or laryngeal involvement. On fiberoptic endoscopy, the esophagus was involved alone in four episodes (33%), the larynx in two episodes (17%), and both structures in six episodes (50%). In six of eight instances, the esophagram was nondiagnostic or markedly underestimated the extent of inflammation. Intravenous amphotericin B or miconazole resulted in the resolution of these infections for variable periods of time. Repeat endoscopy was used to follow the course of the disease. Aerosolized amphotericin B was effective on one occasion in clearing candidal lesions of the larynx and one small area of the left mainstem bronchus. Oral topical therapy was not beneficial. Since the signs and symptoms of laryngitis or esophagitis are often minimal or absent and complications, including strictures, may arise from chronic inflammation, periodic endoscopy and systemic therapy may be necessary.

Twelve patients with chronic mucocutaneous candidiasis were assigned by random allocation to a 6-month course of treatment with ketoconazole or placebo in a double-blind trial. All six recipients of ketoconazole had remission of symptoms and virtually complete regression of mucosal, skin, and nail lesions, whereas only two of the six receiving placebo had even temporary mucosal clearing, and none had improvement of skin or nail disease. The clinical outcome in the ketoconazole-treated group was significantly more favorable (p = 0.001) than in the placebo-treated group. The six patients receiving placebo in the controlled trial were then treated with ketoconazole in an open trial, and all responded favorably. Hepatitis, probably drug induced, developed in one patient after 6 months of treatment but proved to be mild and reversible. Oral ketoconazole is an effective treatment for chronic mucocutaneous candidiasis.

An 8-year-old girl with chronic mucocutaneous candidiasis was treated with Ketoconazole 100 mg daily for 6 weeks. Oral thrush disappeared after a week, while cutaneous lesions cleared within a month. Her nail dystrophy disappeared during the following months. Repeated cultures were negative for Candida albicans. The patient has remained in remission for an 8-month period. Our findings thus support previous reports indicating that Ketoconazole is an effective agent against this disease.

Arabinitol is a pentitol generated in large quantitites by several species of Candida, including Candida albicans. The level of arabinitol in the sera of infected animals and humans was determined by gas-liquid chromatography of an acetone extract of the serum. Experimentally infected mice with pyelonephritis due to C. albicans had elevated levels of arabinitol; rabbits with pyelonephritis did not have elevated levels, nor did rabbits with catheter-induce cystitis, but rabbits with endocarditis developed elevated levels of arabinitol shortly before death. A prospective study in patients clinically suspected of having invasive candidiasis failed to show elevated levels of arabinitol in most. Mice and patients not colonized or infected with yeasts but with renal failure had high serum levels of arabinitol. The data indicate that an elevated level of arabinitol in the serum of a patient without renal disease is suggestive of invasive candidiasis, but normal serum levels do not contradict the diagnosis.

The Acquired Immunodeficiency Syndrome (AIDS) is a recently recognized syndrome caused by a newly described retrovirus, Human T-Cell Lymphotropic Virus-III(HTLV-III). A disease that selectively attacks the immune system, manifested by opportunistic infections and unusual neoplasms, AIDS has continued to be confined primarily to several unique at-risk populations. AIDS has evoked unprecedented interest in the medical community, and care of patients with this nearly universally fatal syndrome presents many unique challenges to the health care team.

Diagnosis, treatment, and possible pathogenesis of candidiasis were studied in 5 species of pinnipeds in captivity: gray seal (Halichoerus grypus), harbor seal (Phoca vitulina), northern fur seal (Callorhinus ursinus), California sea lion (Zalophus californianus), and northern elephant seal (Mirounga angustirostris). The animals were kept outdoors in a freshwater exhibit. Candidiasis was characterized by purulent nasal discharge, inflammation of the lips at the mucocutaneous junction, periocular alopecia, vaginitis, and dermatitis. Administration of ketoconazole at dosages of 5 mg/kg BID and 10 mg/kg SID controlled the disease. Wild gulls were suspected as vectors of Candida albicans.

The course of development of hypocortisolism was studied in 20 patients with autoimmune polyendocrinopathy-candidosis-ectodermal dystrophy (APECED) for 1.3-9.3 years during which time the patients underwent at least three 2-h ACTH tests (2hAT). A slow progression of the disease was evident and could be staged. The earliest indicators of incipient failure were subnormality of the 2-h cortisol level alone or with subnormality of the 2-h increment. The increment was then abolished. A normal basal level was maintained longer. Longer forms of the ACTH tests produced normal responses even after the early stages of failure. A constantly elevated ACTH concentration and low cortisol/ACTH ratio in plasma were likewise signs of advanced hypocortisolism. Current criteria of primary hypocortisolism are thus indicators of the late stages of failure only. The presence of circulating adrenocortical antibodies is predictive of hypocortisolism. Some patients had normal 2hAT responses, but antibodies and subnormal cortisol/ACTH ratios. This may represent a state of compensatory activation of the hypothalamic-pituitary-adrenocortical axis.

Optimal management of the ocular and systemic components of the multiple endocrine deficiency, autoimmune disease, candidiasis syndrome requires early identification of affected individuals. This autosomal recessive syndrome is characterized by hypoparathyroidism, Addison's disease, and chronic mucocutaneous candidiasis among other endocrinopathies and immune disorders. We retrospectively reviewed 16 patients, 14 with the full syndrome and two with the syndrome partially manifested. Four of these patients manifested a self-limited, bilateral keratitis in which the age of onset ranged from 2 to 9 years. Keratitis preceded the onset of any endocrinopathy in two of four patients and was among the first signs of the syndrome. The keratitis was not caused by hypoparathyroidism or candidiasis. The anterior stromal vascularization and scarring resulted in a visual acuity of 20/50 or worse in four of eight affected eyes. We recommend medical management of the corneal disease without surgical intervention.

Idiopathic hypoparathyroidism is a rare disorder produced by parathyroid hormone deficiency of unknown cause. It is often associated with other endocrine abnormalities. Patients with idiopathic hypoparathyroidism frequently develop ectodermal disease, including dry, rough skin: coarse, brittle hair; and lusterless, distally split nails. All of these complaints are relatively common in a dermatologic practice. Chronic mucocutaneous candidiasis is also a manifestation of idiopathic hypoparathyroidism. A patient is presented with a prominent ectodermal dysplasia and a chronic mucocutaneous candidiasis that were due to the underlying idiopathic hypoparathyroidism. A brief review of idiopathic hypoparathyroidism is included, as well as the implications of this diagnosis in terms of differential diagnosis, associated endocrine disorders, and therapy.

Fungal infections of the skin represent a relatively common problem in pediatric dermatology. Although fungal infections of the feet, nails, and groin are uncommon in the pediatric age group, fungal infections of the scalp are very common and must be diagnosed early because they may lead to permanent hair loss if left untreated. Perhaps the most significant change in fungal infections in children has occurred in tinea capitis, including the causative agent and the type of infection this organism may produce; these factors are focused upon in this article. Also discussed are infections caused by the yeast organisms Candida albicans and Pityrosporum orbiculare, as well as the deep mycoses, specifically chromoblastomycosis and cutaneous aspergillosis.