Oculocutaneous albinism.
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Biomedical subjects
Publications and source records attributed to C K Janniger.
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Based on a very large series of childhood scleroderma followed for more than ten years, the authors describe various subsets, which, in several aspects, differ significantly from the adult forms. Of special importance is a higher prevalence of linear scleroderma resulting in deformities and disabilities, as well as impairment of bone development in facial hemiatrophy. The immune abnormalities and vascular involvement leading to fibrosis are presented as the basic events in the pathogenesis. Critical discussion on a possible transitional form between localized and systemic disease and visceral involvement in cutaneous forms is followed by clinically applicable laboratory studies and management, including new therapeutic modalities.
Erythema toxicum neonatorum is a common finding of unknown etiology affecting healthy newborn children, particularly those born at term. It is seen in the first days of life, but rarely at birth. Infants with it often have sterile papulopustules containing a predominance of eosinophils. It is important to distinguish from other cutaneous eruptions of neonates, some of which are serious.
Incontinentia pigmenti is a rare genodermatosis that most commonly involves the skin, eyes, teeth, and central nervous system. We describe it in two generations. Two twin girls were observed at birth with a characteristic whorling pattern of erythema, patch, and plaques, and were later found to have dental anomalies and eye findings consisting of black pigment granules around the macula and optic nerve head. One child also had strabismus, the most common eye change, and an atrial septal defect. A scarring scalp alopecia was evident in both twins and their mother, who also had typical dental findings.
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Seborrheic dermatitis is a common condition that usually appears as simple dandruff. It may affect the scalp, the central part of the face and the anterior portion of the chest, as well as flexural creases of the arms, legs and groin. It occurs most often in infants and in adults between 30 and 60 years of age. Patients with acquired immunodeficiency syndrome may have particularly resistant cases of seborrheic dermatitis. Diagnosis of this condition is generally straightforward, but the differential diagnosis includes a variety of conditions, such as psoriasis vulgaris (sebopsoriasis), atopic dermatitis, tinea capitis and candidiasis, as well as other, more rare conditions. Seborrheic dermatitis may be associated with or caused by a variety of underlying disorders. Treatment is generally topical. Steroid creams, selenium, salicylic acid and coal tar preparations, and pyrithione zinc are frequently used to treat this condition.
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We report two patients with ecchymotic patches that did not suggest the diagnosis of Kaposi's sarcoma. The principal complaint of one patient was facial and periorbital edema with bilateral periorbital ecchymosis. On his trunk were patches resembling pityriasis rosea-like Kaposi's sarcoma. Both types of lesions proved to be Kaposi's sarcoma on histologic examination. The second patient had scattered ecchymotic lesions, with typical lesions of Kaposi's sarcoma elsewhere on his body. The ecchymotic lesions showed a large amount of extravasated red blood cells and no evidence of amyloid. This variant has not been described previously to our knowledge.