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In four medical centers, 40 patients with keratinizing dermatoses were treated with topical tretinoin (vitamin A acid) 0.1% cream and salicylic acid 2% cream in a short-term, double-blind study. Tretinoin was the more effective treatment for several of the keratinizing dermatoses with the exception of palmar-plantar hyperkeratosis, for which it was not effective in the concentration and method of application used. The most striking clinical responses occurred in patients with lamellar ichthyosis and ichthyosis vulgaris. Local adverse reactions-chiefly pruritus, erythema, burning, excoriation, and irritation-were not severe and could be controlled by modification of the treatment regimen.

Systemic tuberculosis with morphologic skin lesions that were essentially identical developed in two infants who were born of tuberculous mothers. In each patient, the infection induced erythematous papules with central crusted dells, noted four and eight weeks after birth, respectively. In each case, the diagnosis of tuberculosis was made by examination of tissue that was obtained at laparotomy. In neither patient was Mycobacterium tuberculosis found in the skin lesions. The conditions of both patients responded well to antituberculous therapy. This article reviews the possible categorization of the skin lesions in these infants.

Yellow nail syndrome is characterized by the triad of abnormally shaped, slow-growing, yellow nails, lymphedema, and chronic respiratory tract disease. Nail changes may precede the other features by several years. The syndrome is most often seen in middle-aged individuals and has not been documented in children under age 11 years. We cared for an 8-year-old girl with this disorder.

Dermal elastic tissue in fetal skin was evaluated by light microscopy using three different staining techniques. Postmortem skin was obtained from the abdominal wall of 45 fetuses ranging in age from 8 to 42 weeks. Elastic fibers were first detected in skin from fetuses of 22 weeks and, with increasing gestational age, appeared to increase in quantity and complexity. Skin from fetuses older than 32 weeks had a well-developed network of elastic fibers throughout the dermis. There was no diminution of dermal elastic tissue during the latter part of the sixth lunar month as had been reported in a previous study.

A 14-year-old girl had acquired progressive kinking of the hair. Light microscopy disclosed flattening of the hair shafts with partial twists at irregular intervals. Cross-sections of paraffin-embedded hairs were elliptic or irregular in configuration. Scanning electron microscopy confirmed the presence of partial twists and demonstrated canalicular grooves that extended for variable lengths along the hair shafts. Acquired progressive kinking of the hair most resembles woolly hair in appearance and structural abnormalities.

We report the successful prenatal diagnosis of ichthyosis in the fetus of a woman whose previous liveborn child was affected with "harlequin ichthyosis". The fetal diagnosis was established through analysis of ultrasonographically guided fetoscopic skin biopsies. These biopsies showed premature hyperkeratosis, most marked around hair follicles and sweat ducts, and forming plugs of hyperkeratotic debris. These observations were in distinct contrast to those in control fetuses, whose epidermis consists of squamous epithelium only a few cells in depth with minimal keratinization.

Acute neutrophilic dermatosis (Sweet syndrome) is a benign self-limited disease in adults. A child with apparent evolution of acute neutrophilic dermatosis to postinflammatory cutis laxa and elastolysis then developed fatal vascular involvement. One other patient with postinflammatory cutis laxa with aortic regurgitation and sudden fatal unrecognized occlusive coronary arterial disease is discussed. If cardiovascular symptoms or signs develop during the course of Sweet syndrome or postinflammatory cutis laxa, a thorough investigation is warranted to rule out potentially fatal coronary arterial disease. Coronary bypass surgery may be the only effective treatment for the severely fibrosed proximal coronary arterial system.

Twenty-two cases of cutis marmorata telangiectatica congenita were evaluated during an 8-year period. All but two patients were examined in the first year of life; 14 of the 22 (64%) were female infants. Four patients had focal cutaneous atrophy associated with the reticulated vascular pattern, and eight had ulcerations of involved skin. Six (27%) had additional anomalies. Of these, three patients had a nevus flammeus, and one had congenital generalized fibromatosis and hemiatrophy. Two of the infants had glaucoma; one also had a facial nevus flammeus and the other had cutis marmorata telangiectatica congenita of the face. A congenital pigmented nevus and a localized venous malformation constituted the remaining associated defects. This disease is an uncommon cutaneous vascular anomaly that is most often solitary but occasionally may be associated with other developmental defects.

Thirty-two patients with histiocytosis X were evaluated and treated at Children's Memorial Hospital, Chicago, during the years 1978 to 1984. Twelve patients (38%) had solitary or multifocal bone lesions, three (9%) had bone lesions and diabetes insipidus, and seventeen (53%) had cutaneous and/or multisystem involvement. Age at diagnosis ranged from 2 days to 15 years. Fifteen patients were 2 years of age or younger at the time of diagnosis. Sixteen patients (50%) had skin infiltrates, of whom seven (43%) had cutaneous lesions documented at birth. Cutaneous lesions included vesicopustules, erythematous papules, nodules, eczematous dermatitis, granulomatous ulcerative lesions, petechiae, and hemorrhagic lesions. Xanthomas and nail dystrophy were not observed. The therapeutic regimen chosen was based on extent of involvement and location of infiltrates. Only two of the thirty-two patients died; both had multisystem disease.

A 65-year-old black female with Sezary syndrome had generalized intractable pruritus, erythroderma, alopecia, onychogryphosis, lumphadenopathy and hepatomegaly. Abnormal lymphocytes with large, convoluted and grooved nuclei (Sezary cells) were identified in the skin and peripheral blood. A striking feature of her disease was severe, deforming arthropathy of the hands and knees, a clinical finding which has been described previously in only one patient with Sezary syndrome. At necropsy no associated lymphoma was found.

Palmoplantar pustules may rarely be associated with chronic recurrent multifocal osteomyelitis in children and young adults. Additional features of this disorder are recurrent low-grade fevers and bone pain. Skeletal radiographs and bone films suggest osteomyelitis, but bone cultures yield no growth of organisms. The cause of this disorder is unknown, and treatment remains unsatisfactory.

Fibroblast cultures from patients with dominant dystrophic epidermolysis bullosa of the albopapuloid variety display deranged glycosaminoglycan metabolism. These cells accumulate increased amounts of sulfated glycosaminoglycans. The mechanism for the greater content of glycosaminoglycans appears to be related to increased synthesis. During the first 6-12 h, intracellular labeled glycosaminoglycans accumulated in the dominant dystrophic epidermolysis bullosa cells at about twice the rate as that of control fibroblasts. In addition, secretion of sulfated glycosaminoglycans was two- to threefold greater than in control cultures. In contrast, both pulse-chase and cross-correction experiments failed to show any evidence for defective degradation of the material. The biochemical trait is genetically specific for albopapuloid dominant dystrophic epidermolysis bullosa, since fibroblasts from patients with other varieties of epidermolysis bullosa did not accumulate increased glycosaminoglycans. The data suggest that in vitro abnormality in glycosaminoglycan metabolism could serve as an important marker for this variety of epidermolysis bullosa and be of genetic and prognostic value in the sporadic patient with epidermolysis bullosa. Although the precise relationship of the defect to the disease has not yet been defined, it is possible that excessive tissue accumulation of glycosaminoglycans may alter collagen fibril deposition, thus, impairing the structural integrity of the skin and leading to posttraumatic blisters and erosions that characterize the disease.

A 10-month-old male infant had keratosis follicularis spinulosa decalvans, an X-linked dominant disorder. His cutaneous abnormalities consisted of generalized hyperkeratosis, spiny follicular papular lesions, universal alopecia, and hypoplastic nails. Ocular changes characteristic of the disease were also present. Unusual findings included deafness, failure to thrive, predisposition to bacterial infections without demonstrable immune defect, and transient hepatomegaly with abnormal liver function studies.

Congenital hypertrichosis universalis is a rare familial disorder felt to be inherited as an autosomal dominant trait. The patient described below was noted to have excessive body hair at birth. The hairiness increased progressively. By two years of age, the patient's face, trunk and limbs were covered with long hair. Only the mucous membranes, palms and soles were spared. The patient's father and paternal grandfather were also noted to be hirsute at birth.

The effect of salicylate on the metabolism of peripheral blood lymphocytes in tissue culture was investigated. Lymphocytes incubated with sodium salicylate at a concentration of 30 mg/100 ml showed increased glucose consumption, lactic acid production, and oxygen consumption, evidence for uncoupling of oxidative phosphorylation. No decrease in cell number or viability (trypan blue dye exclusion) was noted in salicylate-treated cultures. Normal DNA, RNA, and total protein synthesis measured by radioisotope incorporation was depressed in the salicylate-treated cultures. Increased DNA synthesis after the addition of a mitogen (PHA) or antigen (PPD) to the culture was strikingly suppressed by salicylate. The degree of suppression was proportional to the concentration of salicylate used. The effect on RNA and protein synthesis in stimulated lymphocytes was much less pronounced. Acetylsalicylic acid was found to be as active as sodium salicylate in suppressing DNA synthesis, but the p-OH congener (p-OH benzoic acid) did not alter cell respiration, glycolysis, viability, or DNA synthesis. The salicylate effect was reversible as evidenced by return of cellular reactivity upon removal of the drug from the media.