The effects of estradiol on the growth patterns of estrogen receptor-positive hypothalamic cell lines.

Although it appears that the perinatal development of sexual phenotype in the rodent brain is determined by exposure to estradiol, generated locally via aromatization of androgen, the mechanisms underlying this process are not fully understood. We have, therefore, developed an in vitro model of hormone action based upon examining the effects of sex steroids on SV-40-transformed fetal rat hypothalamic cell lines. Using serum-free growth factor-deficient conditions the effects of 17 alpha- and 17 beta-estradiol, testosterone, dihydrotestosterone (DHT), and tamoxifen on survival of two estrogen-binding rat hypothalamic cell lines were examined. In one cell line, RCF-8, both 17 beta-estradiol and testosterone increased survival at picomolar concentrations. This effect was blocked by tamoxifen, but could not be reproduced by the nonaromatizable androgen DHT or the inactive isomer 17 alpha-estradiol. In the other cell line, RCA-6, addition of 17 beta-estradiol led to inhibition of cellular proliferation, which was reversed by the addition of tamoxifen. In an estrogen receptor-negative hypothalamic cell line, RCF-12, estradiol had no net effect on the growth pattern. In summary, the estrogen-binding capacity and the responsiveness to physiological concentrations of estradiol and testosterone, but not DHT, make the RCF-8 cell line a potential in vitro model of hypothalamic sexual differentiation. The use of estrogen-sensitive hypothalamic cell lines provides a unique opportunity for studying the cellular mechanisms underlying this process.

Percutaneous absorption of topically applied triamcinolone in children.

The real and potential problems associated with the percutaneous absorption of topically applied corticosteroids have been used as an argument against the prolonged administration of these drugs. Many pediatricians and dermatologists consider children to be more likely to absorb substantial quantities of topically applied corticosteroids than adults. This study examines the percutaneous absorption of 0.1% triamcinolone acetonide ointment applied four times a day for a period of six weeks to patients with severe atopic eczema. The results of 8 AM and 4 PM plasma cortisol and 24-hour urinary cortisol determinations do not indicate any notable adrenal suppression. No patient had an 8 AM cortisol value that would be considered in the abnormal range. These results indicate that the use of a medium-strength topical corticosteroid for this length of time probably poses no noteworthy hazard from percutaneous absorption.

A syndrome of trichoepitheliomas, milia, and cylindromas.

Three consecutive generations of a family have had members affected with trichoepitheliomas and milia. One member, the proband, also had cylindromas. The pattern of inheritance was a dominant one, with only the male members affected. Histopathologically, the trichoepitheliomas and milia contained keratinizing cysts with laminated centers, peripheral basaloid cells, and a thin granular layer. Fibroblastic stroma and fronding of basaloid cells were also seen in both tumors. The trichoepitheliomas and milia differed mainly in the greater proportion of keratinized cells found in the milia. Clinically, the milia were found only in areas of vellus hairs.

Xanthelasmoidea. An unusual case of urticaria pigmentosa.

Xanthelasmoidea is an old term that has been used to describe yellow papules of urticaria pigmentosa. I used it to describe the cutaneous lesions in a young child with 1- to 2-cm oval papules, each of which was studded with 10 to 20 yellow puncta. Biopsy disclosed the puncta to be small nests of mast cells separated by normal dermis.

Toxic epidermal necrolysis. A review of 75 cases in children.

From 1965 to 1973, 75 patients were admitted to the Children's Hospital of Buffalo, with toxic epidermal necrolysis (TEN). A review of their records show an equal ratio of males to females, with 73 whites and only 2 blacks. No patient was septic with staphylococci, and there was only one death. A comparison of treatments indicates that the disease is probably self-limited and not influenced by antibiotics or corticosteroids.

Trichophytin reactions in children with tinea capitis.

Of 52 patients with proved Trichophyton tonsurans infection of the scalp, 16 had an inflammatory reaction to this infection (kerion), while the rest had only slight scaling and broken hairs (black dot ring-worm). Each patient was tested with trichophytin antigen to determine whether cell-mediated immunity (CMI) was responsible for the differences in these different clinical presentations. Fifteen of 16 with kerion had a 48-hour reaction greater than 10 mm, while none of the 36 with noninflammatory (black dot) tinea capitis had a similar response. This suggests that the patient's immune response (CMI) may be responsible for kerion infections of the scalp and that therapy could be directed initially toward suppressing this reaction.

Intralesional corticosteroids in the treatment of nodulocystic acne.

We evaluated the effectiveness of intralesional injections of corticosteroids in the therapy for nodulocystic acne. Triamcinolone acetonide at a concentration of 0.63 mg/mL was as efficacious as a higher concentration of 2.5 mg/mL. Betamethasone phosphate had little, if any, effect on nodulocystic acne lesions at concentrations of 3.0, 1.5, and 0.75 mg/mL, when compared with saline controls.

Patient concepts and misconceptions about acne.

A questionnaire was completed by 178 patients and relatives with dermatologic conditions as the first phase of a two-part study of the interrelationships between understanding and treatment compliance. Substantial numbers of those surveyed had ideas about cause, treatment, and prognosis that might adversely affect therapy. Diet, dirt, soap and water, topically applied acne gels, and realistic expectations for cures seem to be the most important subjects to emphasize in the written or oral "teaching" of patients with acne.

Atrichia with papular lesions.

We report a case of atrichia with papular lesions in association with common variable immunodeficiency in an 11-year-old boy. There were identical findings in the patient's father. This rare variant of ectodermal dysplasia typically presents with shedding of normal fetal hair within the first three months. Eyelashes are typically spared. Numerous keratin-filled follicular cysts develop over extensive areas of the skin, usually between the age of 2 and 26 years. These lesions are most numerous on the face, neck, scalp, and extremities. Teeth and nails are entirely normal in these patients. The mode of inheritance of atrichia with papular lesions is uncertain. Our case suggests an autosomal-dominant inheritance pattern, while previous reports have shown autosomal recessive inheritance pattern.

Juvenile linear scleroderma associated with serologic abnormalities.

We investigated 24 juvenile cases of linear scleroderma for the presence of systemic disease and serologic abnormalities. Thirteen of 24 patients had antinuclear antibodies (ANA) at titers of 1:40 or greater. Rheumatoid factor (titers greater than or equal to 1:20) was detected in seven of 17 patients tested, five of whom also had ANA. Two of five patients with ANA and rheumatoid factor had systemic diseases, such as nephritis and Raynaud's phenomenon. One patient with ANA developed typical dermatomyositis. Consequently, patients with linear scleroderma may be at some risk for developing systemic collagen-vascular diseases. On initial presentation, patients with linear scleroderma should give a complete history and receive a thorough physical examination as well as undergo laboratory evaluations for the presence of ANA and rheumatoid factor. Long-term observation with periodic reevaluation is appropriate for many members of this group.

Keratosis punctata of the palmar creases.

We determined the prevalence of keratosis punctata of the palmar creases in 534 individuals. The group screened included patients, visitors, and staff in clinics and in a private office setting. The seven cases discovered were all found in black patients, representing 3.1% of this racial group. No relationship to arsenical agents, syphilis, or genetic factors could be ascertained by history.

Nevus of Ota with ipsilateral congenital cataract.

A mongoloid woman had a nevus of Ota, an ipsilateral congenital cataract, and upper extremity hemiatrophy. To our knowledge, no similar patient has been previously reported. We review the literature concerning the nevus of Ota and cataracts in mongoloid indivuduals.

Scurvy. A case caused by peculiar dietary habits.

A 9-year-old girl had the insidious development of lethargy, gingival erosions, and follicular hyperkeratosis with perifollicular hemorrhage. A dietary history disclosed that she consumed only one kind of sandwich and beverage and took no other foods. A skin biopsy specimen was consistent with the diagnosis of scurvy, and marked improvement occurred with ascorbic acid therapy. Although it is an uncommon disorder in the United States, scurvy may occur in persons with prolonged and peculiar dietary habits.

A comparison of the Tzanck smear and viral isolation in varicella and herpes zoster.

We compared the usefulness of the Tzanck smear to viral culture in patients with varicella and herpes zoster. All 11 patients with early lesions of varicella had a positive Tzanck preparation, while only seven (64%) of 11 had a positive culture. Twelve (80%) of 15 patients with zoster had a positive Tzanck preparation, while only nine (60%) of 15 had a positive culture. The predominance of the Tzanck yield over the culture is probably due to the increased difficulty in isolation of the varicella zoster virus. These results suggest that the Tzanck preparation is a valuable tool in the diagnosis of patients with varicella and zoster and offers a much more immediate answer than does viral culture, which often takes one to two weeks. These results contrast with those of our previous study of the Tzanck preparation and herpes simplex, in which the viral culture was more accurate than the Tzanck preparation.

Bacteriology of pustules occurring during treatment of psoriasis.

Pustular lesions developed in 16% of our psoriatic inpatients treated with a modified Goeckerman regimen. The role of bacterial infection in the development of these lesions remains controversial. Representative lesions were cultured, and the recovered organisms were quantitated. We were not able to culture a significant number of bacteria from these lesions. Pustules were prone to develop during the summer months in hirsute patients, in patients with hyperhidrosis, and in patients in whom topical treatment of a more occlusive nature was applied. The male-female ratio was 4.8:1. Topical antibiotic treatment of these pustules in psoriatic patients appeared to be unwarranted since the lesions resolved when topical therapy was discontinued. These pustules appeared to be distinct from pustular psoriasis.

Lipoatrophic panniculitis: a possible autoimmune inflammatory disease of fat. Report of three cases.

We observed three children with a clinically similar presentation of erythematous nodules that expanded centrifugally leaving lipoatrophy. Areas of lipoatrophy coalesced, resulting in clinical pictures similar to partial or total lipodystrophy. Histologic study revealed a lobular panniculitis with a mixed infiltrate of lymphocytes and mononuclear phagocytes. Of these three children, one had insulin-dependent diabetes mellitus and Hashimoto's thyroiditis, one developed juvenile rheumatoid arthritis, and the third developed insulin-dependent diabetes mellitus, suggesting that the pathogenic mechanism may be an expression of autoimmunity.

Gastrointestinal absorption of griseofulvin from corn oil-in-water emulsions: effect of amount of corn oil ingested in man.

The effect of the amount of emulsified corn oil ingested on the gastrointestinal absorption of griseofulvin in man was assessed after oral administration of 5, 10, 15, or 30 gm doses of a corn oil (40% w/w)-in-water emulsion dosage form, each containing 250 mg of microsize griseofulvin. For comparison, griseofulvin absorption from two-125 mg commercial tablets of ultramicrosize drug dispersed in polyethylene glycol 6;000 was also determined. Griseofulvin was almost completely absorbed from the microsize drug emulsions and ultramicrosize drug tablets, whereas 50% of an oral dose is absorbed from commercial microsize griseofulvin tablets. Only 4 gm of emulsified corn oil (as a 10-gm dose of emulsion) is required to maximize the uniformity and extent of griseofulvin absorptions. The emulsion dosage form is uniquely suited for pediatric use.

Epidermolysis bullosa acquisita in childhood. Differentiation from hereditary epidermolysis bullosa.

Epidermolysis bullosa acquisita (EBA), rarely reported in childhood, is described in a 10-year-old black girl. The age of onset during infancy and the clinical appearance mimicked hereditary dystrophic epidermolysis bullosa. Epidermolysis bullosa acquisita was diagnosed by direct immunofluorescence of perilesional skin, indirect immunofluorescence on normal epithelium and saline-split skin, direct immunoelectron microscopy, and immunoblotting of the patient's serum sample against partially purified EBA antigen/carboxyl domain of type VII collagen. Differentiation of mechanobullous disease in children is critical in that significant clinical benefit may be achieved in EBA with prednisone and/or dapsone therapy. A search for associated immunologic abnormalities and HLA-DR typing may help our understanding of EBA.

Psoriasis in children.

Psoriasis in children 16 years of age and younger is common. Frequently the disease is associated with a positive family history and often appears as acute guttate psoriasis. Acute guttate psoriasis is seen commonly after streptococcal infection of the upper respiratory tract, but may be associated with other viral and bacterial diseases as well. Acute guttate psoriasis responds well to treatment but often recurs as more typical small- or large-plaque disease. The treatment of childhood psoriasis should be simple and, if the disease is significant, carried out in the hospital. Results are much more impressive in hospitalized patients than in outpatient settings. Topical corticosteroids should be used only for short periods of time, whereas tar, ultraviolet light, and anthralin have much longer beneficial effects.

Clinical manifestations of human papillomavirus infection in nongenital sites.

Our knowledge of warts dates thousands of years. Most warts represent no more than a transient infection in the hands and feet of children and adults. With the relatively recent medical advances permitting the prolonged survival of immunocompromised hosts, however, HPV-induced lesions have become an important problem. In these patients, lesions represent a recurring, intractable infection that predisposes the patient to the development of skin cancer. Such problems have been appreciated for some time in patients with EV. Newer laboratory techniques have led to an increasing number of clinical entities linked with an HPV cause in the nonimmunosuppressed host. Although evidence incriminating HPV as a causative factor for genital cancers of the cervix and the skin continues to mount, such evidence for nongenital Bowen's disease, certain squamous cell carcinomas of the skin, keratoacanthomas, and other tumors of the skin also has begun to grow. It is to be hoped that continued advances in molecular biologic techniques will further delineate the relationship between HPV and these conditions, lead to an HPV classification scheme that is more utilitarian from a clinical point of view, and ultimately lead to improved treatment.