Palmoplantar keratoderma of Unna-Thost: response to biotin in one family.
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Biomedical subjects
Publications and source records attributed to S Menni.
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A variety of skin and oral mucosal lesions appearing in patients after renal transplantation have been reported in the literature. Because most of these data pertain to adults, we studied a group of children with kidney transplants to investigate the occurrence of skin and/or oral mucosal lesions, compare them with those observed in adults, and contribute to the knowledge of the pathogenesis of these disorders. Our series consisted of 32 patients aged 5 to 18 years, all of whom had skin and/or oral mucosal lesions that were classified as being either drug induced, probably related to uremia, or due to other causes. A possible relationship between drug-induced lesions and the therapeutic immunosuppressive regimen in use was investigated. In addition, two formerly unreported lesions were seen in these patients: hypertrophy of lingual fungiform papillae and onychoschizia. The former occurred only in children receiving cyclosporine. Compared with adults, the frequency of gingival hypertrophy was higher and that of infectious disease was lower.
A 2-year-old boy experienced lichen striatus localized to the left half of the body. Some weeks later he developed a peculiar form of eruptive psoriasis limited to the right half of the body. To our knowledge, unilateral eruptive psoriasis has never been described in the literature. The coincidence in this young patient of lichen striatus and this unusual form of psoriasis arising in so short a period of time leads us to hypothesize a common trigger factor.
The hyperimmunoglobulin E recurrent-infection syndrome is considered a primary immunodeficiency syndrome characterized by recurrent staphylococcal infections both cutaneous and pulmonary, chronic dermatitis, otitis and sinusitis. Our case report can be interesting for the age of the patient (39 years old), for the absence of visceral lesion and for the development of a peculiar gingival hyperplasia. The review of literature has shown that this kind of gingivitis is not usual but not extraordinary.
We studied eight patients with incontinentia pigmenti to investigate the possibility of immunologic abnormalities. In six patients a defect of polymorphonuclear chemotaxis was revealed; lymphocyte subpopulations, serum immunoglobulin levels, and peripheral eosinophils were within normal limits. We hope these findings will stimulate further investigations into the mechanisms involved.
The authors describe a 12-year-old boy with a peculiar presentation of preputial papular lesions similar to molluscum contagiosum. Histopathologic investigation revealed the presence of a sebaceous gland opening directly onto the surface and hyperplasia of the epithelium.
The Authors describe the case of a woman affected by generalized plane xanthoma and IgG multiple myeloma with k type immunoglobulins. The association between the two affections is well known. The antilipoprotein activity of the paraprotein has been proved in some cases, associated or not to hyperlipemia. The pathogenetic mechanism of plane xanthoma formation is described briefly. The possible occurrence of plane xanthoma with multiple myeloma is stressed, along with the possible association with other systemic diseases.
An 11-year-old girl affected by keratosis punctata palmaris et plantaris, histologically showing focal acantholytic dyskeratosis, is described. This case demonstrates that keratosis punctata palmaris et plantaris may represent a new clinical expression of persistent multiple focal acantholytic dyskeratosis.
Based on clinical features, infantile seborrheic dermatitis (ISD) can be classified as follows: true seborrheic dermatitis (SD), psoriasiform seborrheic dermatitis (Psor SD), and erythrodermic seborrheic dermatitis. We reviewed the records of 72 children who had been affected by ISD several years earlier to investigate the evolution of disease, to evaluate the patients for the presence of new skin lesions, and to study family histories with respect to these conditions. In addition, we attempted to determine if there is any connection among the initial features of ISD and the types of skin lesions, and atopy or psoriasis. On reexamination, our patients previously diagnosed as having SD showed a larger variety of new skin lesions than those who had Psor SD, and 15% had developed atopic dermatitis. Atopic dermatitis was not present at follow-up in the children with previously diagnosed Psor SD. Psoriatic lesions were present in similar percentages in both groups at the time of reevaluation. Investigation of family history was not useful in predicting later development of psoriasis or atopic dermatitis. We conclude that the initial clinical features of ISD may be of prognostic value, and that SD and Psor SD are probably two different clinical entities.
The authors describe a study of 8 girls suffering from genital and perianal lichen sclerosus et atrophicus. Following the most recent reports in literature, antibodies against Borrelia burgdorferi have been looked for, and the patients have been treated with propicillin. The results are discussed.
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We describe in detail 6 of 18 children affected by a recurrent summer papular eruption on the elbows, knees, and backs of the hands. In all six, family history, clinical observation, or laboratory data revealed signs of atopy. Phototesting, performed to evaluate the role of ultraviolet rays in the pathogenesis of the disease, gave results similar to those obtained in subjects with atopic dermatitis. Therefore we suggest that this disease may be a peculiar morphologic variant of atopic dermatitis.