Biomedical subjects
Publications and source records attributed to F Cottoni.
We describe three unusual features of juvenile xanthogranuloma that were observed in three different children. We also describe the mixed and clustered forms of juvenile xanthogranuloma and a giant juvenile xanthogranuloma of the nose.
Lichen planus (LP) is rare in children. A review of the literature reveals that it has some peculiarities with respect to sex, localization, clinical aspect, race, and family history. We present an Indian child with the documented peculiarities of infantile LP. A comparison of LP and graft-versus-host disease points to the importance of thymic involution in the pathogenesis of the former, which could explain the rarity of this disorder in infants.
Primary osteoma cutis arises in the deeper dermis for no apparent reason and presents as mature, lamellar, and osteonic bone; secondary cutaneous osteomas are correlated with inflammatory processes, scars, or dysembryoplasia and are always composed of osteoid. Ultrastructural findings of primary cutaneous osteomas have not been reported to date. Light and electron microscopic findings of a case of primary osteoma cutis are described: mineralized areas may be divided into macrocalcification and microcalcification. Macrocalcification consists of lamellar bone. Osteocytes populate the lamellae, whereas collagen fibril distribution is bone-like. Hydroxyapatite deposition presents as globular or needle-like electron-dense material progressively masking the connective tissue matrix. Microcalcifications, which are found in macroscopically normal dermis around the calcified plaque, consist of osteoid tissue inhabited by osteoblast-like cells. Microcalcifications may be interpreted as metastatic calcifications related to the primary osteoma calcified plaque. Primary osteoma cutis may be considered as true bone amartothic formation rather than dermal mineralization.
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We have investigated the presence of the so-called "herpes gestationis factor" in the serum of fifteen patients with bullous pemphigoid and one patient with cicatricial pemphigoid, that were negative for circulating anti-basement membrane antibodies, using the complement fixation test (substrate: human normal skin, 0 group). We have found herpes gestationis factor in 30% of the serologically negative patients. The presence in the serum of an auto-antibody IgG1 (HGF) highly avid for complement, may determine the false serological negativity in this patient.
Cutaneous leishmaniasis (CL) is endemic in Sardinia where 250 cases were reported from 1922 to 1988. The province of Sassari, in the north of the island, shows the highest number of cases. The vertical distribution of localities where cases had occurred is analysed and the age distribution of cases is given. Isolation of parasites was attempted in 12 cases, but successful growth of Leishmania was obtained from three cultures only. The stocks belonged to two different zymodemes of L. infantum: zymodeme Montpellier (ZMON) 24 and ZMON 24 MPI variant. The distribution of dermotropic L. infantum zymodemes in the Mediterranean area is discussed and the presence of a possible parasite reservoir is suggested.
A patient with mixed essential cryoglobulinemia and polysystemic involvement developed cutaneous lesions characterized by erythematopurpuric maculae and blisters over his entire body. Such lesions appeared during the course of treatment with prednisone and cyclophosphamide when penicillin was added to the therapeutic regimen. The diagnosis of drug-related toxic epidermal necrolysis was made on the basis of clinical history and histologic features. The possible relationship with the underlying immunologic aberration and the active immunosuppression is discussed.
We report the clinical features of 62 consecutive patients with lichen planus observed in 18 months. The largest number of cases occurred between 50 and 70 years of age. Thirty-four patients had lichen planus only. In the remainder, lichen planus was associated with chronic liver diseases (16 cases), immune-related disorders (7 cases), and diabetes (5 cases). Mucous-erosive lichen planus was significantly more frequent in cases with lichen planus and other diseases. In all patients with liver diseases the histological features always showed a severe liver involvement. No relationship was observed between lichen planus and the etiology of the liver diseases. Females were more affected by immune-related disorders than males. The above data, together with the increased levels found of IgA, auto-antibodies, and cryoglobulins, even in cases with lichen planus only, suggest that lichen planus results from an immune imbalance, often associated with systemic involvement.
Numerical and structural chromosomal aberrations were found in metaphases from lymphocyte cultures from Sardinian patients with the sporadic type of Kaposi sarcoma, and also in fibroblasts derived from cultured biopsies of the tumoral lesions. In several cases there was evidence of clonal evolution of some of the chromosomal aberrations. The chromosomes most frequently involved in numerical aberrations were 10, 13, 15, 22 and the X and Y chromosomes, and those most frequently involved in translocations and deletions were 7, 13, 15, 22 and the X chromosomes. The hypothesis is made that in Kaposi sarcoma the chromosomal instability and clonal evolution in vivo could be modulated by the immunologic situation peculiar to the condition.
Twelve Sardinian patients affected by histologically defined classic Kaposi's sarcoma (KS) were HLA-A, B, C and DR typed. Compared to 220 age and ethnically matched healthy controls, KS patients showed a significant increase in HLA-DR5 (66.6 vs 23.1%, P less than 0.001) and a considerable decrease in HLA-DR3 (8.3 vs 53.6%, P = 0.0055). No definite association was observed for other HLA antigens. These results confirm the existence of an HLA associated genetic control of KS susceptibility and support the hypothesis that HLA-DR5 plays the role of a predisposition marker while HLA-DR3 bears a genetic resistance to the disease.
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16 patients with Kaposi's Sarcoma were examined with in vitro tests for cellular immunity and with determination of antibody titres to Cytomegalovirus. It was stated that the Cytomegalovirus antibodies were related with new viral infections favoured by the immunosuppression.
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Several patients with Werner's syndrome in a large family group in Sardinia were ascertained three years ago and reported briefly by Rabbiosi and Borroni (1979). Since then two sisters from a second family and a single case from a third family were ascertained. The three families originated from the Northern part Sardinia and no connection between them was found. We provide a detailed clinical description of six of these patients and attempt to estimate the prevalence and the gene frequency of Werner's syndrome in Sardinia. The prevalence was calculated as 1:94,914 for the two districts of Sassari and Nuoro and as 1:202,766 for the whole island. This is the highest prevalence thus far ascertained. Using Dahlberg's formula we obtained an estimate of the gene frequency q = 0.003288 and thus a frequency of Werner's syndrome of 1:92,515. A more rigorous estimate gave a gene frequency q = 0.001483 and thus a frequency of Werner's syndrome of 1:454,505, but because of the small sample size this estimate should be taken with caution.
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