Sclerotic (hypocellular) fibromas of the skin.
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Biomedical subjects
Publications and source records attributed to A Amantea.
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The localization of three monoclonal (A,B,C) anti-cytokeratin antibodies and of an anti-thymostimulin antibody were studied in normal children's thymuses, aged from 2 months to 10 1/2 years and in Down's children thymuses, aged from 5 months to 6 1/2 years. Two anti-cytokeratins were positive in the thymus: the anti-B was found in the epithelial cells of all thymic zones, the anti-C only in the external cells of Hassall's corpuscles. The distribution and the intensity of immuno-reactions were the same in normal and in Down's thymuses. The distribution of anti-thymostimulin was superimposed to the distribution of anti-cytokeratin B and was similar in normal and in the youngest Down's thymuses, whereas in the 6 1/2 years-old Down's thymuses there was a loss of anti-TS reaction in the subcapsular zone. A relationship between the reduction of anti-thymostimulin immuno-reaction and the beginning of an eventual loss of T-lymphocyte differentiation was supposed.
Ultraviolet (UV) irradiation is capable of producing a dose-dependent decomposition of skin surface lipids and particularly of squalene, with the concomitant generation of active lipoperoxides. The biological effects of UV-peroxidated squalene were tested, compared with those produced by synthetic lipoperoxides (cumene hydroperoxide), on some immunological parameters in vivo modified by UVB irradiation. Application of UV-peroxidated squalene as well as cumene hydroperoxide significantly inhibited the induction of contact hypersensitivity to dinitrofluorobenzene in mice, which was associated with a decrease in the number of ATPase positive cells. The effect was dose-dependent (over 40 micrograms for peroxidated squalene and over 20 micrograms for cumene) and relevant after 2 d of treatment. Down-regulation towards the applied hapten was demonstrated. The results indicate that UV-induced lipoperoxides of squalene are capable of inhibiting the induction of contact hypersensitivity in mice and suggest that, among the other photoproducts generated in humans, squalene peroxides may play a role as biochemical messengers of the biological effects of UV irradiation of the skin.
The Authors report 3 cases of "Congenital Smooth Muscle Hamartoma" (CSMH). CSMH presents as congenital patches or slightly indurated plaques with prominent overlying hair, often hyperpigmented. Histopathologic examination showed increased numbers of well-defined smooth muscle bundles in the superficial and deep dermis. CSMH and Becker's nevus should be considered in the differential diagnosis of any congenital hairy hamartoma.
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Two rare cases of perforating pilomatrixoma similar to those reported in the literature are described. Clinical examination showed in two old-women a reddish inflammatory exophytic tumor, 1 cm in diameter, with central erosive surface, with a rapid growth (3-5 months). Histologic examination showed: multiple masses of basophilic cells situated in the upper dermis making contact with the epidermis in a "follicle-like" opening; occurrence of transepithelial elimination phenomena.
The eccrine angiomatous hamartoma is a rare cutaneous lesion histologically characterized by the presence in the intradermal lobules of mature eccrine sweat glands and angiomatous capillary channels. We report a case of eccrine angiomatous hamartoma with unusual lipomatous involvement.
We report a case of multiple minute digitate hyperkeratosis. The patient had hundreds of keratotic lesions--some tiny and spiked, others larger, flat papules--localized on the trunk and arms. Histologic sections showed focal areas of compact hyperorthokeratosis with few dermal changes. Ultrastructurally, keratohyalin granules were smaller than normal. The keratin pattern was one of a normal variant. Odland bodies present. Various family members of the patient, in different generations, were affected.
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Porphyrins in urine, plasma, erythrocytes and feces have been tested in two brothers affected by Rotor's syndrome and in three of their phenotypically normal relatives. In all five subjects normal values of delta-aminolevulinic acid and porphobilinogen in urine, and of prophyrins in plasma, erythrocytes and feces, were found. The two patients showed a marked increase in total urinary coproporphyrin excretion with a high percentage of isomer I. These observations confirm the hypothesis of a different route of the porphyrin excretion in Rotor's syndrome with a shift from the fecal route to the urinary one, and do not agree with the suggestion of an increased hepatic porphyrin production in this type of hyperbilirubinemia.
Two cases of hereditary coproporphyria showed unusual nervous system involvement, one epilepsy with onset in childhood, and the other chronic central and peripheral nervous system damage. The literature is briefly discussed.
An anti- thymostimulin (TS) serum was tested on calf thymus to study the localization of the hormonal factor. The immunoperoxidase method was applied to tissue fixed in Bouin's fluid and embedded in paraffin, or to tissue fixed in paraformaldehyde and embedded in Epon for semi-thin sections. Immuno-reactivity was shown, with DAB- Fluka , in reticulo-epithelial cells in the medulla, and between the cortex and the medulla, while with DAB-Sigma reactivity was found in the cortex as well. The external cells of Hassall's corpuscles were also reactive. Myoid cells were not reactive. In semi-thin sections a weak reactivity was noted at the periphery of a few lymphocytes. Comparison with the localization of other thymic factors, and the possibility of a functional cycle of the epithelial cells synthesizing one or more factors are discussed.
Little is known of the natural progression of untreated porphyria cutanea tarda. We report sixteen cases (fourteen sporadic and two familial) in which the cutaneous and biochemical abnormalities improved without any specific therapy other than the avoidance of hepatic toxins.
In 30 patients affected by Porphyria Cutanea Tarda we determined: plasmatic, erythrocytary and urinary zinc (by atomic absorption spectrophotometry); total porphyrins in plasma and in urines, coproporphyrins and protoporphyrins in erythrocytes (by spectrophotometric methods); haptoglobin, hemopexin, alpha 2-macroglobulin and albumin (by immunological methods). The results obtained were compared with those of normal subjects. In addition, the variables under consideration were statistically analyzed to bring out possible correlations (both simple and partial). The most interesting result (besides the documentation of an increase in the urinary and plasmatic zinc in porphyric patients) was the finding of correlations between plasmatic zinc, plasmatic porphyrins and alpha 2-macroglobulin.
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