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Biomedical subjects

C Zaccone

Publications and source records attributed to C Zaccone.

7 recordsLinked to original sources

Bullous lesions in acrodermatitis enteropathica. Histopathologic findings regarding two patients.

Acrodermatitis enteropathica (AE) is an autosomic recessive disorder affecting early infancy. Two cases of infantile AE with low plasma zinc levels are reported in which unusually prominent bullous and vesicobullous lesions were seen on the hands and feet, in addition to the more typical erythematous and scaly patches. Both psoriasiform and bullous lesions responded dramatically to oral zinc-sulfate supplementation. The histopathologic features of the bullous lesions of AE have not previously been fully examined. Histologically, the bullous lesions were characterized by intraepidermal vacuolar changes with massive ballooning, leading to intraepidermal vesiculation and blistering, with prominent epidermal necrosis and with no acantholysis. The bullous lesions did not arise on erythematous patchy lesions, but developed ex novo on unaffected skin. The histopathologic differential diagnosis with other bullous conditions is discussed.

Acrodermatitis

Evidence for CD8+ cell increase in long-term PUVA-treated psoriatic patients after PUVA discontinuation.

Long-term PUVA-treated psoriatic patients given maintenance therapy (UVA doses greater than 1,000 J/cm2) have been demonstrated to undergo lymphopenia and a decrease in the total number of circulating CD3+ and CD4+ T cells. The aim of this study was to assess whether the impairment of T cells is detectable also in psoriatic patients after long-lasting PUVA discontinuation. A group of 34 psoriatic patients (25 males, 9 females; mean age 52.7 +/- 12.82 years), who had previously been treated by PUVA therapy (average cumulative dose 1,898.48 +/- 1,207.12 J/cm2), was studied 1 year or more after discontinuation of PUVA therapy. The patients studied failed to show any impairment in CD3+ and CD4+ cells. Nevertheless, a significant increase (p less than 0.05) in circulating CD8+ cells (both in the percentage and the total number) was detectable in PUVA patients as compared to appropriate controls. The significance and implications of this finding are not known and need further investigations.

CD4-CD8 Ratio

Lymphopenia and decrease in the total number of circulating CD3+ and CD4+ T cells during 'long-term' PUVA treatment for psoriasis.

The relationship between high-dose PUVA treatment in psoriatic patients and peripheral T lymphocyte subsets (total number and percentage) has been studied. Of the two groups of patients considered, the first included 19 patients, all affected by chronic, progressively worsening psoriasis; they had never been previously treated by photochemotherapy. The second group included 13 psoriatic patients, who had received an average cumulative dose of 2,007.69 +/- 1,191.05 J/cm2. The 'long-term' PUVA-treated group was assessed while undergoing maintenance therapy. No significant differences were found between untreated patients and healthy controls for any of the parameters considered. A significant reduction (p less than 0.05) in the total number of lymphocytes in long-term PUVA-treated patients both versus untreated patients and controls was found. Furthermore, long-term PUVA-treated patients showed a significant reduction (p less than 0.05) in the percentage of lymphocytes as compared with controls. The reduction in the total number of CD3+ and CD4+ T cells was, moreover, significant (p less than 0.05) as compared with untreated patients. The impairment of circulating CD3+ and CD4+ T cells (total number) was only on the borderline of statistical significance vis-à-vis controls. These findings suggest the usefulness of a careful assessment of circulating T lymphocyte subsets in patients who undergo long-term PUVA therapy.

Adult

Outcome of craniocerebral trauma in infants and children.

The authors examined 927 patients affected by craniocerebral trauma and analyzed the neurological complications that occurred most frequently. The study confirmed that the percentage of craniocerebral trauma, gravity, and complications were different in relation to the sex and age of the patients. Based on this analysis, a correlation was made between the traumatic pathologies and socioeconomic-environmental conditions.

Adolescent

[Necrobiosis lipoidica diabeticorum in children. Description of a case].

A case of necrobiosis lipoidica diabeticorum (NLD) in a 12-year-old male patient is described. Diabetes mellitus (DM) was diagnosed at the age of 1.5 years. The onset of the first NLD lesion had been previously observed at the age of 7, on the back of the left foot. Three new lesions appeared at the age of 8, one on the left leg, the others on the thighs. At the age of 12, four infiltrated, reddish patches, with slight central atrophy were evident on his lower extremities. Histopathological features showed foci of collagen degeneration with sclerosis, surrounded by a chronic, mainly perivascular, granulomatous infiltrate, made up of lymphocytes and histiocytes. The patient is now 19-year old, with no eye or kidney failure, owing to a constant metabolic control of DM. A constant follow-up of the patient demonstrated a self-resolution of NLD plaques, with no evidence of further lesions.

Child

Effects of medium-term PUVA therapy on peripheral T-lymphocyte subsets in psoriatic patients.

Three to four months' PUVA treatment is a widely-adopted procedure to induce psoriasis remission and for the purpose of this study is called "medium-term". The 32 psoriatic patients considered revealed a statistically significant baseline decrease in OKT3+ (p less than 0.001), OKT4+ (p less than 0.001) and OKT8+ (p less than 0.001) as compared with 40 healthy controls, while OKT4/OKT8 was normal. Variance analysis within the psoriatic group failed to reveal further significant variation in the immunological parameters during the 3 months under study. Nevertheless, there was a marked trend towards a reduction in OKT4+ cells and OKT4/OKT8 as compared with baseline values after 3 months. These results suggest that "medium-term" PUVA therapy does not statistically restore the pre-existing baseline changes in T-lymphocyte subsets of the psoriatic patients. The non-statistically significant effects as regards OKT4+ may be due to the small number of patients who reached 3 months' treatment (9 patients) but could be regarded as the first step towards the significant changes described here in long-term PUVA-treated psoriatic patients.

Adult

[Werner's syndrome and intracranial meningioma].

A case of Werner's Syndrome in a 47-year-old man, with typical features of progeria associated with intracranial meningioma is described. A revision of the literature showed that meningioma is the most frequent benign neoplasm in Werner's Syndrome. Meningioma is a peculiar model of neoplasm, because of the frequency of cytogenetical aberrations concerning chromosome n. 22. Either chromosome n. 22 and other chromosomal alterations could be detected in peripheral blood lymphocytes of our patient. These findings suggest a correlation between chromosomal instability and the onset of neoplasms in Werner's Syndrome. Furthermore, the possibility of detecting chromosome n. 22 aberrations in peripheral blood lymphocytes of Werner's Syndrome patients could provide a clue to the presence of a meningioma at a preclinical stage.

Humans