Dermo Beta Brachytherapy with 188Re in extramammary Paget's disease.

AIM: Extramammary Paget's disease (EMPD) is a rare neoplastic pathology involving the vulva, scrotum, and perianal areas, and it is characterized by a slow and insidious course. EMPD may also be associated with internal malignancy, and its clinical presentation features long-standing pruritic lesions, eczema-like, refractory to any therapy. The pathogenesis is unclear, and univocal standardization of treatment is yet to be determined. As regard to the patients who suffer from it, women are more often affected than men. The therapeutic approach depends on the extent of involvement; wide surgical excision is the first choice among treatments, but other forms of therapy, alone or in combination, include imiquimod 5%, photodynamic therapy, Mohs surgery as well as external beam radiotherapy and Brachytherapy. In the present paper a new therapeutic alternative is proposed: Dermo-Beta-Brachytherapy (DBBT) with 188Re. METHODS: Five patients with EMPD, one secondary and four primary cases, have been treated by Brachytherapy with DBBT. This therapy has been successfully used for non-melanocytic skin tumors and basically consists in the topical application of a specially designed, tailor-made mould containing a radioactive beta-emitting isotope, rhenium-188. RESULTS: The patients healed completely, after one session in one case and after two sessions in four cases, with 34 months mean follow-up. CONCLUSION: Brachyterapy could represent a new alternative therapy, instead than invasive treatments as surgery and conventional radiotherapy, capable to treat EMPD independently of its extension, with aesthetic and functional satisfactory results.

Dermatological high-dose-rate brachytherapy for the treatment of basal and squamous cell carcinoma.

BACKGROUND: Basal cell carcinoma (BCC) and squamous cell carcinoma (SCC) are among the most common cancers in humans. Various therapies are currently being used to treat these tumours including surgery, topical treatments and radiotherapy. We describe a new treatment for BCC and SCC. This consists of superficial radiotherapy, using synthetic resin containing a radioactive beta-emitting isotope. The resin is applied to the lesion to perform a selective beta-irradiation brachytherapy treatment. METHODS: In total, 53 patients with histologically confirmed diagnosis of BCC and of SCC were enrolled for the treatment. RESULTS: In all treated cases, an apparent clinical remission occurred in approximately 3 months, and complete healing was obtained in 100% of the treated patients; in 82% of the cases, this occurred after a single application. No disfiguring scars or any side-effects were seen. After a follow-up of 20-72 months (mean 51 months), no clinical relapses were observed in the treated patients. Histological examination confirmed complete tumour regression. CONCLUSION: The results indicated that brachytherapy is an effective treatment for BCC and SCC.

Diagnosis of pigmented skin lesions by dermoscopy: web-based training improves diagnostic performance of non-experts.

BACKGROUND: Dermoscopy has been shown to enhance the diagnosis of melanoma. However, use of dermoscopy requires training and expertise to be effective. OBJECTIVES: To determine whether an Internet-based course is a suitable tool in teaching dermoscopy, and to evaluate the diagnostic value of pattern analysis and diagnostic algorithms in colleagues not yet familiar with this technique. METHODS: Sixteen colleagues who were not experts in dermoscopy were asked to evaluate the dermoscopic images of 20 pigmented skin lesions using different diagnostic methods (i.e. pattern analysis, ABCD rule, seven-point checklist and Menzies' method), before and after an Internet-based training course on dermoscopy. Mean +/- SEM sensitivity, specificity and diagnostic accuracy, and kappa (kappa) intraobserver agreement were evaluated for each diagnostic method before and after training for the 16 participants. Differences between mean values were assessed by means of two-tailed Wilcoxon rank-sum tests. RESULTS: There was a considerable improvement in the dermoscopic melanoma diagnosis after the Web-based training vs. before. Improvements in sensitivity and diagnostic accuracy were significant for the ABCD rule and Menzies' method. Improvements in sensitivity were also significant for pattern analysis, whereas the sensitivity values were high for the seven-point checklist in evaluations both before and after training. No significant difference was found for specificity before and after training for any method. There was a significant improvement in the kappa intraobserver agreement after training for pattern analysis and the ABCD rule. For the seven-point checklist and Menzies' method there was already good agreement before training, with no significant improvement after training. CONCLUSIONS: We demonstrated that Web-based training is an effective tool for teaching dermoscopy.

Pyoderma vegetans and ulcerative colitis.

Pyoderma vegetans (PV) is a chronic, vegetating pustular disorder characterized clinically by erythematous vesiculopustular vegetating cutaneous plaques. Marked epidermal hyperplasia, intraepidermal and subepidermal neutrophilic microabscesses and a dermal inflammatory infiltrate are the prominent histopathological findings. We describe a patient with PV associated with ulcerative colitis and mammary Paget's disease. Pustular eruptions associated with ulcerative colitis are reviewed.

The use of high-dose immunoglobulin in the treatment of pyoderma gangrenosum.

BACKGROUND: Immunosuppressive medications such as corticosteroids and cyclosporin are the most commonly employed therapies in pyoderma gangrenosum. We describe a patient with multiple ulcers of pyoderma gangrenosum on the lower extremities in whom immunosuppressive therapy caused serious side effects and had to be discontinued but who was subsequently treated successfully with high dose intravenous immunoglobulin (IVIG). METHODS: IVIG was given intravenously at a dose of 400 mg/kg per day for 5 consecutive days. After 1 week there was an arrest in the progression of the ulcers and a marked reduction in pain. Two weeks later clinical improvement of the ulcers was observed. Subsequently, IVIG was given at a dose of 1 g/kg per day for 2 consecutive days. RESULTS: The treatment induced a dramatic clinical improvement of one ulcer and healing of the others. Side effects were minimal and well tolerated, and consisted of chills and a slight fever, which resolved with the administration of acetaminophen. CONCLUSION: We feel that IVIG can be used in patients with pyoderma gangrenosum in whom conventional therapies are ineffective or produce serious side effects.

Calcipotriol treatment of confluent and reticulated papillomatosis (Gougerot-Carteaud syndrome).

Gougerot-Carteaud syndrome or confluent and reticulated papillomatosis (CRP), was first described by Gougerot and Carteaud as dermatosis. It is generally considered a rare condition. The eruption consists confluent, flat, brown papules localized primarily to the intermammary and interscapular regions with subsequent spread to the breast and abdomen; at the periphery, the papules spread out forming a pigmented reticulated pattern. At present, the aetiology of CRP remains unknown. The two prominent theories are an abnormal host response to fungi and a keratinization defect. Other hypothesis include photosensitivity, genetic factor, amyloidosis cutis and endocrinopathy.

Pachydermoperiostosis: study of epidermal growth factor and steroid receptors.

Pachydermoperiostosis is a rare osteo-cutaneous disease characterized by hypertrophy of bones and surrounding soft tissues. The cutaneous manifestations include coarsening of facial features, cutis verticis gyrata, digital clubbing, hyperhidrosis and seborrhoea. The pathogenetic mechanism of the disease is still debated, and proposed aetiological factors include genetic influences, anomalies in fibroblast activity, or alteration in peripheral blood flow. We studied a patient with the incomplete form of pachydermoperiostosis, assessing epidermal growth factor receptor (EGF-R) and sex hormone steroid receptors (SR) in the affected skin, and also evaluating the urinary excretion of EGF. The results showed high levels of nuclear steroid receptors, increased cytosolic oestrogen receptors, and no detectable progesterone and androgen cytosolic receptors. EGF-R was also undetectable, and the urinary excretion of EGF was elevated. These findings suggest that the increased tissue sensitivity to circulating sex-steroids could induce enhanced tissue EGF/transforming growth factor alpha (TGF-alpha) production and utilization. The SR-EGF-R system could therefore be involved in determining hypertrophy of the affected tissues.

Evaluation of serine alpha 1-antitrypsin and polymorphonuclear leukocyte elastase contents and their immunogenetic correlation in psoriasis.

The purpose of our study was to quantify the serum content of alpha 1-antitrypsin (alpha 1-AT) and polymorphonuclear leukocyte elastase (PMN-E) in of 21 patients affected by active and stationary psoriasis, and 12 normal controls. HLA typing was also performed to identify a correlation among HLA antigens, age at onset of psoriasis and biochemical results. alpha 1-AT levels were within the normal range in all patients, even in those with active, extensive, inherited and juvenile psoriasis, and in the controls. These data allow us to exclude, in our patients, the presence of rare or defective phenotypes, frequently associated with reduced serine levels of alpha 1-AT. The PMN-E serine content was greatly increased in 3 patients, increased in 2, and slightly modified in 6 cases. All patients with the highest PMN-E levels reported a positive family history and absence of pulmonary, hepatic and atopic diseases. An increased psoriatic inheritance has been observed in the CW6-positive subjects (7/20), comparing B13 and DR6 antigen frequency. No correlation among HLA antigens, age at onset, clinical phase, or biochemical results could be established.

Pachyonychia congenita with late onset of nail dystrophy--a new clinical entity?

Pachyonychia congenita syndrome (PCS) is a genetic disease with an autosomal dominant mode of transmission in which the main sign, pachyonychia, usually arises at birth or in childhood together with other disorders of keratinization. A 28-year-old woman developed subungual hyperkeratosis of all toe-nails and thumb-nails associated with pain on pressure and walking. She had a scrotal tongue with leucokeratotic areas, blister formation, plantar hyperkeratosis, palmoplantar hyperhidrosis and dental cavities since childhood. The present case, interpreted as PCS of late onset, could be a clinical variant of the Jadassohn-Lewandowsky syndrome with the late onset of pachyonychia or else an additional form of PCS due to the expression of a new and different allele.

Allergic contact dermatitis of the lip margins from para-tertiary-butylphenol in a lip liner.

We describe the use of gas chromatography with mass spectrometry (GC-MS) in the investigation of a case of allergic contact dermatitis of the lip margins caused by a lip liner. Patch testing identified sensitization to para-tertiary-butylphenol-formaldehyde resin (PTBP-FR), a resin glue frequently used in leather work but rarely found in cosmetics. Investigation specified the para-tertiary-butylphenol (PTBP) component of the resin as the hapten responsible for the allergic contact dermatitis, as well as for associated depigmentation.

[Lyell's syndrome and lymphoma]. / Sindrome di Lyell e linfoma.

The authors present the case of a patient affected by Lyell's Syndrome and Lymphoma that was revealed in the post-mortem examination. At onset the disease showed mucosal hyperaemia, oedema and erosions. In about ten days macular lesions with vesciculation and bullae appeared on the abdomen. General conditions did not seem to be affected. Three weeks later a fever was noted. No important illness, nor drug assumption were detectable in the history of the patient. At the beginning of the fourth week a dramatic worsening occurred with erythrodermic state and high fever. Death occurred at the sixth week in spite of therapy. Bacteriological and virological examinations were always negative. Post-mortem examination showed retroperitoneal and pelvic neoplastic tissue, involving lumboaortic lymph nodes. Histopathology demonstrated a low grade malignancy non Hodgkin Lymphoma, diffused type with small lymphocytes showing plasmocytic differentiation. The lack of drug intake or infectious disease in the history, underlines the peculiar association between the tumor and the syndrome. Rare cases of TEN associated with lymphoproliferative neoplasm are reported in the literature, and the pathogenetic problem of the syndrome is reviewed.

[Clinical variants of pachydermoperiostosis: complete, incomplete and atypical forms]. / La pachidermoperiostosi nelle sue varietà cliniche: forma completa, incompleta e frusta.

We describe two cases of pachydermoperiostosis, that, with the case that we have already published, allow us to present the complete clinical picture of the disease in the three forms: complete, incomplete and "forme fruste". It is interesting to note that the lack of an important symptom (digital clubbing or CVG) does not exclude the diagnosis but defines the clinical variant. The primitive hypertrophic osteoarthropathy is a common feature for the three patients, marked for I and III case, mild, but important from the diagnostic point of view, for the II case. The identification of a new marker of osteoblastic activity: osteocalcin blood level, is very important to determine the degree of activity of the disease. EM study, performed for the first time, shows an enhanced activity of the fibroblasts and a thickening of the basal membrane of the blood vessels. It is own opinion that the clinical picture observed, the vascular, the metabolic, the EM investigations give a further contribution to the understanding of this disease.