Spindle-cell myoepithelioma: A rare case in a finger of a 15-year-old boy.

Cutaneous myoepithelioma is a rare neoplasm of the skin that has become more widely recognized in recent years despite significant diagnostic pitfalls. It is a benign neoplasm with a high recurrence rate if not excised radically, and must be distinguished from its malignant counterpart. Few cases have been described so far and, to our knowledge, no cases in the finger of a child exist in the literature. We report the case of a 15 year-old boy affected by a rare form of locally aggressive spindle-cell myoepithelioma, and suggest a new multidisciplinary approach combining surgical excision and custom brachytherapy.

Eccrine syringofibroadenoma radiation treatment of an unusual presentation.

Benign skin diseases have seldom been treated with radiotherapy, unless resistant to other treatments. Eccrine syringofibroadenoma (ESFA) is a rare benign eccrine tumor. ESFA very rarely presents bilateral lesions, and seldom a nonsurgical treatment has been proposed. An exceptional case of bilateral ESFA of the foot is presented; radiotherapy was effective in treating both lesions with good cosmetic results.

Polyglandular autoimmune diseases in a dermatological clinical setting: vitiligo-associated autoimmune diseases.

Vitiligo is an acquired hypomelanotic disorder characterized by depigmented macules resulting from the loss of functional melanocytes. Many different etiological hypotheses have been suggested for vitiligo, the most recent of which involves a combination of interacting environmental and genetic factors. Among the various pieces of evidence in support of an autoimmune origin of vitiligo, there is the epidemiological association with several autoimmune diseases. The most frequently reported association is with autoimmune thyroiditis; however, other diseases such as rheumatoid arthritis, diabetes mellitus, pernicious anemia and chronic urticaria have been described in variable percentages, depending upon the genetics of the population studied. Among the diseases described in association with vitiligo there are the so-called autoimmune polyglandular syndromes (APS). Here we report 31 cases of APS diagnosed in 113 vitiligo patients, according to the newest classification. Autoimmune association was more present in generalized non segmental vitiligo and was more frequent in females. The most frequent association was with thyroid autoimmune disease, followed by autoimmune gastritis and alopecia areata. ANA positivity was similar to that reported previously in the general population. We stress the importance of an assessment for autoimmune diseases in vitiligo patients.

Modified Matti-Russe technique using a "butterfly bone graft" for treatment of scaphoid non-union.

INTRODUCTION: Autologous bone grafts are used to treat scaphoid non-union with shortening and humpback deformity. The superiority of internally fixed bone graft to embedded bone graft has not been clearly proven. METHODS: Retrospectively analyzed 42 cases of scaphoid waist non-union treated using the "butterfly bone graft" (modified Matti-Russe technique). RESULTS: Complete healing was reached by all patients, with an average time of 4.4 (SD ± 0.7) months. Mayo Wrist Score improved from and QuickDASH score significantly improved in all patients after treatment. CONCLUSIONS: Butterfly bone graft is effective and reliable in treating scaphoid non-union with shortening and humpback deformity.

Photodynamic therapy plus topical retinoids in Darier's disease.

Darier's disease is a rare, genetically determined, chronic disorder of keratinization. Characterized clinically by a symmetrical eruption of hypercheratotic papules with predilection for the seborrhoeic areas of the body. Many treatments have been suggested: emollients, topical retinoids, dermoabrasion, topical steroids and intermittent courses of oral antibiotics. Systemic retinoids are usually the treatment of choice. We present a small case series of patients treated with PDT and topical retinoids with good results. This therapeutic modality could represent an alternative to systemic retinoid treatment.

Drug-induced cutaneous lupus erythematosus after 5 years of treatment with carbamazepine.

Drug-induced lupus is a disease, inducible by several drugs, that shares symptoms and laboratory characteristics with idiopathic lupus erythematosus. We report a case of carbamazepine-induced cutaneous lupus erythematosus which developed after 5 years of treatment in a patient who was on this medication because of an epileptic crisis after cranial trauma. Carbamazepine is a medication rarely implicated in drug-induced cutaneous lupus, moreover there are very few reports of such long periods between the start of therapy and the presentation of the clinical symptoms. We describe this case to emphasize the possibility of cutaneous lupus induction by carbamazepine even after many years of therapy.

Interferon beta-1b modulates MCP-1 expression and production in relapsing-remitting multiple sclerosis.

Monocyte chemoattractant protein-1 (MCP-1) seems to be involved in the pathogenesis of multiple sclerosis (MS). We found that in unstimulated (PHA(-)) and PHA-stimulated (PHA(+)) peripheral blood mononuclear cells (PBMC), MCP-1 and TNFalpha levels are higher in stable untreated MS patients. Interferon gamma (IFNgamma) is higher in relapsing patients in PHA(-) cultures and in stable patients in PHA(+) cultures. Chronic IFNbeta-1b treatment down-regulates TNFalpha, IFNgamma and MCP-1 production except for TNFalpha in relapsing patients. IFNbeta-1b, in vitro, increases MCP-1, TNFalpha and IFNgamma spontaneous production in all patients. Multivariate analysis suggests that MCP-1 production is dependent from clinical status and not from TNFalpha and IFNgamma production. Logistic regression analysis shows that MCP-1 production is significantly modified by treatment. Further studies are needed to clarify the role of MCP-1 in MS.

Treatment of cutaneous calciphylaxis with sodium thiosulfate: two case reports and a review of the literature.

Cutaneous calciphylaxis is a potentially fatal condition characterized by calcium deposition in dermal arterioles and the subsequent development of livedo reticularis, plaques, and extremely painful ulcers. This condition may be present in up to 4% of end-stage renal disease patients. Several treatments, which mainly attempt to control calcium phosphate metabolism, are available for this condition. We describe two patients treated with sodium thiosulfate with good results. Moreover, we also performed a PubMed literature search of sodium thiosulfate treatment for calciphylaxis. We found 41 cases of which most (> 90%) presented a rapid and sustained resolution, indicating this drug is a very good candidate for the treatment of this condition.

Finger thermoregulatory model assessing functional impairment in Raynaud's phenomenon.

Raynaud's Phenomenon (RP) is a paroxysmal vasospastic disorder of small arteries, pre-capillary arteries, and cutaneous arteriovenous shunts of the extremities, typically induced by cold exposure and emotional stress. RP is either primary (PRP) or secondary to systemic sclerosis. In this study we use Control System Theory to model finger thermoregulatory processes in response to a standardized cold challenge (a diagnostic test routinely performed for differential diagnosis of RP). The proposed model is based on a homeostatic negative feedback loop, characterized by five distinct parameters which describe how the control mechanisms are activated and maintained. Thermal infrared imaging data from 14 systemic sclerosis subjects (SSc), 14 PRP, and 16 healthy control subjects (HCS) were processed. HCS presented the fastest active recovery, with the highest gain. PRP presented the slowest and weakest recovery, mostly due to passive heat exchange with the environment. SSc presented an intermediate behavior, with the longest delay of response onset. The estimated model parameters elucidated the level of functional impairment expressed in the various forms of this disease.

Urokinase plasminogen activator mRNA is induced by IL-1alpha and TNF-alpha in in vitro acantholysis.

The role of urokinase type plasminogen activator (uPA) has been well documented in the pathogenesis of pemphigus vulgaris (PV). Activation of plasminogen into active serine protease plasmin initiates extracellular proteolysis leading to acantholysis but the mechanisms underlying this process are not clearly understood. We have previously shown that keratinocyte derived cytokines IL-1alpha and TNF-alpha are involved in PV-induced acantholysis. In the present study we sought to examine whether keratinocyte-derived IL-1alpha and TNF-alpha are correlated with uPA induction in keratinocytes during acantholysis. Normal human keratinocytes were incubated with diluted PV serum. mRNAs for IL-1alpha, TNF-alpha and uPA were examined with RT-PCR at various time points and acantholysis was measured. IL-1alpha, TNF-alpha and uPA mRNAs were all induced in keratinocytes following PV serum stimulation; IL-1alpha/TNF-alpha mRNAs' expression was earlier than the expression of uPA mRNA. To further examine the role of IL-1alpha, TNF-alpha and uPA in acantholysis, we performed antibody blocking studies. Anti-IL-1alpha, anti-TNF-alpha and anti-uPA antibodies suppressed acantholysis by 76%, 80% and 90%, respectively. In addition, anti-IL-1alpha and anti-TNF-alpha antibodies inhibited uPA mRNA induction, whereas anti-uPA antibodies did not alter IL-1alpha/TNF-alpha mRNAs' expression. Our results confirm the role of uPA in acantholysis and suggest an involvement of IL-1alpha/TNF-alpha in uPA induction.

The role of interleukin 10 in the pathogenesis and potential treatment of skin diseases.

UNLABELLED: Interleukin 10 (IL-10) is a key cytokine produced by a multitude of immune effector cells and possesses distinct regulatory effects on immune functioning in the skin. In this article we report the current understanding of the immunobiology of IL-10 and identify the role of IL-10 in cutaneous infection as well as in autoimmune and neoplastic processes. We reviewed the literature to examine the function of IL-10 in different cutaneous disorders. IL-10 can influence and potentially treat T1/T2 differentiation, antigen-presenting cell functioning, antigen-presenting cell-mediated T-cell activation, and T-cell, B-cell, and mast cell growth and differentiation that is aberrant in various disease processes. The literature consensus is that the multitude of effects of IL-10 contribute to the pathogenesis of different skin disorders. In certain circumstances IL-10 could represent novel therapeutic approaches to treating cutaneous diseases. LEARNING OBJECTIVE: At the conclusion of this learning activity, participants should be acquainted with the role of IL-10 in many infectious diseases, autoimmune skin disease, inflammatory processes, and malignancy. Its possible role in the resolution of various skin diseases should be better understood.

Nail psoriasis: combined therapy with systemic cyclosporin and topical calcipotriol.

BACKGROUND: Nail psoriasis is a common problem in psoriatic patients and often it is difficult to cure. Several treatments have been proposed in the last decade using new molecules like vitamin-D analog and/or immunosoppressive drugs both systemically and locally. OBJECTIVE: Our goal was to evaluate a combination of cyclosporin and topical calcipotriol cream versus cyclosporin alone in a matched group of patients treated with cyclosporin alone. METHOD: Fifty-four patients affected by severe psoriasis and nail involvement were selected and matched for severity of nail involvement, sex, age, and cyclosporin dosage. Group A included 21 patients treated with cyclosporin alone (3.5 mg/kg/day) for three months. Group B included 33 patients treated with the same cyclosporin dosage plus, for the same time, topical application of calcipotriol cream twice a day. Evaluation for clinical improvement was the personal feeling of the patient after three months, while clinical appearance of the lesions was evaluated by the same dermatologist using digital pictures and who was blind as to the treatment of the patient. A score ranging from + to +++ was used in order to evaluate the improvement, and data were statistically evaluated with the Wilcoxon test. RESULTS: Both cyclosporin alone and a combination of cyclosporin with topical calcipotriol twice a day were useful for treating nail psoriasis after three months of therapy although the combined therapy showed a better overall result in both mild and severe nail psoriasis. Improvement of the clinical appearance of the nail lesions was seen in about 79% of patients in group B (p < or = 0.0004) versus about 47% of patients in group A (p < or = 0.15). CONCLUSIONS: In patients with severe involvement of nail psoriasis we suggest the use of a combination of topical calcipotriol twice a day with systemic treatment such as cyclosporine.