Calcinosis Cutis and Calciphylaxis in Autoimmune Connective Tissue Diseases.

Calcinosis represents a severe complication of several autoimmune disorders. Soft-tissue calcifications have been classified into five major types: dystrophic, metastatic, idiopathic, iatrogenic, and calciphylaxis. Autoimmune diseases are usually associated with dystrophic calcifications, including calcinosis cutis, occurring in damaged or devitalized tissues in the presence of normal serum levels of calcium and phosphate. In particular, calcinosis cutis has been described in dermatomyositis, polymyositis, juvenile dermatomyositis, systemic sclerosis, systemic lupus erythematosus, primary Sjögren's syndrome, overlap syndrome, mixed connective tissue disease, and rheumatoid arthritis. Calciphylaxis, a severe and life-threatening syndrome presenting with vascular calcifications and thrombosis, has also been associated with some autoimmune conditions. Due to the potentially disabling character of calcinosis cutis and calciphylaxis, physicians' awareness about the clinical presentation and management of these diseases should be increased to select the most appropriate treatment option and avoid long-term complications. In this review, we aim to analyze the clinical features of calcinosis cutis and calciphylaxis associated with autoimmune diseases, and the main treatment strategies evaluated up to now for treating this potentially disabling disease.

Primary cutaneous B cell lymphoma, leg type presenting as a diabetic ulcer: A challenging diagnosis.

INTRODUCTION: A crescent number of reports describe malignant dermal malignancies presenting as diabetic ulcers, such as melanoma, Kaposi's sarcoma, squamous cell carcinoma and cutaneous lymphoma. METHODS: The authors reported the clinical and histopathological features of this challenging case of a PCBCL, leg type presenting as a foot ulcer to exemplify the diagnostic difficulties, mainly when, at the onset, this tumour exhibits uncharacteristic features. CASE REPORT: A 43 years-old male with a 10-year history of compensated type I diabetes developed an ulcerated 3 cm of diameter tumour on the lateral region of the right foot. This lesion had previously been biopsied and treated as a diabetic neuropathic ulcer elsewhere. Due to the appearance of intralesional necrosis associated with stable inflammation and diabetes laboratory parameters, the clinicians made a provisional clinical diagnosis of pyoderma gangrenosum and performed further two incisional biopsies. Histology showed a clear-cut PCBCL, leg type. CONCLUSIONS: Diabetic skin lesions, especially in older patients with persistent non-healing characteristics of pain and tenderness, must be carefully managed through the close correlation of clinical, imaging, and histological features. A correct diagnosis allows avoiding inadequate treatment, which would lead to severe consequences for these patients.

Multimodal treatment of calcific uraemic arteriolopathy (calciphylaxis): a case series.

BACKGROUND: This is an incident series of five dialysis patients with late-diagnosed calcific uraemic arteriolophathy (CUA), severe uncontrolled hyperparathyroidism and infected skin ulcerations. METHODS: A multimodal intervention was based on wound care, antibiotics, surgical debridement, sodium thiosulphate and cinacalcet and associated with regression of skin disease in four cases after varying treatment time periods ranging from 4 to 33 months. RESULTS: Multimodal treatment including sodium thiosulphate and cinacalcet was associated with very favourable local outcomes and survival. This series further confirms that the diagnosis of CUA is rarely made at the nodular, non-ulcerative phase of the disease. CONCLUSIONS: This series contributes to the build-up of case series reporting on the treatment of CUA, and will hopefully serve as a basis of well-conceived comparative effectiveness studies investigating the value of the combined interventions applied so far in this severe condition.

[Calcific uremic arteriolopathy (Calcyphilaxis): a rare disease? Report of three cases]. / Arteriolopatia calcifica uremica: un'entità clinica rara? Report di tre casi.

INTRODUCTION: Calcific uremic arteriolopathy (CUA; CALCYPHILAXIS) is a syndrome that occurs prevalently in patients with chronic kidney disease on dialysis. It is characterized by the medial calcification of skin small arteries leading to necrotic lesions. Several risk factors have been identified: obesity, female gender, diabetes mellitus, hyperphosphatemia, inflammation, treatment with vitamin D, calcium-based phosphate binders and warfarin. MATERIALS AND METHODS: We report three cases of CUA observed from October 2011 to September 2014. RESULTS: The mean age at diagnosis was 56 years (range 33-68). Biochemistry showed: mean levels of PTH=1277 pg/ml (range 1000-1696), serum calcium =10.2 mg/dl (range 9.4-11.1), phosphorus=4.5 mg/dl (range 3.4-5.5). All patients were taking vitamin D, two patients were on warfarin therapy. Following actions were undertaken: interruption of calcium-based phosphate binders, vitamin D and warfarin therapy, initiation of cinacalcet and sodium thiosulfate therapy, use of dialysate with lowest available calcium concentration (1.25 mmol/l), Hyperbaric Oxygen Therapy, surgical dressings of skin lesions three times a week. Significant improvement was observed in mean levels of PTH (331 pg/ml, range 200-465), serum calcium (8.3 mg/dl, range 7.4-9.6) and phosphorus (3.4 mg/dl, range 2.6-3.8). In two out of three patients complete healing of ulcerative lesions was obtained. CONCLUSIONS: These cases underline the importance of early diagnosis of CUA especially in patients with concomitant risk factors and careful clinical monitoring, being CUA characterized by a rapid evolution and high mortality.

LigaSure Haemorrhoidectomy versus Conventional Diathermy for IV-Degree Haemorrhoids: Is It the Treatment of Choice? A Randomized, Clinical Trial.

Introduction. Milligan-Morgan haemorrhoidectomy performed with LigaSure system (LS) seems to be mainly effective where a large tissue demolition is required. This randomized study is designed to compare LigaSure haemorrohidectomy with conventional diathermy (CD) for treatment of IV-degree haemorrhoids. Methods. 52 patients with IV-degree haemorrhoids were randomized to two groups (conventional diathermy versus LigaSure haemorrhoidectomy). They were evaluated on the basis of the following main outcomes: mean operative time, postoperative pain, day of discharge, early and late complications. The time of recovery of work was also assessed. All patients had a minimum follow-up of twelve months (range 12-24). All data were statistically evaluated. Results. 27 patients were treated by conventional diathermy, 25 by LigaSure. The mean operative time was significantly shorter in LS, such as postoperative pain, mainly lower on the third and fourth postoperative day: moreover pain disappeared earlier in LS than CD. The time off-work was shorter in LS, while there was no difference in hospital stay and overall complications rate. Conclusions. LigaSure is an effective instrument when a large tissue demolition is required. This study supports its use as treatment of choice for IV degree haemorrhoids, even if the procedure is more expansive than conventional operation.

[Giant mucocele of the appendix. Case report and review of the literature]. / Mucocele gigante dell'appendice. Descrizione di un caso e revisione della letteratura.

INTRODUCTION: Mucocele is a rare pathology of the appendix characterized by expansion of the lumen for a slow storage of mucous. Represents the 0.2-0.3% of all the appendectomies. Clinical signs and the symptoms are similar to those of the acute appendicitis, while the disease is occasionally recorded and the diagnosis is essentially histological. AIM OF STUDY: To recall the clinical and anatomopathological features of mucocele evaluating the possible evolutions of this rare appendicular pathology according to the updating reports of the literature. MATERIALS AND METHODS: Study of a clinical case. DISCUSSION: In this study diagnostic chriteria and prognostic factors are revised. Authors evaluate anatomopathological classification, possibility of evolution in a preneoplastic and neoplastic lesion and association with other colon cancers. The surgical treatment is evaluated too. CONCLUSIONS: A correct preoperative mucocele diagnosis is emphasized as indispensable in the choice of the proper surgical treatment since a good prognosis is consequent to a radical treatment.

Pancreaticoduodenectomy as the primary therapeutic choice in cystic dystrophy of the duodenal wall in heterotopic pancreas.

On the basis of a review of the literature and description of a clinical case, the aim of this paper was to evaluate the role of pancreaticoduodenectomy as the primary therapeutic choice in a rare, serious condition such as cystic dystrophy of the duodenal wall in heterotopic pancreas. The diagnosis is difficult because of the non-specific clinical manifestations, and radiological and endoscopic imaging are decisive. Computed tomography and magnetic resonance are very useful for demonstrating the presence of cysts in a thickened duodenal wall but endoscopic ultrasonography is the most useful imaging examination. The choice of therapeutic option is still debated. Although some Authors have proposed a medical approach using octreotide or endoscopic treatment for selected patients, pancreaticoduodenectomy is usually proposed for symptomatic patients. When surgery is needed, pancreaticoduodenectomy should be preferred, reserving by-pass procedures for high-risk patients. Because of the non-specific clinical manifestations and the very difficult diagnostic and therapeutic management, these patients need to be studied and treated in specialised, dedicated centres.