[Preventative repair of postoperative ventral hernia considering the markers of connective tissue dysplasia]. / Opredelenie pokazaniia k preventivnomu éndoprotezirovaniiu na osnovanii markerov displazii soedinitel'noi tkani u bol'nykh s posleoperatsionnymi ventral'nymi gryzhami.

OBJECTIVE: To define the indication for preventive mesh implantation in patients scheduled for laparotomy. MATERIAL AND METHODS: There were 108 patients divided into the control (52 patients without anterior abdominal wall hernia) and the main (56 patients with ventral hernia) groups. RESULTS: Predominance of collagen type III over type I was observed in patients older 60 years in the main group. The maximum ratio of collagen types I and III was 1.4 in the main group. The minimum number of inter-fiber spaces (n=5) was noted in patients aged 30-40 years. The maximum density of connective tissue was 250 pixels per inch. CONCLUSION: Analysis of connective tissue structures revealed some important age-related features and markers of connective tissue dysplasia in the main group. An indication for preventive mesh implantation in patients scheduled for laparotomy was determined.

Histopathological diagnosis of acral lentiginous melanoma in early stages.

Acral lentiginous melanoma is a rare variant of melanoma that is associated with a relatively low survival rate. The latter is partly due to the advanced stage in which the tumor is usually diagnosed. The diagnostic delay is mainly due to difficulties in identifying the very early histopathological signs of acral melanoma. The current article is a review of diagnostic clues, concepts, and definitions from the literature, as well as illustrating examples from our own archives. We have sought to provide an article that can be easily consulted in difficult cases of acral lentiginous melanoma.

Onycho-pachydermatit with extensive bone marrow edema predominant in the metacarpals: a "forme fruste" of POPP?

Psoriatic onycho-pachydermo-osteo/periostitis (POPP) syndrome is a rare form of psoriatic arthritis with a combination of (i) psoriatic onychodystrophy, (ii) connective tissue thickening, and (iii) periostitis of the distal phalanges. The treatment of the condition has generally been reported to be unsatisfactory with the traditional regimes. Here, we describe a case whom we believe is one presentation of POPP with extensive bone marrow edema of metacarpal bones without distinctive periostitis.

A young man with intimomedial mucoid degeneration of the brachial artery.

Intimomedial mucoid degeneration is a rare disorder and has been described as a distinctly different entity from Erdheim's cystic medial necrosis. Most studies show a strong predominance in African American females with hypertension. In our case report, we describe the presence of a large brachial aneurysm in a young white male with intimomedial mucoid degeneration.

Dystrophic calcinosis in a patient with rheumatoid arthritis.

We report a rare case of dystrophic calcinosis in a patient with rheumatoid arthritis in bilateral buttock lesions and the right elbow joint. The calcinosis was surgically removed because it caused severe local pain, possible infection, and difficulty in sitting. Because no recommended standard pharmacotherapy exists for dystrophic calcinosis, surgical treatment should be taken into consideration when calcinosis causes severe local pain or restricts activities of daily life.

Undifferentiated connective tissue diseases-related hepatic injury.

Hepatic injury is rarely associated with undifferentiated connective tissue diseases (UCTD). We report, here, a case of a middle-aged woman with UCTD-related hepatic injury, including its case history, clinical manifestations, laboratory findings, treatment and its short-term effect. The patient was admitted to the hospital with symptoms of fatigue, anorexia, low-grade fever and skin rashes. She had a past history of left knee joint replacement. Laboratory tests showed elevated levels of serum transaminase, IgG and globulin, accelerated erythrocyte sedimentation rate, eosinophilia and a high titer of antinuclear antibodies (1:320). Imaging studies showed interstitial pneumonitis and hydropericardium. Liver biopsy showed the features which were consistent with those of connective tissue diseases-related polyangitis. After treatment with a low-dose of oral prednisone, both symptoms and laboratory findings were significantly improved. UCTD-related hepatic injury should be considered in the differential diagnosis of connective tissue diseases with abnormal liver function tests. Low-dose prednisone may effectively improve both symptoms and laboratory tests.

[Case of inferonasal subconjunctival orbital fat herniation].

BACKGROUND: We report a case with orbital fat herniation beneath the inferonasal conjunctiva, which is very rare in the literature. CASE: A 52-year-old non-obese female. The subconjunctival mass in the right eye that had formed spontaneously at a young age was yellow, soft, and easily retreated into the orbit with pressure. A magnetic resonance image showed that the intraconal orbital fat herniated through a space that was formed by the inferonasal sclera and the intermuscular septum between the medial rectus muscle and the inferior rectus muscle. Surgical resection obtained good results. CONCLUSION: This route of fat herniation in this case is similar to that of superotemporal lesions in previous reports. One possible explanation of the mechanism of such a rare lesion is congenital weakness in the inferonasal Tenon's capsule.

Bilateral vocal fold immobility in a patient with overlap syndrome rheumatoid arthritis/systemic sclerosis.

Bilateral vocal fold immobility (BVFI) can be the result of a primary disorder or as an iatrogenic complication of surgery or intubation. Laryngeal involvement can be a rare complication of connective tissue disorders and it usually occurs in association with other symptoms and signs that indicate active disease. We present a case of BVFI in a patient with an overlap syndrome rheumatoid arthritis/systemic sclerosis, referred to our division because of dysphonia and dyspnea. The video-laryngostroboscopy showed the presence of BVFI. Physical examination, blood tests, lung and neck high resolution computed tomography scans did not demonstrate significant abnormalities. She was treated with pulses of intravenous methylprednisolone with slow improvement.

Successful treatment with tacrolimus in a case of lung-dominant connective tissue disease.

A 49-year-old man with dyspnea was found to have reticular opacities and ground-glass attenuation with traction bronchiectasis or bronchiolectasis on computed tomography. The patient met the criteria for lung-dominant connective tissue disease (LD-CTD) and histopathologically exhibited a chronic fibrotic interstitial pneumonia illustrating framework of a usual interstitial pneumonia-like pattern. Due to worsening of the disease, therapy was initiated with corticosteroids in combination with cyclosporine A. However, treatment with these drugs was ineffective. Pirfenidone and intravenous cyclophosphamide therapy also proved ineffective. The cyclosporine A was therefore switched to tacrolimus, and the patient's disease improved, allowing for a reduction in the dose of the corticosteroids. Our experience in this case suggests that treatment with tacrolimus might be useful for treating refractory LD-CTD even when histopathologically chronic fibrotic interstitial pneumonia is evident.

The association of myasthenia gravis and connective tissue diseases. Effects of thymectomy in six cases with rheumatoid arthritis and one case with systemic lupus erythematosus.

OBJECTIVES: To describe the effects of thymectomy in a group of patients with myasthenia gravis (MG) with associated connective tissue diseases (CTD). PATIENTS AND METHODS: We analyzed six patients with CTD and myasthenia. They were followed-up for at least 3 years. RESULTS: Records of a cohort of 132 patients with established diagnosis of MG undergoing thymectomy in our institution between 1987-1999 were reviewed. The percentage of patients with CTD was 5 % (6/132). Five patients had rheumatoid arthritis (RA) and one patient systemic lupus erythematosus (SLE). All patients were women, and the mean age was 38.5 years old (SD 13.7). Mean time of MG diagnosis to operation was 16 months (range from 1 to 144 months). Preoperative Osserman classification was the following: stage IIb, four patients; stage III, one patient; and stage IV, one patient. Before surgery all patients were on anticholinesterase agent (pyridostigmine), and four patients were on corticosteroids. An extended transsternal thymectomy was practiced on five patients and a transcervical thymectomy was performed in the remaining patient. Pathologic findings were as follows: thymic hyperplasia in four patients and thymic atrophy in the other two. Good response (remission or improvement) was present in three patients (50 %) and poor response (no change or worse) in the other three (50 %). CONCLUSIONS: A low response to the thymectomy is observed in patients with MG and associated CTD (RA and an SLE).

Ultrasonography of plantar soft tissues thickness in young people with diabetes.

AIMS: The aims of this study were to examine soft tissue changes in the skin and plantar aponeurosis of young people with Type 1 diabetes mellitus (T1DM) and to evaluate any relationship between any soft tissue changes, arch length, limited joint mobility (LJM) and plantar pressure. METHODS: The thickness of the skin on the plantar surface of the foot and plantar aponeurosis were examined using ultrasound in 216 young people with diabetes and 57 controls. Foot length, arch length, joint mobility, peak pressure and pressure time integrals were evaluated. RESULTS: Skin was not significantly thicker in the diabetic subjects. The plantar aponeurosis was significantly thicker in the diabetic subjects and was associated with foot size, male gender and subtalar joint (ST) LJM (P < 0.01). Males were nearly three times more likely to have thickened plantar aponeurosis. CONCLUSION: Soft tissue thickening in young people with T1DM affects the deeper structures on the plantar surface of the foot rather than the skin. Thickening of the plantar aponeurosis was associated with LJM at the ST joint and male gender, but was not associated with plantar pressure or arch height changes. Plantar aponeurosis thickening does not appear to alter foot mechanics in young people with T1DM.

Acquired disorders of elastic tissue: Part I. Increased elastic tissue and solar elastotic syndromes

Elastic fibers in the extracellular matrix are an integral component of dermal connective tissue. The resilience and elasticity required for normal structure and function of the skin may be attributed to the network of elastic tissue. Advances in our understanding of elestic tissue physiology provide a foundation for studyng the pathogenesis of elastic tissue disorders. Many acquired disorders are nevertheless poorly understood due to the paucity of reported cases. Several acquired disorders in which accumulation or elastotic degeneration of dermal elastic fibers produces prominent clinical and histopathologic features have recenthy been described. They include elasdoterma, linear focal elastosis, and late-onset focal dermal elastosis and must be differentiated from better-known disorders, among them acquired pseudoxanthoma elasticum, elastosis perforans serpiginosa, and Favré-Racouchot syndrome.

Acquired disorders of elastic tissue: Part II. Decreased elastic tissue

Elastic fibers in the extracellular matrix are integral components of dermal connective tissue. The resilience and elasticity required for normal structure and function of the skin are attributable to the network of elastic tissue. Advances in our understanding of elastic tissue physiology provide a foundation for studyng the pathogenesis of elastic tissue disorders. Many acquired disorders are in which loss of dermal elastic tissue produces prominent clinical and histopathologic features have recently been described, including middermal elastolysis, papular elastorrhexis, and pseudoxanthoma-like papillary dermal elastolysis, which must be differentiated from more well-known disorders such as anetoderma, acquired cutis laxa, and acrokeratoelastoidosis.