associated with systemic, even lethal manifestations, fi broblast proliferation and accumulation of acid mucopolysaccharides of the hyaluronic acid. Serum IgG class paraproteinemia is always present and it can be detected in all patients after appropriate or repeat testing (1,2,3).A case of an adult male with IgG paraproteinemia, in whom lichenoid papules evolved to scleromyxedema after two years, is reported. L ichen myxedematosus, also known as papular mucinosis, is a primary diff use cutaneous mucinosis. It is a rare cutaneous myxedematous condition characterized by formation of numerous lichenoid papules. Scleromyxedema, or ArndtGottron syndrome, is a rare, confl uent, papular and sclerotic variant of lichen myxedematosus, which is characterized by diff use thickening of the skin that underlies the papules. Th e condition is AbstractLichen myxedematosus, also known as papular mucinosis, is a primary diffuse cutaneous mucinosis. It is a rare cutaneous myxedematous condition characterized by formation of numerous lichenoid papules. Scleromyxedema, also known as Arndt-Gottron syndrome, is a rare, confl uent, papular and sclerotic variant of lichen myxedematosus, characterized by diffuse thickening of the skin underlying the papules. The condition is associated with systemic, even lethal manifestations, fi broblast proliferation and accumulation of acid mucopolysaccharides of the hyaluronic acid. Serum IgG class paraproteinemia is always present and it can be detected in all patients if appropriate or even repeat testing is used. Herein, we present a 67-year-old patient with a 2-year history of skin problems. He had no health problems other than hypertension and diabetes, wich were both diagnosed 15 years before. On examination, the patient exhibited sclerodermoid lesions with diffuse pseudo-sclerodermatous thickening of the exposed skin, microstomia and sclerodactyly-like changes; on the face, there were numerous solid, shiny 2 -4 mm in diameter skin-coloured lichenoid papules, scattered across the forehead, glabellar area, nasolabial folds, perioral region, ear lobes and the neck. Histopathological examination revealed: highly distinctive collagenosis and fi brosis in the middle dermis, increased fi broblasts; collagen bundles with irregular arrangement and fragmentation; alcian blue-positive deposits with appearance consistent with acid mucins. Serum protein electrophoresis detected IgG lambda paraproteinemia. The patient was treated with systematic corticosteroids during 9 months with subsequent introduction of methotrexate and showed satisfactory results. The etiology of scleromyxedema remains unknown, since the purifi ed IgG paraprotein itself has no direct effects on fi broblast proliferation. In scleromyxedema, numerous therapeutic modalities are proposed, unfortunatelly with limited effects. In colclusion, we report a case of an adult male with lichenoid papules; after a two-year progression, they evolved into scleromyxedema and exhibited well response to conventional therapy.
Pachydermodactyly is a rare, benign form of digital fibromatosis, characterized by asymptomatic and progressive, periarticular and usually symmetrical soft tissue finger swelling, specifically on the lateral aspects of the proximal interphalangeal joints mainly of the second, third, and fourth fingers; it mostly affects young adolescents and is probably due to repeated mechanical injury of the skin (such as repeated clasping or rubbing of crossed fingers), sometimes as a result of obsessive-compulsive disorder, which must be distinguished from obsessive “chewing pads”. This paper presents a male patient aged 19, who presented with first symptoms at the age of 12, and was diagnosed with periarticular hypertrophy: localized soft tissue thickening around the proximal interphalangeal joints of all fingers except the thumbs; slight hypertrophy of the skin; absence of subjective complaints; normal joint function. Dermatological status on admission revealed: symmetrical soft tissue swelling of all fingers of both hands except the thumbs at the level of the proximal interphalangeal joints; normal appearance of the distal parts of all fingers; thickening at the level of the proximal interphalangeal joints, bilateral, almost symmetrical hypertrophy (ulnar and radial) of phalanges of the affected fingers except both index fingers, affecting only the ulnar side. The skin lesions were pain-free on palpation, with homogeneous texture and elastic consistency, freely movable over underlying structures. The affected joints showed no functional deficit. The test results, sonography, radiography and histopathology confirmed our clinical diagnosis-pachydermodactyly. The review of the currently available literature, published between 1973 and 2014, including 99 papers and 160 patients, provided important insight into the characteristics and variations of the disease.
Malignant acanthosis nigricans is a rare obligate paraneoplastic dermatosis which accounts for 20% of all acanthosis nigricans cases. The clinical features of the disease are the same as in the benign forms: symmetrical, hyperpigmented, velvety papillomatous lesions mostly involving the axillae, neck, groins, periumbilical cubital and popliteal areas, mammary areolae and less often mucous membranes. However, unlike other forms, it is characterized by sudden onset and rapid spread, commonly (80%) after the age of 40, which may be a marker of malignancy and a key to early diagnosis, indicating the need for a detailed examination. It is a disorder that has no gender differences. Most cases are detected at the moment of cancer diagnosis (61.3%), in fewer cases (about 20%) prior to cancer diagnosis, and in 21% at a later stage of malignant disease. Acanthosis nigricans is usually associated with one of the three or all three forms of paraneoplastic lesions: florid cutaneous papillomatosis, acanthosis palmaris (tripe palms, pachydermatoglyphia) involving the palms and soles, as well as multiple seborrheic keratosis (sign of Leser-Trélat). We report on a female patient with clinically established three paraneoplastic syndromes: malignant acanthosis nigricans, florid cutaneous papillomatosis, and acanthosis palmaris, which appeared before the diagnosis of advanced gastric adenocarcinoma, leading to fatal outcome.
Systemic scleroderma (SSc) is a multisystem disease with microvascular abnormalities, autoimmune disorders, excessive collagen production and deposition, and fibrosis of the skin and internal organs. According to the simplest, though incomplete classification, there are two forms of SSc: diffuse and limited (formerly acrosclerosis). CREST syndrome is a subtype of limited SSc, characterized by: calcinosis, Raynaud’s phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia. We present a patient with all the features of the CREST syndrome, which appeared at the age of 43 and lasted for 23 years. The patient presented with a gradual development of symptoms during the first ten years, from Raynaud’s phenomenon, skin sclerosis, calcinosis, telangiectasia, and esophageal dysmotility. The diagnosis was based on clinical findings and relevant diagnostic procedures. The article presents a literature review on the epidemiology, etiology, pathophysiology, clinical manifestations, various attempts at classification, diagnostic criteria, and therapeutic modalities. When classifying systemic scleroderma into two main types — diffuse and limited, with CREST syndrome as a variant of the latter, it should be pointed out that both types represent clinical forms of systemic sclerosis, share similar visceral involvement, laboratory abnormalities and course which is variable, as was the case in our patient.
Yellow nail syndrome (YNS) is characterized by yellowish nails, lymphedema, sinusitis, and pulmonary involvement and can be triggered by various underlying conditions, such as sinusitis or titanium exposure from an artificial joint or dental implant. Since YNS is potentially treatable, its timely diagnosis is important. The authors recently experienced a case of YNS in a patient who developed sinusitis, yellowish nails, bilateral edema of the extremities, and subclinical rheumatoid arthritis after the implantation of a cardiac pacemaker made from titanium. This case may be the first report of YNS induced by a titanium cardiac pacemaker. A 65-year-old Japanese man developed edema of the face, bilateral hands and forearms, stiffness of the fingers of both hands, an abnormal sensation that affected the whole body, and generalized rash, in the month after the implantation of a cardiac pacemaker for complete atrioventricular block. The implanted pacing system was composed of a Medtronic Ad-visa MRI™ and CapSureFix MRI™ (Medtronic, Minneapo-lis, USA) leads; the body of the pacemaker was made from titanium, while the tip of the lead was coated in titanium. The patient's medical history included hypertension and right carotid artery stenosis with asymptomatic right cerebral stroke. After undergoing carotid endarterectomy, the patient developed atrioventricular block and a permanent cardiac pacemaker was implanted. After right carotid endarterec-tomy, the stent was not placed. When the patient was diagnosed with stroke, he started to take the following medications: low-dose aspirin, clopido-grel, candesartan, pitavastatin, amlodipine, lansoprazole, and eicosapentaenoic acid. On examination, the patient was alert, hemodynamically stable and afebrile. The abnormal physical findings included yellow-colored fingernails (Fig. 1) and (all) toenails, fine crackles at the lung bases, and swelling of the forearms and hands; the extent of the swelling on the left side was greater than that on the right side (Fig. 2). Blood tests showed normal a leukocyte count and C-reactive protein level and were negative for antinuclear anti-bodies, rheumatoid factor, and anti-cyclic citrullinated pep-tide (CCP) antibodies. The erythrocyte sedimentation rate was 32 mm/h. Spirometry showed no evidence of obstructive or restrictive disease. Whole-body contrast-enhanced CT showed no pulmonary lesions, pleural effusion or any findings suggestive of malig-nancy. Upper gastrointestinal endoscopy and colonoscopy showed no evidence of neoplasms. A hand radiograph indicated mild bone erosion of the hands, which was predominant at the metacarpophalangeal joints. Echography of the joints revealed the thickening of the synovial membranes around the carpal bones with increased blood flow, suggesting the presence of synovitis. Head MRI showed bilateral maxillary sinusitis. Based on the yellow nails and diffuse edema as well as the symptoms and signs in relation to the patient's paranasal sinuses and joints, yellow nail syndrome (YNS) along with sinusitis and ...
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