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.
Lichen 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, confluent, 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, fibroblast 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 fibrosis in the middle dermis, increased fibroblasts; 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 purified IgG paraprotein itself has no direct effects on fibroblast 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.
Yellow Nail Syndrome - a Case Report Paravina, Mirjana; Binić, Ivana; Popović, Danijela ...
Serbian Journal of Dermatology and Venerology,
6/2015, Letnik:
7, Številka:
2
Journal Article
Recenzirano
Odprti dostop
Yellow nail syndrome is a rare disease of unknown etiology. It is clinically characterized by a triad of yellow nails, lymphedema at one or more sites, and chronic respiratory disease (bronchitis, ...bronchiectasis and rhinosinusitis). All nails may be affected, but some may be spared. The nail plates are yellowish green, thickened, occasionally with transverse ridging and onycholysis, with increased longitudinal and transversal over-curvature, with partial or complete separation of the nail plate from the nail bed, without lunula and cuticle and slow nail growth rate. The lymphedema is usually peripheral, affecting the lower limbs, or in the form of pleural effusion.
This is a case report of a 47-year-old female patient who presented with nail changes at the age of 40; two years later the patient developed lymphedema of the lower limbs, and a year later a chronic respiratory disease. The affected nails were yellow to yellow-gray, with thickened nail plates separated from the nail bed, ingrown in the perionychium, without lunula. At the same time, additional examinations revealed the following associated conditions: edema of talocrural joints in both legs, chronic obstructive bronchitis, bronchial asthma, chronic rhinitis with bilateral nasal polyposis, labile arterial hypertension. Apart from the management of chronic respiratory disease, oral vitamin E capsules (200 mg 3 times a day) and topical vitamin E solution were administered over 15 months. The nails began to grow, and the newly grown nails were of normal pigmentation.
In conclusion, we present a case of an adult female patient with yellow nail syndrome, and a recognized association of peripheral edema and chronic pulmonary disease. The patient had a typical clinical picture, all the nails were affected, but showed a favorable response to systemic and topical vitamin E 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.
Verrucous epidermal nevi are noninflammatory, congenital, cutaneous hamartomas composed of keratinocytes, abnormal clone(s) of cells that reflect genetic mosaicism arising from different somatic ...mutations. Some of these mutations are well recognized, but some are still unidentified. Molecular techniques are used for identification and classification of molecular causes of certain epidermal nevi, whereas all verrucous epidermal nevi are divided into epidermolytic and non-epidermolytic types. They are typically present at birth, but may appear during childhood, even later. Their prevalence in adults ranges from 0.1 to 0.5%, equally affecting both sexes, and about 1 in 1000 newborns.
Warty, brown papules without inflammation distinguish verrucous nevi from other epidermal nevi, while presence at birth and persistance distinguish verrucous epidermal nevi from linear viral warts. Epidermolytic and non-epidermolytic verrucous epidermal nevi are almost always hard to distinguish, except by histology. As a rule, verrucous epidermal nevi are asymptomatic, they have a benign course, except occasionally, and therapy is mostly used for cosmetic reasons. Simple excision is usually the treatment of choice. Topical agents are rarely curative, as well as surgery which is associated with relapses, unless both epidermis and the underlying dermis are removed or destroyed at the same time.
We present a case of an otherwise healthy 21-year-old female patient, who presented with a solitary congenital verrucous cauliflower-like lesion in the right zygomatic region of the face. The lesion was present from birth. Due to its gradual growth during years, the lesion became a great esthetic and functional problem for this young patient. There was no family history of similar or any other tumorous skin lesions in the family. On examination, the patient had a solitar unilateral, well defined yellowish cauliflower-like verrucous lesion confined to the right malar side of the face. The lesion was distributed along the lines of Blaschko extending horizontally, from its wider 1.5 cm cauliflower-like part on the right zygomatic region, towards its tail-like 0.5 cm thick end on the preauricular region, in approximately 3 cm long tail-like manner without crossing the midline. Since the patient refused biopsy, no exact differentiation between epidermolytic and non-epidermolytic nevi was possible. The diagnosis of verrucous epidermal nevus was based on history and clinical presentation, as a diagnosis of exclusion. Due to the fact that patients with epidermolytic verrucous epidermal nevi are at risk of parenting a child with bullous ichthyosiform erythroderma, the patient was counseled on this risk, and on the possibility of first-trimester antenatal diagnosis. The lesion was successfully treated by radio-wave surgery.
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.
In this article, the expression for the Drazin inverse of a modified matrix is considered and some interesting results are established. This contributes to certain recent results obtained by Y.Wei 9.
In this paper, we consider the Drazin inverse of a sum of two matrices and derive additive formulas under conditions weaker than those used in some recent papers on the subject. As a corollary we get ...the main results from the paper of Yang and Liu H. Yang, X. Liu, The Drazin inverse of the sum of two matrices and its applications, J. Comput. Appl. Math. 235 (2011) 1412–1417. As an application we give some new representations for the Drazin inverse of a block matrix.
The levels of glutathione (GSH) and glutathione peroxidase (GPx) activity were measured in the erythrocytes of 50 patients with clinically isolated syndrome of CNS (CIS) and 57 patients with ...relapsing remitting multiple sclerosis (RRMS).
A decrease in GSH content and GPx activity showed significance in both study groups compared to the control values (p = 0.0025 and 0.007 for GSH and p = 0.005 and 0.003 for GPx, in CIS and RRMS patients, respectively). The depletions were more pronounced in RRMS than in CIS patients (p = 0.009 for GSH and p = 0.031 for GPx). The results significantly verify the negative correlations between GSH values and clinical severity (r = -0.513, p = 0.004), radiological findings (r = -0.351, p = 0.008) and disease duration (r = -0.412, p = 0.0025) in CIS patients. The same correlations were observed in RRMS patients between GSH values and clinical severity (r = -0.498, p = 0.004) and patients' radiological features (r = -0.454, p = 0.005). No correlations were observed between GSH values and other patient characteristics, or between GPx activity and all tested patient characteristics (p > 0.01).
The results indicate that GSH content and GPx activity both decreased below the normal range and were accompanied with neuroinflammation, but although both might have great importance in neuroinflammation development, the data presented here confirm that only GSH might serve as a marker which is closely correlated with neurological and radiological scoring of acute CNS inflammation.
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