Atopic dermatitis (AD) leads to severe psychosocial disturbances that are quite frequently underestimated. We evaluate the quality of life (QoL) of adult patients with AD and their family members using DLQI, B-IPQ, FDLQI questionnaires, observe psychopathological features with CAQ II questionnaire and evaluate correlations between QoL, psychological disturbances and objective/subjective parameters of disease severity. A reduction in QoL has been proven by all questionnaires with statistically significant relationships between all of them. The QoL of the patient (DLQI, B-IPQ) was found to be correlated to FDLQI. We also confirmed statistically significant relationships between DLQI, FDLQI, B-IPQ and objective severity of AD, as evaluated by SCORAD, in DLQI and FDLQI also by TEWL, in B-IPQ also by IgE. All the QoL questionnaires have statistically significant relationship to subjective symptoms (pruritus and sleep disturbance). Interestingly, no significant relationship between QoL and age and extent of eczema in visible localizations was found. CAQ II revealed high numbers of psychological disturbances - the most often paranoia, hypochondria, suicidal depression, anxiety and depression - and high prevalence of suicidal thoughts (10.9%). Although AD is not life-threatening, its negative impact on the QoL of adult patients and their family members/partners can be further influenced by pathological personality traits of the patients.
- Publikační typ
- časopisecké články MeSH
OBJECTIVE: The project was scheduled as a case-control study to investigate the correlation between MMP-2 (rs243864), MMP-9 (3918242), MMP-12 (rs7123600) and TIMP-2 (rs8176329) polymorphisms and chronic venous disease (CVD) risk. The genotype and phenotype research envisages the testing of possible associations between MMP and TIMP-2 genotypes and phenotypes of CVD. MATERIAL AND METHODS: 150 patients with CVD and 227 controls were enrolled into the study. The MMPs and TIMP-2 genotypes were identified by the PCR method and restriction analysis according to standard protocols. RESULTS: The G allele of MMP-2 -790 T/G was 1.85 times more frequent in men with CVD than in the control group (P = 0.008). The T allele of MMP-9 -1562 C/T was observed 2.571 times more frequently in patients with CVD than in the control individuals (both in men and women) with clinically significant specificity (P = 0.0000009). The G allele of MMP-12 rs7123600 was determined 2.082 times more frequently in female patients with CVD than in the control group with clinically significant specificity (P = 0.02). No significant result in TIMP-2 rs8176329 polymorphism in the case-control study was observed. CVD women with G allele in MMP-2 -790 T/G in the genotype-phenotype study are seen to develop ulceration 2.539 times more frequently (P = 0.003). The G allele of MMP-12 rs7123600 was detected 3.167 times more frequently in CVD women with ulceration compared with CVD women without ulceration (P = 0.007). In CVD men in C6 stage, the incidence of AG genotype in rs7123600 MMP-12 polymorphism was found to be 4.675 times higher compared to CVD women with C6 staging (P = 0.005). The AG genotype in TIMP 2 rs8176329 polymorphism was found to be associated with higher risk of tumour (P = 0.01). CONCLUSION: Studying these polymorphisms can contribute to better identification of patients at higher risk of developing CVD, while providing the most appropriate prevention and treatment strategies for limiting the progression and complications of CVD.
- MeSH
- chronická nemoc MeSH
- dospělí MeSH
- fenotyp MeSH
- genetická predispozice k nemoci * MeSH
- genotyp MeSH
- lidé středního věku MeSH
- lidé MeSH
- matrixové metaloproteinasy genetika MeSH
- mladý dospělý MeSH
- polymerázová řetězová reakce MeSH
- progrese nemoci MeSH
- senioři nad 80 let MeSH
- senioři MeSH
- studie případů a kontrol MeSH
- tkáňový inhibitor metaloproteinasy 2 genetika MeSH
- žilní insuficience komplikace genetika patologie MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mladý dospělý MeSH
- mužské pohlaví MeSH
- senioři nad 80 let MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- Názvy látek
- matrixové metaloproteinasy MeSH
- tkáňový inhibitor metaloproteinasy 2 MeSH
- MeSH
- fenotyp MeSH
- genetická predispozice k nemoci MeSH
- genotyp MeSH
- glutathiontransferasa genetika MeSH
- jednonukleotidový polymorfismus * MeSH
- lidé středního věku MeSH
- lidé MeSH
- psoriáza enzymologie genetika MeSH
- rizikové faktory MeSH
- studie případů a kontrol MeSH
- Check Tag
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- dopisy MeSH
- Geografické názvy
- Česká republika MeSH
- Názvy látek
- glutathione S-transferase T1 MeSH Prohlížeč
- glutathiontransferasa MeSH
- GSTA1 protein, human MeSH Prohlížeč
BACKGROUND: Melanoma is a malignant skin disease. The tumor development is caused by an uncontrollable proliferation of melanocytes. The most common occurrence is on the skin, but melanoma may also develop on the mucous membrane, meninges, and eyes. Some melanomas develop from melanocytic nevus. Acral lentiginous melanoma occurs on palms, feet, fingers and under nails, and is the most common type of melanoma for phototype VI. The most important factor for successful treatment of malignant melanoma is an early detection, excision of the primary tumor and histological staging. Surgical treatment of an early-stage melanoma is a key to successful therapy; however, many patients (mostly men) do not seek medical attention before it istoo late. CASE REPORT: This case study presents a 59-year-old patient, who suffers from white coat syndrome and whose finger was amputated for alleged gangrene. Subsequently, brownish black nodules appeared across his arm. Histological examination proved metastases of malignant melanoma. It was only at this phase, when the patient admitted a nevus at the tip of his amputated finger, from which ulceration and gangrene gradually emerged. CONCLUSION: This case demonstrates a combination of multiple unfavorable factors, which led to delayed diagnosis and therapy.
- MeSH
- chybná diagnóza * MeSH
- gangréna etiologie MeSH
- lentigo maligna kůže diagnóza MeSH
- lidé středního věku MeSH
- lidé MeSH
- nádory kůže diagnóza MeSH
- opožděná diagnóza škodlivé účinky MeSH
- Check Tag
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- Publikační typ
- anglický abstrakt MeSH
- časopisecké články MeSH
- kazuistiky MeSH
We present a case of a young 26-year-old woman, who has been suffering from localised scleroderma (morphea) for 15 years. Recently, a lesion on the dorsum of her right foot ulcerated. Based on a CT scan and X-ray a diagnosis of ulcerative osteomyellitis was established. The patient was treated with a combination of antibiotics. Subsequent histological examinations showed granulomatous tissue and chronic inflammatory changes on top of pseudoepiteliomatous hyperplasia. The patients status was deteriorating, which resulted in a limb amputation under the knee. Three months later, there was a metastasis of squamous cell carcinoma found in the patients inguinal lymph node. In spite of combined therapy (surgery, radioterapy and systemic chemotherapy), new metastases occurred and the patient succumbed to the disease several months afterwards. The case was concluded as a squamous cell carcinoma camouflaged by osteomyelitis. Malignant turn of localised sclerodema is very rare. It usually occurs on the lower extremities of patients with a long course of the disease and is associated with pansclerotic or generalised variants of morphea.
- MeSH
- amputace MeSH
- antibakteriální látky terapeutické užití MeSH
- dospělí MeSH
- fatální výsledek MeSH
- lidé MeSH
- lokalizovaná sklerodermie komplikace MeSH
- lymfatické metastázy MeSH
- nádory kůže etiologie terapie MeSH
- osteomyelitida diagnostické zobrazování terapie MeSH
- radiografie MeSH
- spinocelulární karcinom etiologie terapie MeSH
- vředy na noze (od hlezna dolů) etiologie terapie MeSH
- Check Tag
- dospělí MeSH
- lidé MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
- Názvy látek
- antibakteriální látky MeSH
UNLABELLED: Monoclonal gammopathy may manifest itself through a range of skin disorders, including plane normolipemic xanthoma and necrobiotic xanthogranuloma. The present paper describes two patients with these cutaneous symptoms. The first has extensive areas of skin affected by flat xanthomas, monoclonal gammopathy with > 10% infiltration of bone marrow with clonal plasmocytes and, according to PET-CT, unclear lymphadenopathy in the retroperitoneal area. The size of this lymphadenopathy (histologically no malignant infiltration and no confirmed infectious aetiology) has not changed significantly over a 4-year follow-up. Repeated PET-CT scans showed decrease in SUV value in this infiltration from 7.5 to 3.8. Four cycles of treatment with a combination of bortezomib, cyclophosphamide and dexamethasone brought neither reduction in monoclonal immunoglobulin nor change to skin morphology. We believe that the abdominal lymphadenopathy is associated with xanthomatosis but have been unable to confirm this unequivocally. The second patient is being followed up for more than 10 years, originally for MGUS, later for asymptomatic multiple myeloma. Last year, painful subcutaneous and cutaneous infiltrates, isolated on an upper limb and more frequent on lower limb, started to occur. These infiltrates are palpable. PET-CT imaging provided an excellent depiction of these infiltrates, showing no pathology on the head, chest and abdomen and no osteolytic foci on the skeleton. CT imaging showed clearly numerous infiltrates in the skin and subcutaneous tissue of lower limbs, particularly both shanks, reaching up to 2 cm in depth. The largest infiltrate, measuring 3.5 by 2 by 10 cm, was identified in the distal dorsal part of the right shank. PET imaging of lower limbs showed distinctly pathological accumulation in all infiltrates described above; the accumulation of glucose in the lower part of the right shank reached 10.0 SUV. CT images of lower limbs showed increased density saturated hypodermis even in the areas where there is no increased accumulation of 18 fluoroglucose. Following 40 Gy irradiation, the size of infiltrate in the radiated area decreased and their soreness ceased. CONCLUSION: PET-CT imaging offered information on extra-cutaneous signs of plane normolipemic xanthomas and provided excellent depiction of the areas of the skin and hypodermis affected by necrobiotic xanthogranuloma. Chemotherapy with cyclophosphamide, bortezomib and dexamethasone brought no reduction in monoclonal immunoglobulin concentration, and no reduction in plane normolipemic xanthomas. Radiotherapy targeted at large foci of xanthogranulomas led to partial regression and ceased infiltrate soreness.
- MeSH
- lidé středního věku MeSH
- lidé MeSH
- monoklonální gamapatie nejasného významu komplikace diagnóza terapie MeSH
- nekrobiotický xantogranulom komplikace diagnóza imunologie terapie MeSH
- počítačová rentgenová tomografie MeSH
- pozitronová emisní tomografie MeSH
- xantomatóza komplikace diagnóza imunologie patologie terapie MeSH
- Check Tag
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- Publikační typ
- anglický abstrakt MeSH
- časopisecké články MeSH
- kazuistiky MeSH
- přehledy MeSH
IgA pemphigus, resembling subcorneal pustulous dermatosis, represents a rare complication of IgA type monoclonal gammopathy. The patient dates the onset of initial symptoms of vesicular-bullous disease to 1990. She was first examined at our clinic in 2001 with the following conclusion "type IgA monoclonal gammopathy of unknown significance". The first immunosuppressive treatment of vesicular-bullous disorder was administered in 2003 (dexamethasone 20 mg on days 1-4 and 15-18 in monthly cycles + daily cyclophosphamide 50 mg). Cyclophosphamide was administered for 6 months in total and dexamethasone for further 3 months. During the treatment, intensity of the skin disorder ameliorated and monoclonal IgA levels decreased to non-detectable levels. Nevertheless, skin symptoms recurred immediately after dexamethasone treatment in its original intensity was terminated, even though the concentration of monoclonal immunoglobulin IgA remained below the sensitivity of quantitative detection for further 6 months (positive immunofixation only). Six rituximab 600 mg infusions were administered in a weekly interval after stopping cyclophosphamide and dexamethasone to prevent early recurrence of skin symptoms but this treatment was without any lasting effect. Transformation into multiple myeloma was identified in 2007. First line treatment (cyclophosphamide, adriamycin and dexamethasone - CAD) remained without any haematological or dermatological treatment response. Second line treatment (thalidomide, cyclophosphamide and dexamethasone - CTD) brought about significant deterioration of skin symptoms up to the clinical picture of erythrodermia. Third line treatment (bortezomib 1.3 mg/sqm i.v. on days 1,4, 8 and 15, cyclophosphamide 50 mg daily and dexamethasone 20 mg on days 1-4 and 15-18 in 28-day cycles - VCD) resulted in rapid decline in monoclonal immunoglobulin IgA concentrations immediately following the first cycle and to negative immunofixation after 5 cycles. In total, six VCD cycles were administered. The patient has had no skin symptoms from the third cycle of this treatment and complete skin and haematological remission has been maintained for 12 months after completion of bortezomib-containing treatment. Combined treatment containing bortezomib has proven useful in the treatment of IgA pemphigus accompanying monoclonal gammopathy of uncertain significance transformed into multiple myeloma.
- MeSH
- bortezomib MeSH
- cyklofosfamid aplikace a dávkování MeSH
- dexamethason aplikace a dávkování MeSH
- imunoglobulin A krev MeSH
- kyseliny boronové aplikace a dávkování MeSH
- lidé MeSH
- mnohočetný myelom komplikace farmakoterapie MeSH
- paraproteinemie komplikace MeSH
- pemfigus komplikace imunologie patologie MeSH
- protokoly protinádorové kombinované chemoterapie terapeutické užití MeSH
- pyraziny aplikace a dávkování MeSH
- senioři MeSH
- vezikulobulózní nemoci kůže komplikace diagnóza MeSH
- Check Tag
- lidé MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- anglický abstrakt MeSH
- časopisecké články MeSH
- kazuistiky MeSH
- přehledy MeSH
- Názvy látek
- bortezomib MeSH
- cyklofosfamid MeSH
- dexamethason MeSH
- imunoglobulin A MeSH
- kyseliny boronové MeSH
- pyraziny MeSH
BACKGROUND: Amiodarone has belonged to frequently used antiarrhythmic in the treatment of supraventricular and ventricular tachyarrhytmias since the sixties of the twentieth century. Amiodarone is a chemically iodinated benzofuran derivative with mono-N-desethylamiodarone as its major metabolite. OBJECTIVE: This review is focused on numerous adverse reactions. The incidence of amiodarone induced side-effects ranges from 16-98% of patients receiving amiodarone and it appears to be dose related. CASE REPORTS: We describe three cases of hyperpigmentation after using amiodarone in elder men. CONCLUSION: Skin side effects are common, they usually occur as photosensitivity, more rarely as a slate-grey discoloration of the skin. Amiodarone induced slate-grey pigmentation is commonly observed in unprotected light exposed skin. Its incidence ranges from 2-57%. Hyperpigmentation is due to accumulation of amiodarone and its metabolites in the skin.
- MeSH
- amiodaron škodlivé účinky MeSH
- antiarytmika škodlivé účinky MeSH
- hyperpigmentace chemicky indukované MeSH
- léková dermatitida etiologie MeSH
- lidé středního věku MeSH
- lidé MeSH
- senioři MeSH
- Check Tag
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- senioři MeSH
- Publikační typ
- anglický abstrakt MeSH
- časopisecké články MeSH
- kazuistiky MeSH
- přehledy MeSH
- Názvy látek
- amiodaron MeSH
- antiarytmika MeSH
Over a period of 18 years, 17 patients with proven Langerhans cell histiocytosis (LCH) were treated at the Haematological Clinic in Brno. In 13 of them, the disease was diagnosed at adult age, and 4 patients were referred to the centre with LCH diagnosed at early child age. One of these 4 patients suffered from repeated recurrences of the disease at adult age and was diagnosed with progressive neurodegenerative damage of the CNS at the age of 25 which in its terminal phase resulted in the patient's immobility, loss of sphincter control, incapacity to communicate and death at the age of 32. LCH was diagnosed at adult age in 13 patients. The form with primary bone involvement was detected in 8 out of 13 patients (62%). Only 2 of 13 patients (15%) had multiple bone lesions upon diagnosis, the remaining 6 patients (46%) had only one lesion at the time of diagnosis. Repeated recurrence of bone involvement was only recorded in 3 out of 13 patients (23%). The combination of recurrent bone involvement and the development of lung affection (dyspnoea, irritating cough, nodularities and cysts in HRCT images) were documented in 2 out of 13 patients (15%). One of the patients diagnosed with LCH at the age of37 had repeated recurrence of bone involvement, which was also treated by 2 cycles of high-dose chemotherapy and autologous transplantation. He died of bronchopneumonia due to the affection of the lungs by LCH at 48 years of age. Primary extraoseal (extamedular) involvement was diagnosed in 5 out of 13 patients (38%) (mandibular gum infiltration, single cervical node infiltration, hand skin infiltration, infiltration of the perineal region and infiltration of the hypophysial infundibular and primary lung form of LCH). In the 1st case, excision was the solution applied to the infiltration of the lingual side ofthe gums, without further recurrence. In the 2nd case, the infiltrated region of skin over the metacarpophalangeal joint was irradiated and the infiltration disappeared. In the 3rd case, the first sign ofthe disease was diabetes insipidus in a 34-year-old man, and an infiltrate in the anal region similar to condylomata acuminata. The diagnosis was confirmed 2 years after the development of diabetes insipidus from perianal infiltrates. After treatment with leustatin in 4 cycles (10 mg a day for 5 consecutive days), control MR showed that the infiltration in the hypophysial infundibular had disappeared, while the finding in the perianal region only regressed by 50% after therapy with leustatin, the reason for subsequent application of radiotherapy (20 Gy). The finding in the perianal region is normal one year after therapy, but substitution therapy with adiuretin is still necessary. The 4th patient was a case of LCH with primary pulmonary involvement diagnosed on the basis of HRCT and lavage with an immunohistochemical proof (expression of CD1 and of protein S-100) of a high number of Langerhans cells. The occurrence of LCH at adult age is rare and the disease may affect the skeleton as well as other organs. Therefore each new osteolytic lesion should be submitted for histological exam, as well as each pathologic formation, because diagnosing the disease without a microscopic and immunohistochemical exams is not possible. In the case of occurrence of diabetes insipidus at adult age, LCH should be considered as one of the possible underlying diseases. LCH pulmonary involvement should be considered in patients with an interstitial pulmonary process and the examinations should be focused accordingly (thoracoscopy with sampling for histological exams or bronchoalveolar lavage) plus the indispensable immunohistochemical examination.
- MeSH
- dítě MeSH
- dospělí MeSH
- histiocytóza z Langerhansových buněk * diagnóza patologie terapie MeSH
- lidé středního věku MeSH
- lidé MeSH
- Check Tag
- dítě MeSH
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- anglický abstrakt MeSH
- časopisecké články MeSH
- kazuistiky MeSH
Timely diagnosis of malignant diseases largely depends on attention being given to early symptoms and on timely start of an extensive diagnostic process. Only this way can a tumour be diagnosed in its initial stage, and better effect of therapy can be achieved. The following overview provides a list of systemic (paraneoplastic - distant) manifestations of a tumour, and of symptoms related to local tumour expansion. The objective of the overview is to draw attention to all early symptoms of malignant diseases in patients, and to contribute to timely diagnosis and treatment.
