Ocena morfologii i dystrybucji naczyń włosowatych w kapilaroskopii wału paznokciowego u chorych na twardzinę układową i zdrowych osób

Introduction: Systemic sclerosis (SSc) is a connective tissue disease characterized by vascular damage and immunological abnormalities leading to fibrosis that can damage multiple organs. The pathogenesis is complex and still poorly understood. However impaired angiogenesis in SSc has a major role in tissue injury and sequelae fibrosis. Nailfold capillaroscopy (NC)/nailfold videocapillaroscopy (NVC) is safe and non-invasive methods used to investigate microvascular changes in the peripheral circulation and it is a method of great diagnostic value in diagnosing and monitoring the patients with SSc. Typical microvascular alterations, called scleroderma pattern characterized by giant capillaries, haemorrhages and successive loss of capillaries, are observed at NC/NVC in a significant percentage of SSc patients, hence our interest was focused on the assessment of NVC in patient with systemic sclerosis (SSc). Material and methods: Thirty patients with SSc according to the ACR and EULAR criteria and healthy volunteers underwent NVC assessment. Nailfold capillaroscopy was performed by a videocapillaroscope and the picture of the capillaries at the hands were documented and evaluate. Results: NVC disturbed patterns were significantly prominent in SSc patients (p < 0.05) compared to the healthy control group. A normal capillaroscopic pattern was not observed in patients with SSc. The number of loops/mm was significantly lower in SSc group (p < 0.05) and was 4.28 capillaries/mm (min.1/mm; max. 10/mm). We did not notice significant difference in frequency of mega-capillaries (lcSSc/dcSSc: 41%/29%, p > 0.05) and avascular areas (lcSSc/dcSSc: 64%/57%, p > 0.05) between limited (lSSc) and diffuse (dSSc) SSc. Conclusions: Severe capillary damage is characteristic for SSc patients therefore NVC seems to be useful for selection of patients developing SSc

Choroba Kyrlego

Kyrle’s disease is an acquired perforating dermatosis, which presents as hyperkeratotic papules mainly on the calves, but also on the upper extremities and the trunk. Most commonly it is associated with diabetes mellitus or renal failure, but it exist also without any metabolic disorders. Kyrle’s disease is characterized by the transepithelial elimination of certain dermal material, this feature being common to all acquired perforating disorders. Various therapies have been reported — including keratolytics (salicylic acid and urea), topical and oral retinoids, followed by psoralen plus Ultraviolet A radiation and laserotherapy.Choroba Kyrlego jest zaliczana do nabytych dermatoz perforujących, w których przebiegu dochodzi do przeznaskórkowej eliminacji uszkodzonego materiału tkankowego. Charakteryzuje się obecnością hiperkeratotycznych grudek i blaszek zlokalizowanych najczęściej na podudziach, ale mogących również zajmować kończyny górne i tułów. Najczęściej towarzyszy cukrzycy i niewydolności nerek wymagającej dializoterapii, zdarza się jednak, że występuje samodzielnie. Opisuje się różne metody terapeutyczne tego schorzenia — od miejscowych preparatów keratolitycznych (mocznik, kwas salicylowy), miejscowych i ogólnych retinoidów, poprzez leczenie psoralenem z naświetlaniem UVA (PUVA) aż po laseroterapię

Zespół nietrzymania barwnika – opis przypadku

Incontinentia pigmenti is a rare genodermatosis, caused by mutations of the NEMO gene, located at X chromosome. It is usually lethal for males, resulting in the observation that most patients are females. Cutaneous manifestations are the most characteristic signs, often occuring in the neonate period and are subdivided into 4 stages. Additionaly, other defects may be associated with the disease including abnormalities of: teeth, eyes, central nervous system, bones. We report the case of a 4-month-old female, presenting skin manifestations of IP just after birth.Zespoł nietrzymania barwnika (IP, incontinentia pigmenti) to rzadko występująca genodermatoza, związana z mutacją w genie NEMO, zlokalizowanym na chromosomie X. U płci męskiej jest zazwyczaj postacią letalną, dlatego dotyczy głownie płci żeńskiej. Zmiany skorne, będące najbardziej charakterystycznym objawem choroby, występują często już w okresie niemowlęcym, obejmując 4 stadia. Dodatkowo zajęte mogą być inne tkanki i układy, na przykład zęby, narząd wzroku, ośrodkowy układ nerwowy, kości. W pracy przedstawiono przypadek niemowlęcia płci żeńskiej, u ktorego zmiany skorne, charakterystyczne dla IP, wystąpiły już przy urodzeniu

Circulating angiostatin serum level in patients with systemic sclerosis

Introduction : Systemic sclerosis (SSc) is achronic connective tissue disease characterized by microangiopathy with inadequate angiogenesis. Angiostatin (AS) is a potent antiangiogenic factor specifically inhibiting proliferation and inducing apoptosis of vascular endothelial cells. Aim : To evaluate the level of angiostatin in the serum of patients with SSc. Material and methods : Serum levels of AS were measured in 20 SSc patients and 12 healthy controls. Results : A statistically significant difference in the serum levels of AS in SSc patients was observed compared to the control group (636.51 vs. 869.20 ng/ml; p = 0.012). Significant correlations between limited and disseminated SSc (lSSc/dSSc) were not found, however, a difference between lSSc and the control group was demonstrated (620.00 vs. 869.20 ng/ml; p = 0.011). The serum level of AS was not associated positively with organ changes caused by SSc. However, a statistically significant lower serum level of AS was observed in patients with SSc and no esophageal (p = 0.008) or pulmonary changes (p = 0.007) compared to the control group. Conclusions : Our results reveal significant differences in AS level in SSc patients compared to the healthy controls, and suggest that a low level of AS may occur as a result of impaired angiogenesis

Rowell’s syndrome – case report and review of the literature

Introduction. Rowell’s syndrome is a rare subtype of subacute lupus erythematosus associated with erythema multiforme-like skin lesions, positive antinuclear antibodies and anti-Ro/La-antibodies. Objective . To present a case of Rowell’s syndrome. Case report . We describe a case of an 82-year-old man who presented with erythema multiforme-like eruption on the upper extremities and trunk and fulfilled the diagnostic criteria of Rowell,s syndrome. Conclusions . Patients with these characteristic clinical and immunological features of Rowell,s syndrome are rarely seen and reported in the literature. The discussion concerning the character of the disease is still ongoing

The use of isotretinoin in low doses and unconventional treatment regimens in different types of acne: a literature review

High effectiveness of isotretinoin treatment for severe types of acne resistant to antibiotics has been widely recognized. However, the recommended doses in conventional therapy, according to consensus of the Polish Dermatological Society, may cause serious adverse effects. Thus, research into less stressful, alternative treatment regimens with the use of low doses of isotretinoin has been carried out. The aim of the paper was to review the selected papers where authors present the results of their studies on different regimens with the use of isotretinoin in low doses in patients with acne, evaluate their efficacy, patient satisfaction, frequency of adverse effects, recurrences and also treatment costs

Acroosteolysis of distal phalanges in patient with systemic sclerosis – case report

Introduction. Acroosteolysis is a recognized, but under-researched and forgotten manifestation of systemic sclerosis (SSc). Objective . To present a case of exuberant acroosteolysis and subcutaneous tissue calcinosis in the course of SSc. Case report. The patient, aged fifty-three with limited systemic sclerosis (lSSc) for 8 years duration, was under dermatological treatment because of ulcerative inflammation of the 2nd and 4th finger of the right hand. Progressive acroosteolysis of the distal phalanx with calcinosis and severe digital ischaemia was diagnosed. Conclusion . There is growing evidence suggesting that acroosteolysis is an important manifestation of SSc due to the correlation with progressive microangiopathy and severity of digital ischaemia