Exceptionally large juvenile xanthogranuloma – a case report

Juvenile xanthogranuloma (JXG) is a rare, benign skin lesion pathologically classified as a non-Langerhans cell histiocytosis. The lesions appear within the first year of life in 75% of patients, predominantly on the head or neck, growing up to 5mm in size. While the etiology is mostly infectious, it can also be caused by genetic variants. In most patients, the condition has an easy course and resolution. Histopathological features include a histiocytic invasion of the superficial dermis, with additional eosinophils, lymphocytes and plasma cells. The lesion typically stains with anti-CD4, anti-XIIIa and CD68 markers

Digital Clubbing in Hereditary Hemorrhagic Telangiectasia/Juvenile Polyposis Syndrome

Hereditary hemorrhagic telangiectasia (HHT) (Osler- Weber-Rendu Syndrome) is a rare autosomal dominant vascular disorder characterized by the presence of multiple arteriovenous malformations (AVMs) and recurrent bleeding episodes. The diagnosis is based on the Curacao criteria: (i) spontaneous recurrent epistaxis, (ii) mucocutaneous telangiectasia, (iii) AVMs of visceral organs, and (iv) first degree relatives with a similar condition (1). Due to a common genetic pathway and SMAD4 gene mutation, juvenile polyposis syndrome (JPS) may coexist with HHT (2). The disease burden is high in overlapping HHT/JPS, but digital clubbing may be the only physical finding. Continuous meticulous management may improve the quality of life and reduce the risk of complication

Vitamin D – The True and the False about Vitamin D

Vitamin D has a positive impact on our overall health. Also there are a few conditions with strong evidence for a protective effect of vitamin D, such as bone diseases, internal cancers, multiple sclerosis, hypertension and DM type 1. Skin is the major source of vitamin D through the action of UVB light on keratinocytes, although the biologically active form of vitamin D is not exclusively produced in the kidney, but also in prostate, colon, skin and osteoblast where it acts as an autocrine or paracrine hormone. In the past decade raising incidence of skin cancers, especially melanoma and its connection with sun exposure lead to a sun protection policies and practices as part of the public health campaigns. The question is how much solar UV exposure is adequate to maintain the balance between the risk and the benefit. We as dermatologists have to raise public awareness of the potential health effects from excessive exposure to UV radiation but also we have to be aware that adequate blood level of vitamin D is necessary for optimal health. So future recommendation on sun protection have to balance between the risk and benefits of sun exposure, as well as to promote vitamin D supplementation as a safe alternatives in high risk population

Treatment Options for Pediatric Psoriasis

Psoriasis is a multifactorial inflammatory papulosquamous disease affecting 0.5% to 2% of paediatric population. Paediatric psoriasis, presenting similarly to adult psoriasis, significantly reduces patients’ quality of life, often requiring individualized treatment approach for each patient. Combination and rotational therapy are helpful in reducing toxicity and maximizing efficacy. Patients with mild and limited disease respond well to topical treatment with steroids or vitamin D analogues, unlike moderate and severe psoriasis where sufficient remission is rarely achieved. Therefore phototherapy, systemic immunomodulators, or biologic agents are next line of treatment to be considered. There is a limited data available on the use and long-term safety of biologics in the paediatric population. Biologic agents must be administered by experienced dermatologists, only in patients with moderate-to-severe plaque psoriasis, intolerant or refractory to other systemic conventional disease-modifying treatment or phototherapy, or if those agents are contraindicated. Psoriasis is a multifactorial inflammatory papulosquamous disease affecting 0.5% to 2% of paediatric population. Paediatric psoriasis, presenting similarly to adult psoriasis, significantly reduces patients’ quality of life, often requiring individualized treatment approach for each patient. Combination and rotational therapy are helpful in reducing toxicity and maximizing efficacy. Patients with mild and limited disease respond well to topical treatment with steroids or vitamin D analogues, unlike moderate and severe psoriasis where sufficient remission is rarely achieved. Therefore phototherapy, systemic immunomodulators, or biologic agents are next line of treatment to be considered. There is a limited data available on the use and long-term safety of biologics in the paediatric population. Biologic agents must be administered by experienced dermatologists, only in patients with moderate-to-severe plaque psoriasis, intolerant or refractory to other systemic conventional disease-modifying treatment or phototherapy, or if those agents are contraindicated. </div

The Mechanisms of UV Radiation in the Development of Malignant Melanoma

The sunlight was one of the first agents recognized to be carcinogenic for humans. There is convincing evidence from epidemiologic studies that exposure to solar radiation is the major cause of cutaneous melanoma in light-pigmented populations and plays a role in the increasing incidence of this malignancy. The molecular mechanisms by which UV radiation exerts its varied effects are not completely understood, however, it is considered that UVA and UVB are equally critical players in melanoma formation. Whereas UVA can indirectly damage DNA through the formation of reactive oxygen radicals, UVB can directly damage DNA causing the apoptosis of keratinocytes by forming the sunburn cells. Besides action through mutations in critical regulatory genes, UV radiation may promote cancer through indirect mechanisms, e.g. immunosuppression and dysregulation of growth factors. The carcinogenic process probably involves multiple sequential steps, some, but not all of which involve alterations in DNA structure

Phototoxic and Photoallergic Skin Reactions

Indirect action of sun together with different exogenous agents (systemic medications and topically applied compounds) sometimes may result in phototoxicic and photoallergic reactions. Drug-induced photosensitivity reactions refer to the development of cutaneous disease as a result of the combined effects of a drug and light (mostly spectrum within the UVA and visible light range or UVB range). The aim of the review was to show the prominent features of phototoxic and photoallergic reactions, which occur in sun-exposed areas, including face, neck, hands and forearms. Phototoxic reactions are significantly more common than photoallergic reactions and mosty resemble to exaggerated sunburn. Photoallergic reactions appear only in a minority of individuals and resemble allergic contact dermatitis on sun-exposed areas, although sometimes may extend into covered areas. Generally, the physical examination and a positive patient’s history of photosensitivity reactions on substances are of great importance for the diagnostics. The treatment of these reactions includes identification and avoidance of offending agent and application of anti-inflammatory dressings, ointments and corticosteroids

Photosensitivity Skin Disorders in Childhood

Photosensitivity in childhood is caused by a diverse group of diseases. A specific sensitivity of a child’s skin to ultraviolet light is often the first manifestation or a clinical symptom of photodermatosis. It might indicate a serious underlying systemic disease such as lupus erythematosus or dermatomyositis, or a rare group of genetic skin disorders like Xeroderma pigmentosum, Cockayne syndrome, Trichothyodystrophy, Bloom syndrome, Rothmund-Thomson and Kindler syndrome as well as metabolic disorders and cutaneous porphyria. Photosensitivity secondary to topical or systemic agents may also cause photosensitivity in children. Early recognition and prompt diagnosis may prevent complications associated with unprotected exposure to sunlight and avoid actinic injuries that can leed to malignant skin changes

Utjecaj izraženosti E-kadherina i N-kadherina u primarnom melanomu i metastazama u limfnim čvorovima na prognozu bolesti [Expression of E-cadherin and N-cadherin in primary cutaneous melanoma and nodal metastases as prognostic markers]

Development of melanoma is a complex process influenced by growth factors and cytokines, as well as, interactions between malignant cells and benign surrounding cells. Cadherins are molecules involved in the intercellular communication and play a critical role in tumor formation and progression. The aims of this study were to analyze E- and N-cadherin expression in cutaneous malignant melanoma and its metastases in regional lymph nodes, to compare E- and N-cadherin expression with traditional prognostic factors and to evaluate their relevance as prognostic markers for survival of melanoma patients. The expression was determined using monoclonal antibodies to E- and Ncadherin and calculating the intensity of reaction and percentage of positive cells. Significant difference in the expression of E-cadherin between melanocytes and melanoma cells, as well as primary cutaneous melanoma cells and metastasis was determined. E-cadherin expression was lower in melanoma compared to melanocytes (p<0,001), and in metastasis of melanoma compared to primary tumor (p<0,001). The results for N-cadherin indicated stronger expression in metastasis compared to primary melanoma (p<0,001), and in primary melanoma compared to melanocytes (p=0,008). There was no difference in expression of Ncadherin in primary melanoma with or without metastasis. Strong N-cadherin expression indicated poor outcome (p=0,010). Low E-cadherin expression was related to higher reccurence rate of melanoma (p=0,032). The results in this study confirmed a switch in E- and N-cadherin expression during melanoma developement and invasion. N-cadherin is a potential prognostic factor in patients with melanoma metastasis and poor outcome and could be used in selecting more aggresive treatment options for patient with melanoma metastasis