[Skin care and wound management in patients with inherited bullous epidermolysis]. / Njega koze i zbrinjavanje rana u bolesnika s nasljednom buloznom epidermolizom.

Inherited epidermolysis bullosa is a group of diseases characterized by skin/mucous membrane fragility and development of blisters and erosions after insignificant mechanical trauma. It is a multisystemic disease with complications occurring on numerous organs other than the skin. As there is no cure for these diseases, treatment consists of early recognition and therapy of complications, quality wound care and skin protection. Optimal wound treatment depends primarily on the type of the disease, localization and type of wounds. Apart from good skin care, treatment of these patients requires intensive supportive therapy in which various specialists must be involved.

[Treatment of langerhans cell histiocytosis in children]. / Lijecenje histiocitoze langerhansovih stanica u djece.

Langerhans' cell histiocytosis (LCH) is a disease characterised by pathologic accumulation and proliferation of histiocytes, cells from the monocyte-macrophage system, in various tissues and organs. In this retrospective study we analyzed patients charts treated in the Department of pediatric hematology and oncology at the University Hospital Zagreb with the diagnosis of LCH. Twenty-two children were diagnosed between January 1st 1996 and December 31st 2010, and all were treated with chemotherapy. 19 patients survived (86%) and the remaining 3 (14%), all under the age of 2 with multisystem disease, died. At the time of diagnosis 12 children (55%) presented with single-system disease, the most common were bone lesions in 8 children (36%). All children were treated according to protocols LCH-I and LCH -III. Eight children had mild complications of treatment and the disease itself. Diabetes insipidus remains in 4 children.

Correlation between Family History and the Age of Onset of Childhood Acne in Relation to Sex and Type of Acne.

Acne vulgaris is a common chronic skin disorder of the pilosebaceous unit with a wide range of clinical presentations, which depend on the age of onset of acne, sex, family history of acne, and genetic factors, especially the genes affecting keratinization and desquamation. This retrospective study investigated pediatric acne using the patients' past medical history, with patients aged from newborns to 15 years of age. Acne were further stratified by 5 parameters: sex, age, family history, acne type, and localization. Our main aim was to investigate the possible association between selected parameters and the presence or absence of family history of acne. We did not find statistically significant correlation between sex, age of onset, and positive family history of acne. Furthermore, we did not find any association between age of onset and family history according to family members (mother/father/brother or sister). However, we found statistically significant correlation between sex and type of acne. This retrospective analysis of pediatric acne in Croatia did not reveal statistically significant correlation between positive family history and sex, age of onset, and clinical type of acne. In analyzing the correlation between family history and localization of acne, however, we found that the number of patients with acne localized on both the face and trunk and positive family history was statistically significant higher than expected.

Acute hemorrhagic edema of infancy: case report.

Acute hemorrhagic edema of infancy (AHEI) is a benign form of leukocytoclastic vasculitis that typically affects children between 4 and 24 months of age. The etiology remains unknown. The potential triggers of AHEI include preceding bacterial or viral infections, immunizations and drugs. The onset of AHEI is often dramatic with petechiae, ecchymoses, and annular, nummular or targetoid purpuric lesions usually appearing on the extremities, face, or ears. We report on a case of AHEI that occurred after upper respiratory tract infection.

Chronic Recurrent Multifocal Osteomyelitis (CRMO) and Synovitis Acne Pustulosis Hyperostosis Osteitis (SAPHO) Syndrome - Two Presentations of the Same Disease?

The two most common entities among generally rare but under-diagnosed autoinflammatory bone disorders are chronic recurrent multifocal osteomyelitis (CRMO) and synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome. Due to their similarities, many authors consider CRMO to be a subtype of SAPHO syndrome. The aim of this study was to compare clinical, laboratory, and imaging features and outcomes of patients with CRMO and SAPHO. The analysis of the data from 6 children with CRMO (four girls and two boys, age 3.5-14 years) and of 6 children (6 boys, age 13.5-17.5 years) with SAPHO syndrome was performed. The initiating symptoms in all patients with CRMO were bone pain with multifocal bone lesions. There were no skin manifestations. Five out of six patients achieved control with nonsteroidal anti-inflammatory drugs (NSAIDs) and corticosteroids, while one patient required disease-modifying antirheumatic drugs (DMARDs). The initiating symptom in five patients with SAPHO syndrome were severe acne, while in one patient acne occurred two years after the disease onset. Two patients typically developed inflamed sternoclavicular joints and sternum, while the others showed changes affecting other skeletal regions. Three patients achieved control with NSAIDs and corticosteroids, the others required DMARDs and TNFα inhibitors. In comparison with patients with CRMO, patients with SAPHO suffered more frequent and longer lasting exacerbations. In conclusion, CRMO and SAPHO syndrome have an array of common characteristics, but also a number of differences. Nevertheless, further investigation into the etiopathogenesis is required to establish a definite relationship between CRMO and SAPHO.

Differential diagnosis of skin lesions in the diaper area.

Diaper dermatitis is one of the most common skin problems in children. It most commonly presents as an acute irritant contact dermatitis but a great number of dermatoses can manifest with lesions in the diaper area and have to be considered in differential diagnosis. The etiology, clinical picture and differential diagnosis of skin changes in the diaper area are presented.

Differential diagnosis of neonatal and infantile erythroderma.

Neonatal and infantile erythroderma is a diagnostic and therapeutic challenge. Numerous underlying causes have been reported. Etiologic diagnosis of erythroderma is frequently difficult to establish, and is usually delayed, due to the poor specificity of clinical and histopathologic signs. Differential diagnosis of erythroderma is a multi-step procedure that involves clinical assessment, knowledge of any relevant family history and certain laboratory investigations. Immunodeficiency must be inspected in cases of severe erythroderma with alopecia, failure to thrive, infectious complications, or evocative histologic findings. The prognosis is poor with a high mortality rate in immunodeficiency disorders and severe chronic diseases such as Netherton's syndrome.

Treatment of childhood psoriasis.

Psoriasis is a common disease in children and adolescents. Because of the chronic course of the disease, appropriate choice of therapy in particular stage of the disease, so-called rotation therapy, is of paramount importance. This article provides a review of therapeutic options for childhood psoriasis. Local therapy for psoriasis in children consists of corticosteroid preparations, calcipotriol, tars and dithranol, local retinoids, and local immunomodulators. Phototherapy (narrow band UVB, photochemotherapy PUVA baths) is now a part of psoriasis therapy in children. Systemic therapy retinoids (acitretin) methotrexate, cyclosporine is only used in severe forms of the disease such as erythrodermic, pustular and arthritic psoriasis. All these therapeutic options can be used as monotherapy or in various combinations.

Inflammatory epidermolysis bullosa acquisita in a 4-year-old girl.

This study presents a case of linear immunoglobulin A dermatosis-like epidermolysis bullosa acquisita in a 4-year-old girl showing rapid, widespread and inflammatory skin lesions. The diagnosis was confirmed by histopathology, direct and indirect immunofluorescence, various immunoblotting analyses and enzyme-linked immunosorbent assays. Despite the severe clinical manifestations, the disease was successfully controlled by combination therapy of oral prednisolone and dapsone.

Inherited epidermolysis bullosa - the spectrum of complications.

Epidermolysis bullosa is a group of inherited diseases that are characterized by skin and mucosal fragility and blister formation. A wide variety of extracutaneous manifestations can develop as well as various complications of the disease such as severe anemia, growth retardation, esophageal stenosis, mutilating deformities of hands and feet, glomerulonephritis leading to chronic renal failure, and many others. One of the most important and often occurring complications is the development of cutaneous squamous cell carcinomas that grow and metastasize quickly. The objective of this paper is to give dermatologists a review of major complications encountered in patients with epidermolysis bullosa. Since these complications occur so often and can be considered to be part of the clinical picture, it is mandatory to develop a multidisciplinary well-educated team involved in follow-up and treatment of these patients.