Pyogenic Granuloma of the Proximal Part of a Nail Unit in a Child.

Dear Editor, We present a case of proximal pyogenic granuloma in 4-year-old child. The patient presented to our Department due to a fast-growing lesion on the proximal part of the nail unit. The lesion had appeared over several weeks, and it was extremely painful for the child. On the day of the 1st visit, the lesion was not bleeding but was very painful during examination and photo-documentation. Clinically, it presented as an exogenous tumoral lesion of the proximal 1/3 of the nail, partially exulcerated with one part exhibiting coagulated hemorrhage and with uneven coloration (Figure 1). The lesion was not sharply demarcated. Dermoscopically, the majority of the lesion presented an unspecific dermoscopic structure, orange background color, and matched the criteria for a vascular lesion: few unspecific vessels and hemorrhage. The "sticky fiber" sign was also present (Figure 2). Since the lesion was fast-growing and due to the unspecific dermoscopic appearance, the child was referred to a pediatric surgeon and a complete excisional biopsy of the lesion was performed. The dermoscopy of pyogenic granuloma has been already described (1). The histology report confirmed pyogenic granuloma. Pyogenic granulomas of the nail unit are not a common finding, but our case confirms that even this location can be site of this type of benign lesion. It more commonly found in the periungual region and can be expected due to adverse effects of different kinds of systemic therapies. However, due to differential diagnosis that includes different types of tumors occurring at the nail unit, most importantly amelanotic melanoma and SCC, it is suggested to excise or take a biopsy of this type of lesion to be able to exclude aggressive tumor types, which are very rare but not impossible the in pediatric population (2). In cases of unquestionable diagnosis, several local treatments are available. Since the lesion presented a destructive nature in our case, we decided to perform excisional biopsy followed by histology, which in our case was both a diagnostic and therapeutic procedure.

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.

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.

Association of Generalized Psoriasis and Mixed Glomerulonephritis in a 10-year-old Girl.

Generalized psoriasis and renal function disorder were previously described in sporadic adult cases, revealing a new entity - psoriatic nephropathy. So far there have been only two cases describing this association in children. We present and discuss a case of 10-year-old girl with the unique biopsy findings of double glomerulonephritis associated with the simultaneous onset of generalized psoriasis.

Kerion Celsi due to Microsporum canis with a Dermatophytid Reaction.

Microsporum (M.) canis is the most common fungus to cause tinea capitis in Europe, especially in the Mediterranean region and South and Central Europe. Fungal scalp infections caused by M. canis tend to be non-inflammatory. Recently, a growing number of cases of tinea capitis characterized by inflammatory infection caused by M. canis and M. gypseum have been registered. We present a case of highly inflammatory tinea capitis, also known as kerion celsi, caused by M. canis in a 6-year-old-patient. Scalp infections due to M. canis are a growing problem in dermatological practice. Changes in epidemiology, etiology, and clinical patterns of fungal infections due to M. canis are significant. Greater awareness of this problem is needed in order to establish proper diagnosis and successful treatment strategy for these patients.

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.

[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.

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.

[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.

Subcutaneous panniculitis-like T-cell lymphoma in a 19 month-old boy: a case report.

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare type of T-cell lymphoma of CD3+CD8+ phenotype characterized by deep-seated skin nodules or plaques mimicking panniculitis, a result of neoplastic lymphocytes infiltrating the subcutaneous fatty tissue. We present a case of a 19-month year old boy with SPTCL diagnosed and successfully treated in our institution. Disease first presented with symptoms of high fever and painful erythematous nodule located below the umbilicus. Later on the infiltrates appeared on the face, legs, arms and the back of the body. As the most decisive in obtaining the diagnosis, skin biopsy showed atypical, small to medium-sized lymphatic cells infiltrating the deeper dermal layers as well as the subcutaneous adipous tissue surrounding the adipocytes. Immunohystochemical analysis showed neoplastic lymphocytes positive for CD2, CD3, CD5, CD7, CD8, Tia-1, granzyme B and perforine, and negative for CD20, CD34, TDT and CD56. No infiltration of blood vessels or epidermis was evident. Specific T-cell lymphomas protocol (EURO-LB 02) was then initiated which resulted with rapid regression of all general and local symptoms. The treatment was completed according to schedule and the child is now, 24 months after the initiation of the treatment, in complete remission.

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.

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.

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.

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.

Phototherapy in pediatric patients.

The treatment of children with psoriasis, atopic dermatitis (AD), pityriasis lichenoides, and scleroderma poses a therapeutic problem because all therapeutic options are associated with numerous side effects. Therefore ultraviolet A and B (UVA and UVB) phototherapy is presented as a possible alternative to some of these therapies, primarily topical and systemic corticosteroids, in children. Our results in treating children with phototherapy and psoralen plus UVA (PUVA) bath phototherapy over the past 5 years are reported. UVB therapy (TL01) was used in 20 psoriatic children (6 boys, 14 girls; ages 6-14 years) during the stage of disease exacerbation and in 9 children (3 boys, 6 girls; ages 8-16 years) with pityriasis lichenoides. Combined UVA/UVB phototherapy was applied in 21 AD children (7 boys, 14 girls; ages 4-15 years). Photochemotherapy with local application of a PUVA bath was used in six children (2 boys, 4 girls; ages 9-16 years) with circumscribed scleroderma and in one girl with systemic scleroderma. All children received short courses of phototherapy with either no maintenance or short maintenance. All three therapeutic protocols resulted in a certain degree of improvement in most of the study patients. None of the patients exhibited any early phototherapy side effects. We conclude that phototherapy and PUVA bath are valuable and safe therapeutic options for selected children who do not respond to other treatments.