Svetlana Popadić, Zorica Ramić, Ljiljana Medenica, Vera Pravica and Dušan Popadić
We examined single nucleotide rs11465553 polymorphism in the interleukin-17F gene causing valine to isoleucine substitution at the position 155 in the third exon of IL17F gene in Serbian patients with psoriasis and healthy blood donors. For the first time we found rs11465553 G (valine) and A (isoleucine) allele frequency in healthy Serbian population and in psoriasis patients, but without statistically significant difference between the two groups.
Svetlana Popadić, Zorica Ramić, Ljiljana Medenica, Marija Mostarica-Stojkovic and Dušan Popadić
Numerous clinical studies demonstrate benefits of dietary supplementation with fish oils in autoimmune diseases and other inflammatory diseases such as psoriasis, multiple sclerosis, systemic lupus erythematodes and so on. Docosahexaenoic acid (DHA) is an omega-3 fatty acid which is abundantly found in fish oil. In the present study we investigated effects of DHA on proliferation of human keratinocytes established from skin of seven adult donors, cultivated in growth medium that allows optimal cell proliferation. We found a dose-dependent inhibition of cell proliferation when keratinocytes were incubated with 6.25, 12.5 and 25 -μM of DHA. Inhibition of proliferative capacity considerably varied in keratinocyte cultures derived from different donors, particularly when incubated with the lowest concentration of the assessed substance. Lactate dehydrogenase-release assay excluded necrosis of cultivated keratinocytes as a cause of decreased proliferation. Our results suggest that DHA may potentially be used as a routine adjuvant therapy, with classical therapy of inflammatory hyperproliferative skin diseases.
In Europe, only 1% of all patients with lichen planus are children. We report a case of lichen planus in a 5-year-old boy with blisters developing on papules. There was no history of Hepatitis B virus vaccination in the past 12 months. Routine laboratory analyses showed no abnormalities. Hepatitis B surface antigen and anti-Hepatitis C virus antibodies were negative. Our patient had disseminated and coalesced papules on the trunk and extremities with vesicles and bullae on the hands and feet. Histopathology confirmed the diagnosis of lichen planus and bullous lichen planus. Negative direct immunofluorscence test excluded lichen planus pemphigoides. The boy was treated with prednisone 1 mg/kg/ day (the dose was gradually tapered and discontinued over the next 1.5 month), ultraviolet B phototherapy, fluocinolon acetonide, and topical pimecrolimus 1% cream till complete remission after 2.5 months. Bullous form of lichen planus is seen in 1-16% of all children with lichen planus. Two months upon the completion of therapy, there were no signs of relapse. In our case, short course of systemic corticosteroids and ultraviolet B phototherapy have been safe and effective. This was the only pediatric case of bullous lichen planus treated in our Clinic in the last 20 years. Long-term prognosis of childhood lichen planus is not predictable, and there is no consensus regarding the treatment of childhood lichen planus.
Isidora Sbutega, Svetlana Popadić, Petar Ivanovski, Biljana Arsov and Miloš Nikolić
Peutz-Jegher’s syndrome is a hereditary disorder characterized by melanocytic macules on the lips and buccal mucosa and multiple gastrointestinal hamartomatous polyps. It is caused by a mutation localized on chromosome 19p13.3. Skin and mucosal pigmentation may be present at birth and usually occur in early childhood, but occasionally may develop later. It is associated with an increased risk of malignancy for gastrointestinal carcinoma and also for breast, ovarian, testiscular, pancreatic and gallbladder cancer. We report a 12-year-old girl who presented with disseminated petty yellowish macules on the bridge of her nose, numerous brown to bluish black macules on her lips and buccal mucosa. Mucocutaneous pigmentation has been present from the age of five, with a negative family history. In our patient, esophageal endoscopy was normal, while the endoscopy of stomach and duodenum revealed multiple diminutive polyps. After clinical evaluation, there were no indications for therapy. Further follow up was suggested. Continuous surveillance is very important for patients with Peutz-Jegher’s syndrome in order to reduce risks of cancer and prevent other morbidity and mortality.
Branislav Lekić, Mirjana Gajić-Veljić, Svetlana Popadić and Miloš Nikolić
IgA pemphigus (IGAP) is a rare autoimmune bullous disease characterized by IgA deposits on keratinocyte cell surfaces. The IGAP is classified into: 1) subcorneal pustular dermatosis (SPD) type, and 2) intraepidermal neutrophilic (IEN) IgA dermatosis type. So far, only 9 children with IGAP have been described in the literature, of whom only 3 with SPD type. We report a 3-year-old boy with SPD type of IGAP. Clinically, he presented with pruritic vesicles, pustules and erosions on the face, trunk, groin area, and extremities. Histopathology showed subcorneal pustules containing a few acantholytic cells. Direct immunofluorescence (DIF) test of Tzanck smear showed intercellular IgA deposits on the surface of the groups of epidermal cells. Oral dapsone and prednisone induced remission after two weeks; the treatment was discontinued 11 months later, and complete remission was achieved during 19 months without any treatment. Direct immunofluorescence of Tzanck smear is a simple, sensitive, rapid and non-aggressive test, very suitable for the diagnosis of IGAP in children.
Iva Maširević, Biljana Marenović, Svetlana Popadić and Miloš Nikolić
Hydroa vacciniforme is a rare, idiopathic, chronic photodermatosis that usually begins in childhood and resolves spontaneously in early adulthood. It is characterized by appearance of vesicles on sun-exposed areas. The vesicles crust and heal within one to six weeks, leaving vacciniform/varioliform scars. We report an 11-year-old boy with a 5-year history of recurrent blisters on sun-exposed areas that deteriorated each summer. He was treated with antimalarials, topical photoprotective agents, sun avoidance, dietary fish oil and supplementary doses of vitamin D3. Strict adherence to the regimen resulted in remission.
Igor Kapetanović, Vesna Reljić, Martina Bosić and Svetlana Popadić
Basal cell carcinoma is the most common form of cutaneous cancer. In majority of cases it is locally invasive with slow growth, ranging in size from a couple of milimeters to a couple of centimeters and located primarily on sun-exposed regions. Giant basal cell carcinoma, defined as a tumor that is larger than 5 cm in diameter, is a very rare type of cutaneous malignancy accounting for 0.5-1% of all basal cell carcinomas. We present a case of a 74-year-old man with a 17 x 14 cm giant basal cell carcinoma in the right supraclavicular region. Detailed history revealed that the lesion had started as a papule 15 years before presentation. Despite its growth, the lesion was neglected until admission. Histological examination of skin lesion confirmed superficial and focally infiltrative types of basal cell carcinoma. Electron radiotherapy was administered with 54 Gy total dose delivered in 20 daily fractions which resulted in healing of lesions and adequate response. Thus, definitive radiotherapy can be just as effective as excision when the criteria are met.
Svetlana Popadić, Mirjana Gajić-Veljić, Biljana Marenović and Miloš Nikolić
Mastocytosis refers to a group of diseases characterized by a clonal proliferation and accumulation of mast cells in one or more tissues/organs with different clinical presentations. In children, limited cutaneous forms of mastocytosis are rather frequent, while systemic mastocytosis is rare. The diagnosis of cutaneous mastocytosis is based on clinical findings and histopathology. We present a patient who developed skin lesions at the age of 18 months. Clinical findings, confirmed by histopathology, were consistent with diffuse cutaneous mastocytosis. The follow-up period was 7 years. The treatment included oral antihistamines in combination with mast cell stabilizers, mild topical steroids and avoidance of friction. During the follow-up period, there were no signs of systemic involvement, and the quality of life was preserved, despite the large surface of affected skin. This case report should increase the awareness and knowledge of clinicians about this rare form of cutaneous mastocytosis in the pediatric population.
Branislav Lekić, Mirjana Gajić-Veljić, Dušan Škiljević, Svetlana Popadić and Ljiljana Medenica
Apocrine adenocarcinoma is a rare form of sweat gland malignancy mostly affecting adults without evident prevalence for sex or race. Clinically, it presents as a single or a multi-nodular mass or plaque in the axillary or anogenital region, with no additional symptoms. This neoplasm is locally invasive, grows slowly and has an ability to metastasize to visceral organs, regional lymph nodes and bones. We report a case of infiltrating apocrine adenocarcinoma on the scrotum and pubic area with extramammary pagetoid spread into the groin. The immunohistological staining patterns suggested that both extramammary Paget’s disease and apocrine adenocarcinoma derived from the apocrine gland, because the tumor cells were positive for cytokeratin (CK) 7 and gross cystic disease fluid protein-15 (GCDFP-15), but negative for CK20 and prostate-specific antigen (PSA). The results of this case study will facilitate the understanding of this malignant tumor.
Svetlana Popadić, Andreja Vujanac, Biljana Arsov, Petar Ivanovski and Miloš Nikolić
Ectodermal dysplasias are a large group of disorders characterized by developmental dystrophies of one or more ectodermal structures. Hypohidrotic ectodermal dysplasia is a rare genodermatosis associated with abnormal development of sweat glands, teeth, and hair. Its incidence is 1:100.000 newborns. The full expression of X-recessive forms are only seen in males, while female heterozygotes are moderately or very slightly affected. The disease is characterized by sparse hair, oligodontia, and reduced or absent sweeting, light hair, distinctive facial features, palmoplantar keratoderma.
We report an 11-year-old boy with hypohidrotic ectodermal dysplasia. Despite extensive skin, teeth and hair manifestations, his physical and psychomotor growth and development were normal.