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  • Author: Mirjana Gajić-Veljić x
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Open access

Branislav Lekić, Mirjana Gajić-Veljić and Miloš Nikolić

Abstract

Wells syndrome (WS) is a rare inflammatory skin disease of unknown etiology. Possible triggers for WS include insect bites/stings, infections, medications, malignancies, and vaccination. Most cases have been reported in adults, but WS may also occur in children.

We report a case of idiopathic WS in a 12-year-old boy, who presented with pruritic papulonodular and granuloma annulare-like lesions on his legs. The patient had an excellent response to topical and systemic corticosteroids.

WS may present as plaque, granuloma annulare-like, urticaria-like, papulovesicular, bullous, papulonodular, or fixed drug eruption-like lesions. Erythematous annular lesions are most common in adults, while plaques are mostly found in children. The histopathologic features are dynamic, starting with dermal edema and infiltration of eosinophils, then flame figures develop, and finishing with the appearance of histiocytes and giant cells.

Our patient represents a rare pediatric case with granuloma annulare-like WS syndrome.

Open access

Branislav Lekić, Mirjana Gajić-Veljić, Svetlana Popadić and Miloš Nikolić

Abstract

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.

Open access

Jovan Lalošević, Mirjana Gajić-Veljić, Jelena Stojković-Filipović, Martina Bosić and Miloš Nikolić

Abstract

Pachyonychia congenita (PC) is a rare inherited disorder of keratinization characterized by hypertrophic nail dystrophy, painful palmoplantar blisters, cysts, follicular hyperkeratosis and oral leukokeratosis. These pathological clinical features are resulting from mutations in keratin proteins including KRT6A, KRT6B, KRT6C, KRT16, and KRT17. We present a 6-year-old girl with hypertrophic nail dystrophy, follicular hyperkeratosis, circumscribed plantar keratoderma and oral leukokeratosis. The features were consistent with the diagnosis of PC. The patient has been registered in the International Pachyonychia Congenita Research Registry (IPCRR) and is waiting for a detailed genetic analysis. The IPCRR has contributed to publication of numerous papers which emphasized the importance of the mutation type affecting various clinical presentations of PC. Based on recent data, a new classification system has been developed for PC, and it is gradually replacing the earlier classifications. It is based almost exclusively on the mutated genes. In this report we have raised the hypothesis that distinctive clinical features may be highly suggestive of a specific keratin mutation.

Open access

Svetlana Popadić, Mirjana Gajić-Veljić, Biljana Marenović and Miloš Nikolić

Abstract

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.

Open access

Branislav Lekić, Mirjana Gajić-Veljić, Dušan Škiljević, Svetlana Popadić and Ljiljana Medenica

Abstract

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.

Open access

Jovan Lalošević, Branislav Lekić, Mirjana Gajić-Veljić, Dušan Škiljević, Katarina Đukić and Ljiljana Medenica

Abstract

Verrucous carcinoma (VC) is a rare variant of a well-differentiated squamous cell carcinoma (SCC) with a low grade of malignancy. Epithelioma cuniculatum (EC) is a subtype of VC, usually found on the sole of the foot.

Two patients, a 55-year-old female, and a 77-year-old male, with VC were treated at the Clinic of Dermatology and Venereology, Clinical Center of Serbia, from 2002 to 2011. Both patients presented with a tumor on the foot. Incisional biopsies showed a well differentiated squamous cell carcinoma. Foot x-rays showed bone involvement in one case. One patient underwent surgical amputation of the lower extremity, while the other had a partial amputation of the affected foot.

In the initial stage of the disease, it is difficult to distinguish pseudoepitheliomatous hyperplasia from verrucous carcinoma. The superficial biopsy of EC lesion may mislead to a histopathological diagnosis of warts or condylomas. Multiple deep biopsies are necessary for accurate and timely diagnosis of verrucous carcinoma.

Open access

Miloš M. Nikolić, Zoran Stamenović, Mirjana Gajić-Veljić and Jelica Vukićević

Abstract

Tinea capitis (TC) is a worldwide health problem and a specific therapeutic challenge. The objective of this study was to establish the causative agents and clinical types and to present management options for TC. From 1993 to 2002, we treated 354 children (61% were boys) with TC. The data for the period 1998-2002, were analyzed in detail. After clinical and Wood's light examination, specimens were directly microscopically analyzed and cultivated. Superficial TC was diagnozed in 230 (65%), and kerion in 124 patients (35%), respectively. Of 51 kerion patients, 3 children (6%) had erythema nodosum. Griseofulvin was given at a dose of 15 to 25 mg/kg (average: 18 mg/kg). A 100% cure rate was achieved. In the period 1998-2002, M. audouinii was predominantly found in superficial TC. Oral griseofulvin therapy should be accompanied by shaving the hair from the scalp and topical imidazole creams, which provides 100% cure rate of superficial TC (in 6.5 weeks) and of kerion (in 5.4 weeks).

Open access

Svetlana Popadić, Mirjana Gajić-Veljić, Sonja Prćić, Željko Mijušković, Dragan Jovanović, Lidija Kandolf-Sekulović and Miloš Nikolić