Browsing by Author "Gajic-Veljic, Mirjana (24767639800)"

Annular lichenoid dermatitis of youth (ALDY), first described in 2003, represents an uncommon entity whose etiopathogenesis is still debated. Futhermore, the optimal treatment for ALDY is yet to be established. We report a 9-year-old girl who presented with annular and oval erythematous lesions mostly on her trunk, with several lesions on the neck, groin, flanks, and upper extremities. The lesions had histological and immunohistochemical features characteristic for ALDY. Treatment with H1-antihistamines, topical corticosteroid, and UVB therapy was unsuccessful, while systemic treatment with cyclosporine induced complete remission. © 2020 Wiley Periodicals LLC.

There is no universally accepted treatment for severe pediatric alopecia areata (AA). This prospective study comprised 73 patients (aged 1–18 years) with severe AA (>30% of scalp surface area): 37 received 1-day intravenous dexamethasone pulses (1-DP) and 36 received 3-day pulses (3-DP), monthly, for 6–12 months. Also, all patients applied topical clobetasol propionate under plastic wrap occlusion. Patients achieving >50% regrowth were considered good responders (GR). All patients reached short term, while 65/73 were available for the long-term follow-up (mean 33.3 ± 15.3 vs. 27.7 ± 14.3 months, 1-DP and 3-DP, respectively). Relapses during therapy were more frequent in 1-DP group. 3-DP patients were more frequently GR in comparison with 1-DP. 3-DP patients with disease duration <6 months had better outcomes. Patients without Hashimoto thyroiditis (HT) had 9.8-fold higher chance of being GR in comparison with HT patients. The best results were achieved in AA plurifocalis (AAP). No patient had severe short-term side-effects. At the long-term follow-up, 67% of 3-DP patients had stable results. Only 14.2% AAP patients experienced relapses. Patients had no long-term side-effects. 3-DP were more efficacious than 1-DP. Short disease duration and no HT were good prognostic factors. 3-DP protocol is well-tolerated, with beneficial effects and long-lasting results in severe pediatric AA. © 2019 Wiley Periodicals, Inc.

There are no widely accepted therapy protocols for severe alopecia areata (AA). We treated 65 children/adolescents with AA affecting >30% of scalp. Fourty-three percent of patients had AA plurifocalis (AAP). Fifty-seven percent had AA subtotalis (AAS), AAP+ophiasis (AAP+OPH), and alopecia totalis/universalis (AT/AU). Long-term follow-up (median 96 months) data were available for 69% of patients. Oral dexamethasone (prednisolone 5 mg/kg equivalent) was given once in 4 weeks. Patients received 6, 9, or 12 pulses. Clobetasol propionate 0.05% ointment under plastic wrap occlusion was applied 6 days a week. Hair growth was assessed on a scale ranging 0-100% of regrowth in individual AA lesions. Regrowth >50% was considered good response. Six to twelve months months after the therapy, 56.9% of patients had >75% of hair regrowth. In AAP, 65.5% had complete regrowth. 61.5% of all patients were considered good responders. Significantly, higher percentage of good responders was found in AA lasting ≤12 months. No patients had serious side effects. There was no change in stability of the hair status at the long-term follow-up. Most AA patients had beneficial effects with this protocol. Best results were in AAP and AAP+OPH. Combined topical and oral pulse corticosteroid therapy of AA in children shows long-lasting results, without serious side effects. © 2015 Wiley Periodicals, Inc.

Granulomatosis with polyangiitis (GPA), also known as Wegener's granulomatosis, is a rare disease in childhood. Of 39 GPA patients that we diagnosed during a 20-year period, only 3 (7.7%) were younger than 18 years. We report the course of GPA in three girls whose disease started at the ages of 16, 11, and 6 years. All had cutaneous manifestations: the first had necrotizing vasculitis, the second had palpable purpura, and the third had right upper-eyelid edema and infiltration and proptosis caused by extraocular pseudotumor, initially histologically misdiagnosed as orbital immunoglobulin G4 (IgG4)-related disease. Unlike with skin vasculitis and glomerulonephritis, upper-airway and orbital inflammation were resistant to immunosuppressive therapy. Our report emphasizes that children presenting with cutaneous vasculitis, chronic eyelid swelling, sinusitis, or hoarseness should be tested for antineutrophil cytoplasmic antibodies. We emphasize that the upper-eyelid edema and infiltration, with histologic characteristics of orbital IgG4-related disease, may be the initial presentation of localized GPA in children, a feature that, until now, has been described only in adults. © 2012 Wiley Periodicals, Inc.

Objective. To study the role of deoxyribonuclease (DNase) I activity and ANCA in propylthiouracil (PTU)-induced lupus-like syndrome (LLS).Methods. We compared 36 SLE patients with 17 PTU-induced LLS patients diagnosed from 2008 to 2014. We studied ANCA profile (MPO, PR3, lactoferrin, CTG, elastase, bactericidal/permeability-increasing protein), anti-dsDNA, anti-ENA, anti-nucleosome, anti-histone, anti-C1q, anti-aCL, complement components, cryoglobulins and serum DNase I activity. Healthy persons and patients without LLS treated with PTU comprised the control groups. Twelve LLS patients were serologically and clinically followed for 4.1 (s.d. 2.0) years.Results. PTU-induced LLS patients less frequently had arthritis, renal and neurological manifestations, but more frequently had fever, purpura, urticarial-like vasculitis and ulceration (P < 0.01). PTU-induced LLS patients more frequently had polyspecific ANCA (anti-MPO, anti-elastase and anti-PR3 were most commonly detected) (P < 0.01). SLE patients more frequently had anti-dsDNA, anti-ENA, anti-nucleosome, anti-C1q (P < 0.01) and anti-histone antibodies (P < 0.05). PTU-induced LLS patients had lower DNase I activity than SLE patients and controls (P < 0.01). Discontinuation of PTU increased DNase I activity, although it did not reach the levels of controls (P < 0.01). After remission, MPO-ANCA decreased (P < 0.01), but persisted for a long time.Conclusion. PTU, as a trigger, and low DNase I activity, as a predisposing factor, may lead to LLS. Polyspecific ANCAs are useful markers for differentiating SLE from PTU-induced LLS. Low DNase I activity might be an important prognostic biomarker for PTU-induced LLS. Monitoring of ANCA and DNase I activity may prevent long-lasting exposure to causal drugs, unnecessary immunosuppressive therapy and severe complications of LLS. © The Author 2015. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved.

Orofacial granulomatosis, a rare disease in childhood, is characterized by orofacial swelling in the absence of systemic disease. We report the case of a 12-year-old girl with asymptomatic erythematous infiltration of her upper lip, cheeks, and chin that had persisted for more than 2 years; biopsy confirmed granuloma formation. Because a large area was affected, intralesional corticosteroids were inappropriate and six cycles of 3-day intravenous pulse corticosteroid therapy (dexamethasone 1.5mg/kg), repeated once after 4 weeks, was given. Our patient also received oral chloroquine and topical emollients. At the end of the sixth pulse cycle, the infiltration had completely resolved, leaving slight residual erythema. © 2017 Wiley Periodicals, Inc.

Background: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening diseases that are most frequently caused by drugs. Objectives: The purpose of this study was to summarize 20 years of experience with SJS and TEN in the largest dermatology clinic in Serbia. Methods: The study included 38 patients treated during the period 1993-2012. The patients were classified into three groups according to whether they were diagnosed with SJS, a condition representing an overlap of SJS and TEN (SJS/TEN), or TEN. Patients with TEN were also divided into three groups according to the modality of therapy: supportive therapy (ST) only (n = 3); ST plus systemic corticosteroids (SC) (n = 8); and ST plus SC plus IV immunoglobulins (IVIG) (n = 6). Results: The study population included 13 SJS patients, eight SJS/TEN patients, and 17 TEN patients. The disease had started at a mean ± standard deviation (SD) of 7.1 ± 3.5 days after the commencement of treatment with the offending drug. The disease resulted in three lethal outcomes, all of which occurred in TEN patients. However, the predicted mortality for the whole group was 5.6 in 38 patients, whereas that for the TEN group was 3.97 in 17 patients. The differences between actual and predicted rates of mortality were not significant. Among the three groups of TEN patients, there were no significant differences in the commencement of re-epithelialization or the duration of hospitalization. Conclusions: In the present study, nonsteroidal anti-inflammatory and anti-infective drugs were the most frequent causative agents (eight patients in each group). In the group of SJS and SJS/TEN patients treated with ST and SC, the mortality rate was 0%. In TEN patients, the mortality rate was 17.6% (three of 17 patients). There were no significant differences in mortality rate among the three TEN treatment groups, but the results may have been biased by the small number of patients. © 2014 The International Society of Dermatology.