To evaluate specificity, level, and avidity of antineutrophil cytoplasmic antibodies (ANCA) in sy... more To evaluate specificity, level, and avidity of antineutrophil cytoplasmic antibodies (ANCA) in systemic lupus erythematosus (SLE). There are no studies of ANCA avidity in SLE. Level (ELISA) and avidity (ELISA) of myeloperoxidase (MPO-), proteinase 3 (PR3-), lactoferrin (LF-), cathepsin G, elastase (EL-), and bactericidal/permeability increasing protein (BPI)-ANCA in 142 SLE patients were studied. SLE activity was measured by SLEDAI-2 K. 25/40 ANCA-positive patients were immunoserologically followed (12 ± 2 months). 40/142 (28.2%) SLE patients were ANCA-positive: LF- (21/40), MPO- (19/40), EL- (6/40), PR3- (3/40), and BPI-ANCA (1/40). Only LF-ANCA were associated with renal manifestations (p < 0.05), and positive predictive value for renal involvement in ANCA-positive SLE was 76.2%. LF-ANCA-positive patients had higher SLEDAI-2 K (p < 0.05) and more frequently had anti-dsDNA (p < 0.05), low C3 (p < 0.001), and low C4 (p < 0.05) than LF-ANCA-negative patients. LF-ANCA level was in a positive correlation with SLEDAI-2 K, anti-dsDNA, and anti-C1q (p < 0.01) and in a negative correlation with C3 and C4 (p < 0.05). LF-ANCA avidity was higher than MPO-, EL-, PR3-, and BPI-ANCA avidity (p < 0.01). In LF-ANCA-positive patients, renal manifestations were associated with higher LF-ANCA level (p < 0.01) and avidity (p < 0.05). Based on LF-ANCA level and avidity, the receiver operating characteristic curves for discriminating patients with and without renal involvement had areas under the curves of 0.988 (95% CI: 0.949–1.00) and 0.813 (95% CI: 0.607–1.00), respectively. After the follow-up period, number of LF-ANCA-positive patients decreased (p < 0.01). In contrast to other ANCAs, only LF-ANCA level correlated with activity and standard serological SLE markers. LF-ANCA level and avidity might be biomarkers of renal involvement in SLE. LF-ANCA are promising serological marker in SLE. Key Points • LF- and MPO-ANCA were most frequently found, while EL-, PR3-, and BPI-ANCA were rarely detected in SLE. • In contrast to other ANCAs, only LF-ANCA were associated with renal involvement, and their level correlated with the activity and standard serological markers of SLE. • LF-ANCA avidity was higher than other ANCAs’ avidity; LF-ANCA level and avidity might be useful biomarkers of renal manifestations in SLE. • Detection of ANCA specificity, level, and avidity may help in the diagnosis of particular clinical SLE phenotypes. Key Points • LF- and MPO-ANCA were most frequently found, while EL-, PR3-, and BPI-ANCA were rarely detected in SLE. • In contrast to other ANCAs, only LF-ANCA were associated with renal involvement, and their level correlated with the activity and standard serological markers of SLE. • LF-ANCA avidity was higher than other ANCAs’ avidity; LF-ANCA level and avidity might be useful biomarkers of renal manifestations in SLE. • Detection of ANCA specificity, level, and avidity may help in the diagnosis of particular clinical SLE phenotypes.
Serbian Journal of Dermatology and Venereology, 2016
Wells syndrome (WS) is a rare inflammatory skin disease of unknown etiology. Possible triggers fo... more 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-l...
Acta dermatovenerologica Alpina, Pannonica, et Adriatica, 2006
Originally believed to be of histiocytic origin, regressing primary cutaneous anaplastic large-ce... more Originally believed to be of histiocytic origin, regressing primary cutaneous anaplastic large-cell lymphoma is a CD30 (Ki 1) positive T-cell lymphoma with histologic high grade malignancy, but with an often favorable clinical course with regression of individual lesions. We present a case of a 69-year-old white woman with an 8-month history of noduloulcerative lesions on her right lower leg, otherwise in good general health. The clinical, histologic, and immunohistochemical findings pointed to CD30 positive primary cutaneous anaplastic large-cell lymphoma. There were no signs of generalization, lymph node or internal organ involvement. After 2 years of activity, the disease regressed. During the 10-year follow-up period, no signs of disease reactivation were noted. Accurate recognition of this lymphoma is important to avoid unnecessary aggressive treatments.
Epidermal in vivo nuclear staining is occasionally noted in the lupus band test (LBT) in patients... more Epidermal in vivo nuclear staining is occasionally noted in the lupus band test (LBT) in patients with connective tissue diseases (CTD). The exact clinical significance of this finding remains unelucidated, especially in association with a positive LBT We have reviewed the clinical and serological characteristics of patients with in vivo-bound antinuclear antibodies (ANA) in keratinocytes. Between 1990-1999 speckled IgG staining in keratinocyte nuclei was observed in 31 LBT specimens. We had detailed clinical and laboratory data for 22/31 patients. The present study comprises 22 patients with in vivo-bound ANA (8 cases with mixed CTD (MCTD), 10 with systemic lupus erythematosus (SLE), 2 with Sjögren's syndrome (SS), one with undefined CTD and one clinically healthy mother of a child with neonatal lupus erythematosus), and 22 consecutive CTD patients (2 MCTD, 15 SLE, 5 SS) without in vivo-bound ANA. Antinuclear, anti-dsDNA and anti-extractable nuclear antigens (ENA) antibodies we...
Antimitochondrial M5 type antibodies (AMA M5) have been described in patients with antiphospholip... more Antimitochondrial M5 type antibodies (AMA M5) have been described in patients with antiphospholipid syndrome (APS), thrombocytopenia and autoimmune hemolytic anemia. The aim of this study was to describe the clinical and immunological characteristics of a series of patients with AMA M5. We analyzed 71 patients with AMA M5 seen consecutively at our centres during the last 8 years. The clinical and immunological characteristics of diseases expression were compared with 70 consecutive disease control patients without AMA M5. Compared with the control group, AMA M5 positive patients presented higher prevalence of false positive VDRL test (P &lt; 0.001) and thrombocytopenia (P = 0.002) with lower levels of anti-beta2 glycoprotein I antibodies. In AMA M5 patients (56 female, 15 male) a heterogeneous group of disorders were found. Twenty-seven (38%) patients fulfilled the Sapporo criteria for the classification of the APS. Laboratory criteria were met in 55 (77.5%), and clinical criteria in 31 (43.7%) patients. Six patients initially presented with non-criteria features of APS during follow-up period developed APS. Younger patients with AMA M5 should be carefully observed for the development of APS, even in the absence of serological criteria, while elderly must be screened for monoclonal gammopathy and hematological disorders.
Circumscribed palmar or plantar hypokeratosis (CPH) is a rare condition, usually asymptomatic, co... more Circumscribed palmar or plantar hypokeratosis (CPH) is a rare condition, usually asymptomatic, consisting of a well-demarcated erythema with central depression and hyperkeratotic border which divides it from the normal skin. We report a 77-year-old woman with a characteristic lesion of circumscribed palmar hypokeratosis on the right palm. Clinically, the lesion simulated porokeratosis of Mibelli, but histologically there was no cornoid lamella, while the characteristic depression of epidermis, with sharp stair in stratum corneum between the normal and involved skin was present. This is the first case of CPH reported in south-east Europe. After 9-year follow-up and various treatment modalities, we confirmed resistance of CPH. Since malignant transformation has been documented, careful follow-up was recommended.
Acta dermatovenerologica Alpina, Pannonica, et Adriatica, 2006
We report two unusual patients with Rothmund-Thomson syndrome (RTS), a rare genodermatosis. The f... more We report two unusual patients with Rothmund-Thomson syndrome (RTS), a rare genodermatosis. The first patient is a 5-year-old girl with congenital poikiloderma, photosensitivity, plantar punctate keratoderma, stunted growth and severe mental retardation. Plantar keratoderma associated with RTS has been reported only once. The second patient is a 21-year-old female presenting with rounded "moon" face, trunk obesity, coeliac disease, short stature and mild mental retardation. This is the first case of RTS associated with coeliac disease.
Cutaneous necrotizing eosinophilic vasculitis (CNEV) is a rare type of vasculitis. Eosinophilic v... more Cutaneous necrotizing eosinophilic vasculitis (CNEV) is a rare type of vasculitis. Eosinophilic vasculitis is a necrotizing vasculitis with eosinophilic vascular infiltration, in which eosinophils mediate vascular damage in the disease process. We present a case of an 18-year-old girl who developed palpable purpura and hemorrhagic bullae over the lower extremities associated with itching, 7 days after the commencement of penicillin therapy. Plasma cryofibrinogen was positive. Histopathology showed an infiltration of eosinophils within and around the vessel walls and a complete absence of nuclear dust and neutrophils. Oral prednisone at 1 mg/kg induced remission in 2 weeks; the prednisone dose was tapered and discontinued after 2.5 months. There was no evidence of recurrence after 37 months of follow-up. Our patient represents a rare case of drug/penicillin-induced CNEV associated with cryofibrinogenemia, without systemic organ involvement.
Annular lichenoid dermatitis of youth (ALDY), first described in 2003, represents an uncommon ent... more 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.
There is no universally accepted treatment for severe pediatric alopecia areata (AA). This prospe... more 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.
IgA pemphigus (IGAP) is a rare autoimmune bullous disease characterized by IgA deposits on kerati... more 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, ...
Successful control of atopic dermatitis (AD) in children depends on parents' knowledge on the... more Successful control of atopic dermatitis (AD) in children depends on parents' knowledge on the disease and attitude toward ill child, but there is a lack studies exploring parental knowledge, attitude, and behaviors. The aim of this study was to investigate parents' knowledge, attitude, and behavior toward AD. A cross-sectional study was conducted at the Clinic of Dermatovenereology, Clinical Center of Serbia, Belgrade, between February 2015 and March 2016. Parents of children with AD were invited to complete the questionnaire, which was comprised of five parts: parental sociodemographic characteristics, demographic and clinical characteristics of children, knowledge, attitude, and behavior. To assess factors associated with a higher knowledge level on AD, stronger positive attitude, and more supportive behavior, we performed two multiple linear regression models. The average parental knowledge score was 9.5 ± 1.9 out of 12. The level of knowledge did not correlate with paren...
JDDG: Journal der Deutschen Dermatologischen Gesellschaft, 2016
Die meisten früheren Arbeiten zu den klinisch-epidemiologischen Merkmalen von bullösen Autoimmune... more Die meisten früheren Arbeiten zu den klinisch-epidemiologischen Merkmalen von bullösen Autoimmunerkrankungen (AIBD) konzentrierten sich vor allem auf eine einzige Krankheitsentität oder nur eine Krankheitsgruppe; nur in wenigen Studien wurde die Inzidenz verschiedener AIBD untersucht. Bei der vorliegenden Studie war es unser Ziel, das gesamte Spektrum der AIBD zu betrachten, die Inzidenz der häufigsten AIBD zu ermitteln und die zeitlichen Trends ihres Auftretens in Zentralserbien über einen Zeitraum von 20 Jahren zu untersuchen. Wir rekrutierten retrospektiv 1161 AIBD-Fälle, die in Zentralserbien von Januar 1991 bis Dezember 2010 neu diagnostiziert wurden. Die Diagnose stützte sich auf eine strikte klinische, histologische und immunhistologische Beurteilung. Folgende Inzidenzraten wurden für die einzelnen Erkrankungen ermittelt: 4,35 pro eine Million Einwohner/Jahr (pME/Jahr) für Pemphigus, 4,47 pME/Jahr für Pemphigoid, 1,42 pME/Jahr für Dermatitis herpetiformis (DH), 0,25 pME/Jahr IgA-Dermatose und 0,08 pME/Jahr für Epidermolysis bullosa acquisita. Im betrachteten Zeitraum stieg die altersbereinigte Inzidenzrate für Pemphigus und insbesondere für Pemphigoid signifikant an, während sie für DH, allerdings nicht signifikant, abnahm. Unsere Studie befasst sich zum ersten Mal mit den Inzidenzraten des gesamten Spektrums der AIBD in Serbien und untersucht die zeitlichen Trends ihres Auftretens über einen Zeitraum von 20 Jahren. Nach unserem besten Wissen wurde ein ähnlicher Befund wie der unsere, dass nämlich die Inzidenzraten von Pemphigus und Pemphigoid vergleichbar sind, bisher noch nicht publiziert.
JDDG: Journal der Deutschen Dermatologischen Gesellschaft, 2016
While most previous surveys on the clinico-epidemiological features of autoimmune bullous disease... more While most previous surveys on the clinico-epidemiological features of autoimmune bullous diseases (AIBDs) have predominantly focused on a single disease entity or just one disease group, there have been only few studies examining the incidence of various AIBDs. In the present study, we set out to determine the spectrum of AIBDs, to estimate the incidence of the most common AIBDs, and to examine their temporal trends in Central Serbia over a period of 20 years. We retrospectively recruited 1,161 new AIBD cases diagnosed in Central Serbia during the period from January 1991 to December 2010. The diagnosis was based on strict clinical, histological, and immunohistological evaluation. The incidence rates were: 4.35 per million population/year (pmp/year) for pemphigus, 4.47 pmp/year for pemphigoid, 1.42 pmp/year for dermatitis herpetiformis (DH), 0.25 pmp/year for linear IgA disease, and 0.08 pmp/year for epidermolysis bullosa acquisita. In the period observed, age-adjusted incidence rates significantly increased for pemphigus and particularly for pemphigoid, whereas they decreased, albeit not significantly, for DH. For the first time, our study evaluates the incidence rates of the entire spectrum of AIBDs in Serbia, and examines their temporal trends over a 20-year period. To the best of our knowledge, our finding of similar incidence rates for pemphigus and pemphigoid has previously not been reported.
To evaluate specificity, level, and avidity of antineutrophil cytoplasmic antibodies (ANCA) in sy... more To evaluate specificity, level, and avidity of antineutrophil cytoplasmic antibodies (ANCA) in systemic lupus erythematosus (SLE). There are no studies of ANCA avidity in SLE. Level (ELISA) and avidity (ELISA) of myeloperoxidase (MPO-), proteinase 3 (PR3-), lactoferrin (LF-), cathepsin G, elastase (EL-), and bactericidal/permeability increasing protein (BPI)-ANCA in 142 SLE patients were studied. SLE activity was measured by SLEDAI-2 K. 25/40 ANCA-positive patients were immunoserologically followed (12 ± 2 months). 40/142 (28.2%) SLE patients were ANCA-positive: LF- (21/40), MPO- (19/40), EL- (6/40), PR3- (3/40), and BPI-ANCA (1/40). Only LF-ANCA were associated with renal manifestations (p < 0.05), and positive predictive value for renal involvement in ANCA-positive SLE was 76.2%. LF-ANCA-positive patients had higher SLEDAI-2 K (p < 0.05) and more frequently had anti-dsDNA (p < 0.05), low C3 (p < 0.001), and low C4 (p < 0.05) than LF-ANCA-negative patients. LF-ANCA level was in a positive correlation with SLEDAI-2 K, anti-dsDNA, and anti-C1q (p < 0.01) and in a negative correlation with C3 and C4 (p < 0.05). LF-ANCA avidity was higher than MPO-, EL-, PR3-, and BPI-ANCA avidity (p < 0.01). In LF-ANCA-positive patients, renal manifestations were associated with higher LF-ANCA level (p < 0.01) and avidity (p < 0.05). Based on LF-ANCA level and avidity, the receiver operating characteristic curves for discriminating patients with and without renal involvement had areas under the curves of 0.988 (95% CI: 0.949–1.00) and 0.813 (95% CI: 0.607–1.00), respectively. After the follow-up period, number of LF-ANCA-positive patients decreased (p < 0.01). In contrast to other ANCAs, only LF-ANCA level correlated with activity and standard serological SLE markers. LF-ANCA level and avidity might be biomarkers of renal involvement in SLE. LF-ANCA are promising serological marker in SLE. Key Points • LF- and MPO-ANCA were most frequently found, while EL-, PR3-, and BPI-ANCA were rarely detected in SLE. • In contrast to other ANCAs, only LF-ANCA were associated with renal involvement, and their level correlated with the activity and standard serological markers of SLE. • LF-ANCA avidity was higher than other ANCAs’ avidity; LF-ANCA level and avidity might be useful biomarkers of renal manifestations in SLE. • Detection of ANCA specificity, level, and avidity may help in the diagnosis of particular clinical SLE phenotypes. Key Points • LF- and MPO-ANCA were most frequently found, while EL-, PR3-, and BPI-ANCA were rarely detected in SLE. • In contrast to other ANCAs, only LF-ANCA were associated with renal involvement, and their level correlated with the activity and standard serological markers of SLE. • LF-ANCA avidity was higher than other ANCAs’ avidity; LF-ANCA level and avidity might be useful biomarkers of renal manifestations in SLE. • Detection of ANCA specificity, level, and avidity may help in the diagnosis of particular clinical SLE phenotypes.
Serbian Journal of Dermatology and Venereology, 2016
Wells syndrome (WS) is a rare inflammatory skin disease of unknown etiology. Possible triggers fo... more 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-l...
Acta dermatovenerologica Alpina, Pannonica, et Adriatica, 2006
Originally believed to be of histiocytic origin, regressing primary cutaneous anaplastic large-ce... more Originally believed to be of histiocytic origin, regressing primary cutaneous anaplastic large-cell lymphoma is a CD30 (Ki 1) positive T-cell lymphoma with histologic high grade malignancy, but with an often favorable clinical course with regression of individual lesions. We present a case of a 69-year-old white woman with an 8-month history of noduloulcerative lesions on her right lower leg, otherwise in good general health. The clinical, histologic, and immunohistochemical findings pointed to CD30 positive primary cutaneous anaplastic large-cell lymphoma. There were no signs of generalization, lymph node or internal organ involvement. After 2 years of activity, the disease regressed. During the 10-year follow-up period, no signs of disease reactivation were noted. Accurate recognition of this lymphoma is important to avoid unnecessary aggressive treatments.
Epidermal in vivo nuclear staining is occasionally noted in the lupus band test (LBT) in patients... more Epidermal in vivo nuclear staining is occasionally noted in the lupus band test (LBT) in patients with connective tissue diseases (CTD). The exact clinical significance of this finding remains unelucidated, especially in association with a positive LBT We have reviewed the clinical and serological characteristics of patients with in vivo-bound antinuclear antibodies (ANA) in keratinocytes. Between 1990-1999 speckled IgG staining in keratinocyte nuclei was observed in 31 LBT specimens. We had detailed clinical and laboratory data for 22/31 patients. The present study comprises 22 patients with in vivo-bound ANA (8 cases with mixed CTD (MCTD), 10 with systemic lupus erythematosus (SLE), 2 with Sjögren's syndrome (SS), one with undefined CTD and one clinically healthy mother of a child with neonatal lupus erythematosus), and 22 consecutive CTD patients (2 MCTD, 15 SLE, 5 SS) without in vivo-bound ANA. Antinuclear, anti-dsDNA and anti-extractable nuclear antigens (ENA) antibodies we...
Antimitochondrial M5 type antibodies (AMA M5) have been described in patients with antiphospholip... more Antimitochondrial M5 type antibodies (AMA M5) have been described in patients with antiphospholipid syndrome (APS), thrombocytopenia and autoimmune hemolytic anemia. The aim of this study was to describe the clinical and immunological characteristics of a series of patients with AMA M5. We analyzed 71 patients with AMA M5 seen consecutively at our centres during the last 8 years. The clinical and immunological characteristics of diseases expression were compared with 70 consecutive disease control patients without AMA M5. Compared with the control group, AMA M5 positive patients presented higher prevalence of false positive VDRL test (P &lt; 0.001) and thrombocytopenia (P = 0.002) with lower levels of anti-beta2 glycoprotein I antibodies. In AMA M5 patients (56 female, 15 male) a heterogeneous group of disorders were found. Twenty-seven (38%) patients fulfilled the Sapporo criteria for the classification of the APS. Laboratory criteria were met in 55 (77.5%), and clinical criteria in 31 (43.7%) patients. Six patients initially presented with non-criteria features of APS during follow-up period developed APS. Younger patients with AMA M5 should be carefully observed for the development of APS, even in the absence of serological criteria, while elderly must be screened for monoclonal gammopathy and hematological disorders.
Circumscribed palmar or plantar hypokeratosis (CPH) is a rare condition, usually asymptomatic, co... more Circumscribed palmar or plantar hypokeratosis (CPH) is a rare condition, usually asymptomatic, consisting of a well-demarcated erythema with central depression and hyperkeratotic border which divides it from the normal skin. We report a 77-year-old woman with a characteristic lesion of circumscribed palmar hypokeratosis on the right palm. Clinically, the lesion simulated porokeratosis of Mibelli, but histologically there was no cornoid lamella, while the characteristic depression of epidermis, with sharp stair in stratum corneum between the normal and involved skin was present. This is the first case of CPH reported in south-east Europe. After 9-year follow-up and various treatment modalities, we confirmed resistance of CPH. Since malignant transformation has been documented, careful follow-up was recommended.
Acta dermatovenerologica Alpina, Pannonica, et Adriatica, 2006
We report two unusual patients with Rothmund-Thomson syndrome (RTS), a rare genodermatosis. The f... more We report two unusual patients with Rothmund-Thomson syndrome (RTS), a rare genodermatosis. The first patient is a 5-year-old girl with congenital poikiloderma, photosensitivity, plantar punctate keratoderma, stunted growth and severe mental retardation. Plantar keratoderma associated with RTS has been reported only once. The second patient is a 21-year-old female presenting with rounded "moon" face, trunk obesity, coeliac disease, short stature and mild mental retardation. This is the first case of RTS associated with coeliac disease.
Cutaneous necrotizing eosinophilic vasculitis (CNEV) is a rare type of vasculitis. Eosinophilic v... more Cutaneous necrotizing eosinophilic vasculitis (CNEV) is a rare type of vasculitis. Eosinophilic vasculitis is a necrotizing vasculitis with eosinophilic vascular infiltration, in which eosinophils mediate vascular damage in the disease process. We present a case of an 18-year-old girl who developed palpable purpura and hemorrhagic bullae over the lower extremities associated with itching, 7 days after the commencement of penicillin therapy. Plasma cryofibrinogen was positive. Histopathology showed an infiltration of eosinophils within and around the vessel walls and a complete absence of nuclear dust and neutrophils. Oral prednisone at 1 mg/kg induced remission in 2 weeks; the prednisone dose was tapered and discontinued after 2.5 months. There was no evidence of recurrence after 37 months of follow-up. Our patient represents a rare case of drug/penicillin-induced CNEV associated with cryofibrinogenemia, without systemic organ involvement.
Annular lichenoid dermatitis of youth (ALDY), first described in 2003, represents an uncommon ent... more 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.
There is no universally accepted treatment for severe pediatric alopecia areata (AA). This prospe... more 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.
IgA pemphigus (IGAP) is a rare autoimmune bullous disease characterized by IgA deposits on kerati... more 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, ...
Successful control of atopic dermatitis (AD) in children depends on parents' knowledge on the... more Successful control of atopic dermatitis (AD) in children depends on parents' knowledge on the disease and attitude toward ill child, but there is a lack studies exploring parental knowledge, attitude, and behaviors. The aim of this study was to investigate parents' knowledge, attitude, and behavior toward AD. A cross-sectional study was conducted at the Clinic of Dermatovenereology, Clinical Center of Serbia, Belgrade, between February 2015 and March 2016. Parents of children with AD were invited to complete the questionnaire, which was comprised of five parts: parental sociodemographic characteristics, demographic and clinical characteristics of children, knowledge, attitude, and behavior. To assess factors associated with a higher knowledge level on AD, stronger positive attitude, and more supportive behavior, we performed two multiple linear regression models. The average parental knowledge score was 9.5 ± 1.9 out of 12. The level of knowledge did not correlate with paren...
JDDG: Journal der Deutschen Dermatologischen Gesellschaft, 2016
Die meisten früheren Arbeiten zu den klinisch-epidemiologischen Merkmalen von bullösen Autoimmune... more Die meisten früheren Arbeiten zu den klinisch-epidemiologischen Merkmalen von bullösen Autoimmunerkrankungen (AIBD) konzentrierten sich vor allem auf eine einzige Krankheitsentität oder nur eine Krankheitsgruppe; nur in wenigen Studien wurde die Inzidenz verschiedener AIBD untersucht. Bei der vorliegenden Studie war es unser Ziel, das gesamte Spektrum der AIBD zu betrachten, die Inzidenz der häufigsten AIBD zu ermitteln und die zeitlichen Trends ihres Auftretens in Zentralserbien über einen Zeitraum von 20 Jahren zu untersuchen. Wir rekrutierten retrospektiv 1161 AIBD-Fälle, die in Zentralserbien von Januar 1991 bis Dezember 2010 neu diagnostiziert wurden. Die Diagnose stützte sich auf eine strikte klinische, histologische und immunhistologische Beurteilung. Folgende Inzidenzraten wurden für die einzelnen Erkrankungen ermittelt: 4,35 pro eine Million Einwohner/Jahr (pME/Jahr) für Pemphigus, 4,47 pME/Jahr für Pemphigoid, 1,42 pME/Jahr für Dermatitis herpetiformis (DH), 0,25 pME/Jahr IgA-Dermatose und 0,08 pME/Jahr für Epidermolysis bullosa acquisita. Im betrachteten Zeitraum stieg die altersbereinigte Inzidenzrate für Pemphigus und insbesondere für Pemphigoid signifikant an, während sie für DH, allerdings nicht signifikant, abnahm. Unsere Studie befasst sich zum ersten Mal mit den Inzidenzraten des gesamten Spektrums der AIBD in Serbien und untersucht die zeitlichen Trends ihres Auftretens über einen Zeitraum von 20 Jahren. Nach unserem besten Wissen wurde ein ähnlicher Befund wie der unsere, dass nämlich die Inzidenzraten von Pemphigus und Pemphigoid vergleichbar sind, bisher noch nicht publiziert.
JDDG: Journal der Deutschen Dermatologischen Gesellschaft, 2016
While most previous surveys on the clinico-epidemiological features of autoimmune bullous disease... more While most previous surveys on the clinico-epidemiological features of autoimmune bullous diseases (AIBDs) have predominantly focused on a single disease entity or just one disease group, there have been only few studies examining the incidence of various AIBDs. In the present study, we set out to determine the spectrum of AIBDs, to estimate the incidence of the most common AIBDs, and to examine their temporal trends in Central Serbia over a period of 20 years. We retrospectively recruited 1,161 new AIBD cases diagnosed in Central Serbia during the period from January 1991 to December 2010. The diagnosis was based on strict clinical, histological, and immunohistological evaluation. The incidence rates were: 4.35 per million population/year (pmp/year) for pemphigus, 4.47 pmp/year for pemphigoid, 1.42 pmp/year for dermatitis herpetiformis (DH), 0.25 pmp/year for linear IgA disease, and 0.08 pmp/year for epidermolysis bullosa acquisita. In the period observed, age-adjusted incidence rates significantly increased for pemphigus and particularly for pemphigoid, whereas they decreased, albeit not significantly, for DH. For the first time, our study evaluates the incidence rates of the entire spectrum of AIBDs in Serbia, and examines their temporal trends over a 20-year period. To the best of our knowledge, our finding of similar incidence rates for pemphigus and pemphigoid has previously not been reported.
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Papers by Milos Nikolic