RESUMO
Cheilitis granulomatosa (CG) is a subset of orofacial granulomatosis (OFG) and considered to be a monosymptomatic form of Melkersson-Rosenthal syndrome (MRS), which is characterized by the triad of chronic lip swelling, facial paralysis, and fissured tongue. The labial swelling is thought to be associated with an orofacial swelling, which affects the chin, cheeks, and oral mucosa. Histologically, it is distinguished by noncaseating granulomas consisting of lymphohistiocytes and giant cells. Although systemic steroids with or without intralesional triamcinolone injections are the mainstay of treatment, and various agents have been proposed for this rare disease, no successful treatment modality has been reported in the literature yet. Herein, we present our experience with three different CG cases, which showed a varied level of positive response to the combination therapy.
Assuntos
Bochecha , Queilite , Queixo , Paralisia Facial , Células Gigantes , Granuloma , Granulomatose Orofacial , Lábio , Síndrome de Melkersson-Rosenthal , Mucosa Bucal , Doenças Raras , Esteroides , Língua Fissurada , TriancinolonaRESUMO
BACKGROUND: Adverse cutaneous drug reactions (ACDRs) are common and are responsible for increased morbidity, mortality, and socioeconomic costs. OBJECTIVE: The purpose of our study was to investigate the common drugs and clinical patterns related to ACDRs using an electronic drug adverse reaction reporting system at a single secondary referral center. METHODS: We conducted a retrospective analysis of the ACDR database between January 2014 and April 2016 at the Ilsan Paik Hospital. RESULTS: The study analyzed 320 patients with ACDRs (male:female ratio=93:227; mean age 50.8±17.8 years). Using a Korean causality evaluation algorithm, the percentage of drugs with a possible relationship with ACDRs was calculated to be 50.6%, while the percentage with a probable relationship was 44.7%. Antibiotics (44.0%), radiocontrast media (15.1%), and non-steroidal anti-inflammatory drugs (NSAIDs) (14.3%) were the most commonly implicated drugs. Antibiotics, including cephalosporins (30.6%) and quinolones (10.2%), were responsible for the majority of the ACDRs. Acetic acid (5.9%) and propionic acid (5.9%) derivatives of NSAIDs were also common causative agents. The most common clinical presentations were maculopapular exanthema (33.4%), pruritus (30.9%), and urticaria (25.7%). Severe ACDRs were significantly associated with older age, eosinophilia, and underlying heart and renal diseases (p<0.05). CONCLUSION: Antibiotics, radiocontrast media, and NSAIDs were identified as common causes of ACDRs. Older age, eosinophilia, heart disease, and renal disease were associated with severe ACDRs.
Assuntos
Humanos , Ácido Acético , Sistemas de Notificação de Reações Adversas a Medicamentos , Antibacterianos , Anti-Inflamatórios não Esteroides , Cefalosporinas , Meios de Contraste , Dietilpropiona , Efeitos Colaterais e Reações Adversas Relacionados a Medicamentos , Eosinofilia , Exantema , Coração , Cardiopatias , Mortalidade , Prurido , Quinolonas , Estudos Retrospectivos , Centros de Cuidados de Saúde Secundários , UrticáriaRESUMO
Kaposi's sarcoma (KS) is a vascular neoplasm originating from vascular and lymphatic endothelial cells. Iatrogenic KS mainly develops in organ transplant patients or after receiving immunosuppressive therapy. An 81-year-old man presented with multiple dark-purplish nodules, plaques, and patches on the right leg for 3 weeks. Previously, the patient was treated with prednisolone 10∼30 mg/day for chronic obstructive pulmonary disease for 3 months, and percutaneous transluminal angioplasty was performed 1 month previously for the treatment of peripheral arterial occlusive disease. A biopsy specimen of the nodule showed closely packed spindle cells forming slit-like vascular structures, which were consistent with KS. Despite the dosage reduction of prednisolone for treatment, the skin lesions progressed aggressively throughout the entire body, and the patient died after 5 months. We report a case of iatrogenic prednisolone-associated KS rapidly progressing to the entire body shortly thereafter.
Assuntos
Idoso de 80 Anos ou mais , Humanos , Angioplastia , Arteriopatias Oclusivas , Biópsia , Células Endoteliais , Glucocorticoides , Doença Iatrogênica , Perna (Membro) , Prednisolona , Doença Pulmonar Obstrutiva Crônica , Sarcoma de Kaposi , Pele , Transplantes , Neoplasias VascularesRESUMO
BACKGROUND: The diagnosis of bullous pemphigoid is made based on clinical, histologic, and immunofluorescence features. OBJECTIVE: The purpose of this study was to analyze the factors that may affect the positivity and intensity of direct immunofluorescence (DIF) in patients with bullous pemphigoid. METHODS: We performed a retrospective review of 41 cases of bullous pemphigoid at Ilsan Paik Hospital between January 2008 and December 2014. We investigated the positivity of DIF, immunofluorescence intensity of C3, age, sex, biopsy sites, extent of disease, duration of disease, and the degree of inflammatory cell infiltration. RESULTS: Twenty-seven of 41 (65.9%) cases had positive DIF results for either IgG or C3, and 14 of 41 (34.1%) had negative DIF results for both IgG and C3. Twenty-one cases (51.2%) of IgG and 25 cases (61.0%) of C3 had characteristic linear C3 deposition on the dermo-epidermal junction. Disease duration influenced DIF positivity (p<0.05). Although a higher positive rate of DIF was observed in biopsy specimens taken from the upper extremities than in those from other sites, the difference was not statistically significant. Sex, age, extent of disease, and the degree of inflammatory cell infiltration were not significantly associated with the positivity of DIF. There was no relationship between fluorescence intensity of C3 and the degree of inflammatory cell infiltration. CONCLUSION: This study suggests that the long duration of disease (more than 10 days) may increase the positivity of DIF. Age, sex, biopsy site, extent of disease, and the degree of inflammatory cell infiltration had no influence on DIF positivity.
Assuntos
Humanos , Biópsia , Diagnóstico , Fluorescência , Imunofluorescência , Técnica Direta de Fluorescência para Anticorpo , Imunoglobulina G , Penfigoide Bolhoso , Estudos Retrospectivos , Extremidade SuperiorRESUMO
Neonatal lupus erythematosus (NLE) is a rare autoimmune disease that has a clinical spectrum of cutaneous, cardiac, and systemic abnormalities in neonates. It is caused by transplacental passage of maternal anti-Ro and/or anti-La autoantibodies, which result in skin lesions such as subacute cutaneous lupus erythematosus, congenital heart block, and liver function and hematologic abnormalities. We report a case of NLE in a 31-day-old female infant who was born to a clinically asymptomatic mother with anti-SSA/Ro and anti-SSB/La antibodies. The baby presented with multiple erythematous patches and annular plaques on the face and trunk. The skin biopsy showed slight follicular plugging, focal hydropic degeneration of the basal epidermis and mild edema, telangiectasia, and perivascular and interstitial lymphohistiocytic infiltration in the upper dermis. Her serological tests were positive for antinuclear antibody (ANA), anti-SSA/Ro, and anti-SSB/La. These findings are consistent with NLE. The mother also had a positive autoantibody profile for ANA, anti-SSA/Ro, and anti-SSB/La without clinical symptoms.
Assuntos
Feminino , Humanos , Lactente , Recém-Nascido , Anticorpos , Anticorpos Antinucleares , Autoanticorpos , Doenças Autoimunes , Biópsia , Derme , Edema , Epiderme , Bloqueio Cardíaco , Fígado , Lúpus Eritematoso Cutâneo , Mães , Testes Sorológicos , Pele , TelangiectasiaRESUMO
Neonatal lupus erythematosus (NLE) is a rare autoimmune disease that has a clinical spectrum of cutaneous, cardiac, and systemic abnormalities in neonates. It is caused by transplacental passage of maternal anti-Ro and/or anti-La autoantibodies, which result in skin lesions such as subacute cutaneous lupus erythematosus, congenital heart block, and liver function and hematologic abnormalities. We report a case of NLE in a 31-day-old female infant who was born to a clinically asymptomatic mother with anti-SSA/Ro and anti-SSB/La antibodies. The baby presented with multiple erythematous patches and annular plaques on the face and trunk. The skin biopsy showed slight follicular plugging, focal hydropic degeneration of the basal epidermis and mild edema, telangiectasia, and perivascular and interstitial lymphohistiocytic infiltration in the upper dermis. Her serological tests were positive for antinuclear antibody (ANA), anti-SSA/Ro, and anti-SSB/La. These findings are consistent with NLE. The mother also had a positive autoantibody profile for ANA, anti-SSA/Ro, and anti-SSB/La without clinical symptoms.
