RESUMO
BACKGROUND: Sarcoidosis is a chronic granulomatous inflammatory disorder involving many organs including the skin with various clinical manifestations. OBJECTIVE: The purpose of this study was to investigate the clinico-pathological characteristics of sarcoidosis. METHODS: A total of 19 patients with cutaneous sarcoidosis were enrolled in this study. The hospital charts and histopathological slides of the patients with sarcoidosis diagnosed at Asan Medical Center from 1989 to 1999 were reviewed. RESULTS: 1. Three male and sixteen female patients were included(M:F=1:5.3). Age at diagnosis ranged from 4 to 62 years. The mean age was 46 years in male patients and 44.9 years in female patients. 2. Multiple or several(more than 2) skin lesions were common(78.9%). Extremities were the favored sites. Clinically the lesions were presented as subcutaneous nodules, plaques, nodule, macules or papules, patches, psoriasiform, and pitted scar in decreasing order. Cutaneous lesions arising from the scar were present in 3 cases. Six patients(31.6%) complained of systemic symptoms such as fever or weight loss. 3. The systemic involvement was present in 11 cases(57.9%). Lung, hilar lymph nodes, and mediastinal lymph nodes were the most commonly involved organs. 4. Laboratory examinations revealed elevated erythrocyte sedimentation rate(71.4%) and angiotensin converting enzyme(ACE)(90%). In all the patients with elevated level of serum ACE the involvement of intrathorax was present, however one patient with systemic involvement showed normal level of ACE. Serum calcium level was normal in all the patients and the level of CD4/CD8 revealed variable results(1.3 to 3.9). 5. Histopathological evaluation revealed non-caseating epithelioid cell granulomas(100%) with multinucleated giant cells(52.6%) and asteroid body(31.6%). In 11 cases(57.9%), fibrosis was the prominent finding and in two cases(10.5%) partial interstitial necrosis was noted. Histopathologically subcutaneous pattern was predominant in 8 cases(42.1%). The patients with subcutaneous sarcoidosis did not show any specific clinical or laboratory differences from the other types in our study. CONCLUSION: Cutaneous sarcoidosis showed varied clinical and histopathological manifestations and ran various clinical courses. In cutaneous sarcoidosis women were more commonly involved than in systemic sarcoidosis and more elderly patients were involved in cutaneous sarcoidosis. Extensive work-up for systemic involvement may be mandatory when sarcoidosis is presented with cutaneous lesions or the level of serum ACE is elevated in cutaneous sarcoidosis. The level of CD4/CD8 may be a insignificant laboratory index in sarcoidosis. Also, subcutaneous sarcoidosis may not be a marker of the systemic involvement.
Assuntos
Idoso , Feminino , Humanos , Masculino , Angiotensinas , Sedimentação Sanguínea , Cálcio , Cicatriz , Diagnóstico , Células Epitelioides , Extremidades , Febre , Fibrose , Pulmão , Linfonodos , Necrose , Sarcoidose , Pele , Redução de PesoRESUMO
Toxic epidermal necrolysis(TEN) is the most dramatic, life-threatening cutaneous drug reaction. TEN is characterized by extensive detachment of the epidermis undergoing full-thickness necrosis. The pathogenetic pathway and specific treatments of TEN have not yet been identified. Recently, several reports suggested that apoptosis in TEN is promoted by proteins such as Fas antigen and high dose intravenous immunoglobulin(IVIG) inhibits Fas-mediated cell death by blocking the Fas receptor. We report a case of TEN in a 26-year-old woman who had been treated with prednisolone due to iritis. She was treated with high dose IVIG(0.4g/Kg/day for 4 days one day and 0.7g/Kg/day for consecutive). In our case, the progression of skin lesion was interrupted after IVIG infusion and favorable vital outcome was achieved with mild adverse effects.
Assuntos
Adulto , Feminino , Humanos , Receptor fas , Apoptose , Morte Celular , Epiderme , Imunoglobulinas , Imunoglobulinas Intravenosas , Irite , Necrose , Prednisolona , Pele , Síndrome de Stevens-JohnsonRESUMO
Toxic epidermal necrolysis(TEN) is the most dramatic, life-threatening cutaneous drug reaction. TEN is characterized by extensive detachment of the epidermis undergoing full-thickness necrosis. The pathogenetic pathway and specific treatments of TEN have not yet been identified. Recently, several reports suggested that apoptosis in TEN is promoted by proteins such as Fas antigen and high dose intravenous immunoglobulin(IVIG) inhibits Fas-mediated cell death by blocking the Fas receptor. We report a case of TEN in a 26-year-old woman who had been treated with prednisolone due to iritis. She was treated with high dose IVIG(0.4g/Kg/day for 4 days one day and 0.7g/Kg/day for consecutive). In our case, the progression of skin lesion was interrupted after IVIG infusion and favorable vital outcome was achieved with mild adverse effects.
Assuntos
Adulto , Feminino , Humanos , Receptor fas , Apoptose , Morte Celular , Epiderme , Imunoglobulinas , Imunoglobulinas Intravenosas , Irite , Necrose , Prednisolona , Pele , Síndrome de Stevens-JohnsonRESUMO
Accessory tragus is a rare congenital malformation of the external ear. It is usually presented as congenital, firm papules located anterior to tragus commonly. Other less common sites include the cheek and lateral neck anterior to the sternocleidomastoid muscle. Though the possibility of occurrence on the neck, there was no reported case. We present herein a typical case of accessory tragus on the right lateral neck in 24-year-old female.