RESUMO
Paraneoplastic pemphigus (PNP) is seen most frequently in the setting of Castleman's disease (CD) in childhood. We report herein a 10-year-old girl with PNP appearing a few weeks after resection of a recurrent CD. Despite improvement in skin and mucosal lesions with prednisolone and azathioprine, she had severe bronchiolitis obliterans and died from respiratory failure a few months later.
Assuntos
Hiperplasia do Linfonodo Gigante/cirurgia , Síndromes Paraneoplásicas/patologia , Pênfigo/patologia , Azatioprina/uso terapêutico , Bronquiolite Obliterante/complicações , Hiperplasia do Linfonodo Gigante/tratamento farmacológico , Criança , Evolução Fatal , Feminino , Humanos , Imunossupressores/uso terapêutico , Síndromes Paraneoplásicas/tratamento farmacológico , Síndromes Paraneoplásicas/cirurgia , Pênfigo/tratamento farmacológico , Prednisolona/uso terapêutico , Insuficiência Respiratória/etiologia , Índice de Gravidade de DoençaRESUMO
BACKGROUND: Negative direct immunofluorescence (DIF) is a predictor of immunologic remission in pemphigus vulgaris. Recently, it has been shown that plucked hair can be used as substrate for DIF in the diagnosis of pemphigus. OBJECTIVE: We sought to compare hair DIF in patients with pemphigus vulgaris in clinical remission with conventional DIF for the assessment of immunologic remission. METHODS: A total of 55 patients with pemphigus vulgaris fulfilling the following inclusion criteria were enrolled: absence of any lesion and daily prednisolone dosage equal or less than 10 mg without adjuvant drug in the preceding 6 months. Biopsy specimen and plucked hair were processed for DIF. Intercellular deposition of IgG and/or C3 was considered positive. RESULTS: Conventional DIF and hair DIF were positive in 28 (50.9%) and 36 (65.5%) patients, respectively. Hair DIF had a sensitivity of 0.79 (95% confidence interval [CI] 0.59-0.92), a specificity of 0.48 (95% CI 0.29-0.68), a positive predictive value of 0.61 (95% CI 0.44-0.77), and a negative predictive value of 0.68 (95% CI 0.43-0.87). LIMITATIONS: Small sample size is the main limitation of this study. CONCLUSIONS: The sensitivity of hair DIF was not high enough to allow us to suggest it as a substitute for conventional DIF. On the other hand, one cannot disregard positive cases of hair DIF in the setting of negative biopsy DIF. As hair plucking is less invasive than biopsy, the following approach could be suggested: hair DIF may be repeated in patients in clinical remission until negative; then conventional DIF should be performed, too. The physician can decide to stop treatment only when DIF assays on both substrates are negative.
Assuntos
Cabelo/imunologia , Pênfigo/imunologia , Adolescente , Adulto , Idoso , Feminino , Técnica Direta de Fluorescência para Anticorpo , Humanos , Masculino , Pessoa de Meia-Idade , Pênfigo/diagnóstico , Pênfigo/tratamento farmacológico , Indução de Remissão , Adulto JovemRESUMO
Vaginal involvement in pemphigus vulgaris has previously been described. In all those cases a pelvic examination was needed to explore the lesions. We describe a patient with pemphigus vulgaris who had pemphigus erosions on a prolapsed uterus (i.e., on the everted surface of vagina). The patient had widespread lesions of pemphigus in other mucosal and cutaneous sites. Biopsy, antibodies against desmoglein 1 and 3, and direct and indirect immunofluorescence were confirming. The erosions on the prolapsed uterus were resistant to treatment; other mucosal and cutaneous lesions responded rapidly to prednisolone and azathioprine. After lowering the dose of prednisolone the patient was referred to a gynecologist for a vaginal hysterectomy. This case was unique because her vaginal lesions could be easily examined and followed.
Assuntos
Pênfigo/complicações , Prolapso Uterino/complicações , Idoso , Autoanticorpos/imunologia , Desmogleína 1/imunologia , Desmogleína 3/imunologia , Resistência a Medicamentos , Feminino , Humanos , Histerectomia Vaginal , Imunossupressores/uso terapêutico , Pênfigo/diagnóstico , Pênfigo/tratamento farmacológico , Pênfigo/imunologia , Prednisolona/uso terapêutico , Prolapso Uterino/imunologia , Prolapso Uterino/cirurgiaRESUMO
Herein we report three married women referred to Dermatology Clinic of Loghman Hakim Hospital, Tehran, Iran in 2017 for evaluation and treatment of genital warts. Two patients were complaining of flat-topped papules on their labia major and the third one was presented with asymptomatic papillary projections on her vestibule and inner aspect of both labia minora. Histological examination revealed the diagnosis of syringoma, lymphangioma circumscriptum (LC) and vestibular papillomatosis respectively. Familiarity with these uncommon conditions which clinically mimic genital warts helps to prevent labeling a patient with sexually transmitted disease before histological confirmation and prevent unnecessary treatment.
RESUMO
A 47-year-old woman presented with a history of yellow plaques on her eyelids. These lesions had been diagnosed clinically as xanthelasma and treated five times with topical applications of trichloroacetic acid (TCA) 33 percent. Despite flattening of the original lesions, the patient noticed extension of the lesions on the site of treatment following each session. Skin biopsy showed characteristic findings of xanthelasma. It appears that, in rare instances, xanthelasma palpebrarum may progress following TCA application by a Koebner-like phenomenon.
Assuntos
Cáusticos/efeitos adversos , Doenças Palpebrais/tratamento farmacológico , Ácido Tricloroacético/efeitos adversos , Xantomatose/tratamento farmacológico , Progressão da Doença , Doenças Palpebrais/patologia , Feminino , Humanos , Pessoa de Meia-Idade , Falha de Tratamento , Xantomatose/patologiaRESUMO
BACKGROUND: Longitudinal melanonychia (LM) can be a challenging sign since it may be caused by a wide variety of benign and malignant conditions. Cutaneous melanoma is the most important cause of LM. Objective: We performed this study to examine different aspects of LM in Iran, where cutaneous melanoma is rare. METHODS: In this cross-sectional study, we reviewed medical records and pathology reports of a total of 96 patients presenting with LM. These patients had been visited and undergone nail biopsy in Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran. Demographic, clinical, and pathological data were recorded. RESULTS: The most common diagnosis was junctional nevi in 28 patients (29.2%) followed by melanoma in 19 patients (19.8%). Patients had a mean age of 42.4 years (± 19.4). The mean ages in the groups with junctional nevi and melanoma were 33.3 (± 19.5) and 51.9 (± 17.8), respectively; their difference was statistically significant (P value = 0.001). Hutchinson's sign was present in 10 patients, 9 of which had melanoma. Also, melanoma was only observed in patients presenting with a solitary nail lesion. Nails mostly affected by melanoma were middle fingers of the hands (7 patients) and thumbs (6 patients). Out of 18 patients with nail dystrophy, 13 (72.2%) were diagnosed with melanoma. LIMITATIONS: Only patients who have undergone biopsy were studied. CONCLUSION: Melanoma is an important cause of LM in Iranian patients and should especially be suspected in older patients who present with a solitary nail lesion on their middle finger or thumb. Other findings that direct us toward melanoma are presence of Hutchinson's sign and nail dystrophy.
RESUMO
BACKGROUND: Computer-aided dermoscopy using artificial neural networks has been reported to be an accurate tool for the evaluation of pigmented skin lesions. We set out to determine the sensitivity and specificity of a computer-aided dermoscopy system for diagnosis of melanoma in Iranian patients. METHODS: We studied 122 pigmented skin lesions which were referred for diagnostic evaluation or cosmetic reasons. Each lesion was examined by two clinicians with naked eyes and all of their clinical diagnostic considerations were recorded. The lesions were analyzed using a microDERM dermoscopy unit. The output value of the software for each lesion was a score between 0 and 10. All of the lesions were excised and examined histologically. RESULTS: Histopathological examination revealed melanoma in six lesions. Considering only the most likely clinical diagnosis, sensitivity and specificity of clinical examination for diagnosis of melanoma were 83% and 96%, respectively. Considering all clinical diagnostic considerations, the sensitivity and specificity were 100% and 89%. Choosing a cut-off point of 7.88 for dermoscopy score, the sensitivity and specificity of the score for diagnosis of melanoma were 83% and 96%, respectively. Setting the cut-off point at 7.34, the sensitivity and specificity were 100% and 90%. CONCLUSION: The diagnostic accuracy of the dermoscopy system was at the level of clinical examination by dermatologists with naked eyes. This system may represent a useful tool for screening of melanoma, particularly at centers not experienced in the field of pigmented skin lesions.
Assuntos
Dermoscopia/métodos , Diagnóstico por Computador , Melanoma/diagnóstico , Neoplasias Cutâneas/diagnóstico , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Sensibilidade e EspecificidadeRESUMO
Syringomas are benign adnexal tumors derived from the intraepidermal portion of eccrine sweat ducts. Usually, they present as soft, flesh-colored to slightly yellow dermal papules on the lower eyelids of healthy individuals. We report an 18-year-old man with rare presentation of eruptive syringomas involving his trunk and extremities, with linear arrangement on the arms and forearms. A biopsy obtained from the lesions of the dorsum of the hands showed eccrine syringoma with a lymphocytic inflammatory infiltration around superficial blood vessels and eccrine ducts. We used the 585-nm and 595-nm pulsed dye laser for treatment of inflammatory lesions of forearm and trunk with no success.
Assuntos
Neoplasias das Glândulas Sudoríparas/patologia , Siringoma/patologia , Adolescente , Humanos , MasculinoRESUMO
Anti-desmoglein 3 and 1 autoantibodies are involved in the pathogenesis of pemphigus diseases. Our objective was to assess the value of ELISA in the diagnosis of pemphigus and its correlation with the severity of pemphigus vulgaris. Based on clinical presentation and histopathologic confirmation for the diagnosis of the pemphigus, 38 patients took part in the study. Sera of the patients were tested by desmoglein 1 and desmoglein 3 ELISA. Also, direct immunofluorescence was performed for all patients which revealed positive results in 36 patients (94.7%). ELISA was positive in 37 of 38 pemphigus patients (Sensitivity: 97.3%). The relationship between desmoglein 1 index values and skin severity was statistically significant (p<0.05). Desmoglein 3 index values increased with oral severity although this was not statistically significant. Iranian patients similar to Indian patients had higher positive anti-desmoglein 1 autoantibodies. Desmoglein-ELISA test is appropriate in the diagnosis of pemphigus. Desmoglein 1 index value is statistically correlated with the severity of pemphigus vulgaris.
Assuntos
Desmogleínas/imunologia , Pênfigo/diagnóstico , Adolescente , Adulto , Idoso , Autoanticorpos/sangue , Autoanticorpos/imunologia , Desmogleína 1/imunologia , Desmogleína 3/imunologia , Ensaio de Imunoadsorção Enzimática , Feminino , Técnica Direta de Fluorescência para Anticorpo , Humanos , Masculino , Pessoa de Meia-Idade , Boca/patologia , Pênfigo/imunologia , Pênfigo/patologia , Pele/patologia , Adulto JovemRESUMO
BACKGROUND: Pemphigus vulgaris (PV) is a rare autoimmune blistering disease of the skin and mucous membranes. It varies in its clinical profile and epidemiologic characteristics in different parts of the world. OBJECTIVE: To determine the clinical features of PV in Iran in a prospective manner. METHODS: The study included 140 patients with newly registered PV attending our dermatology clinic between January 2003 and June 2004. RESULTS The mean age at the onset of the disease was 41.5 +/- 15.7 years, with a female to male ratio of 1.59 : 1. At presentation to our clinic, both skin and mucosal involvement was observed in 95 patients (67.9%). Cutaneous lesions without the involvement of the mucous membranes were seen in nine patients (6.4%), and exclusive mucosal involvement was present in 36 patients (25.7%). The most common initial localization of the disease was the oral cavity, which was involved in 93 patients (77.5%). The most frequent cutaneous and mucosal sites involved were the thorax and oral cavity, respectively. CONCLUSION: Although minor differences were noted, the results of this study are in relatively good agreement with the literature with regard to the age, gender, and initial presentation of PV in Iran. Some skin sites, such as the scalp, thorax, and axilla, may be more commonly affected in men. Patients with initial mucosal lesions were significantly younger than those with initial cutaneous involvement. Mucosal lesions other than the oral mucosa may be more common than previously thought.