RESUMO
Tuberous sclerosis complex (TSC) is an autosomal dominant neurocutaneous syndrome causing hamartomatous growths in multiple organs. Facial angiofibromas occur in up to 80% of patients and can be highly disfiguring. Treatment for these lesions is challenging. Recently, topical rapamycin has been proposed as an effective option to treat angiofibromas but a commercially available compound has not yet been developed in Europe. We conducted a retrospective review with the aim to update the current data on the use of topical rapamycin in the treatment of angiofibromas in TSC, focusing on the optimal concentration and trying to establish which vehicle should be preferred. Thirty-nine reports describing the use of topical rapamycin in the treatment of angiofibromas in TSC were considered, involving a total of 483 patients. An improvement of the lesions has been shown in over 90% of subjects, particularly if the treatment was started at early stages. Several different formulations (ointment, gel, solution and cream) with a wide range of concentrations (0.003%-1%) were proposed, of which a pharmacological analysis has also been performed. Topical rapamycin can be considered an effective and safe option for the treatment and the prevention of facial angiofibromas in younger patients, but the best formulation has yet to be established. Our review demonstrates that ointment and gel should be preferred, but it is not clear which concentration is optimal. However, according to this study, the 0.1% concentration represents the first choice. Long-term and comparative studies between topical rapamycin formulations are required in order to establish which treatment has a better outcome and lower recurrence rate.
Assuntos
Angiofibroma , Neoplasias Faciais , Esclerose Tuberosa , Humanos , Sirolimo/uso terapêutico , Inibidores de MTOR , Esclerose Tuberosa/complicações , Esclerose Tuberosa/tratamento farmacológico , Pomadas/uso terapêutico , Angiofibroma/complicações , Angiofibroma/tratamento farmacológico , Neoplasias Faciais/complicações , Neoplasias Faciais/tratamento farmacológico , Imunossupressores/uso terapêutico , Serina-Treonina Quinases TORRESUMO
The outbreak of chilblain-like lesions (CLL) coincidentally to the COVID-19 pandemic is a topic of great concern. SARS-CoV-2 was initially hypothesized as the etiologic agent of CLL, but, since nasopharyngeal swabs seldom resulted positive, dermatologists' attention focused on the search for specific SARS-CoV-2 antibodies. Many papers were published contemporarily on this topic, reporting limited case series. We reviewed the English literature up to the first July 2020 and, excluding single case reports, we considered 13 studies that serologically investigated 220 patients. The presence of specific antibodies was detected in 18 subjects (8.2%): isolated IgA were found in 6 patients, IgA and IgG in 1, isolated IgG in 5, and IgM in 2. In 4 patients, isotypes were not specified. Our review demonstrated a high prevalence of negative serological results in CLL: antibodies were observed only in a few patients, that are even less excluding those with positive IgA, not clearly involved in the pathogenesis of the disease. In conclusion, although it is still uncertain whether CLL are related to SARS-CoV-2 infection, patients affected by CLL seem not to be prone to shedding the virus, hence, if they are asymptomatic, we can reassure them, thus avoiding hospital referral.
Assuntos
Anticorpos Antivirais/sangue , Anticorpos/sangue , Teste Sorológico para COVID-19 , COVID-19/diagnóstico , Pérnio/diagnóstico , SARS-CoV-2/imunologia , Biomarcadores/sangue , COVID-19/epidemiologia , COVID-19/imunologia , COVID-19/virologia , Pérnio/epidemiologia , Pérnio/imunologia , Pérnio/virologia , Interações Hospedeiro-Patógeno , Humanos , Valor Preditivo dos Testes , Medição de Risco , Fatores de Risco , SARS-CoV-2/patogenicidade , Estudos SoroepidemiológicosRESUMO
The use of biotechnological therapies for moderate-to-severe psoriasis is ever-expanding and it is becoming increasingly more frequent to encounter different unforeseen events during their use, such as fertile patients becoming pregnant and breastfeeding, development of infections due to personal habits like tuberculosis, hepatitis B virus, hepatitis C virus, or HIV, scheduling of surgical procedures, need of vaccinations, development of malignancy, and evaluation of dose tapering. As any clinician may experience at least one of these unexpected events, it should be good practice to know how to manage them. Thus, a practical analysis has been proposed in this study.
Assuntos
Infecções por HIV , Mycobacterium tuberculosis , Neoplasias , Psoríase , Aleitamento Materno , Redução da Medicação , Feminino , Infecções por HIV/diagnóstico , Infecções por HIV/tratamento farmacológico , Hepacivirus , Vírus da Hepatite B , Humanos , Gravidez , Psoríase/diagnóstico , Psoríase/tratamento farmacológico , Vacinação/efeitos adversosAssuntos
Alitretinoína/administração & dosagem , Fármacos Dermatológicos/administração & dosagem , Ceratodermia Palmar e Plantar/tratamento farmacológico , Feminino , Humanos , Ceratodermia Palmar e Plantar/patologia , Pessoa de Meia-Idade , Doença de Papillon-Lefevre/tratamento farmacológico , Doença de Papillon-Lefevre/patologiaAssuntos
Fibroma/diagnóstico , Dedos/patologia , Feminino , Fibroma/patologia , Humanos , Pessoa de Meia-IdadeAssuntos
Melanoma/epidemiologia , Neoplasias Cutâneas/epidemiologia , Fator de Necrose Tumoral alfa/antagonistas & inibidores , Fármacos Dermatológicos/efeitos adversos , Fármacos Dermatológicos/farmacologia , Fármacos Dermatológicos/uso terapêutico , Humanos , Psoríase/tratamento farmacológico , Estudos Retrospectivos , RiscoAssuntos
Adalimumab/administração & dosagem , Produtos Biológicos/administração & dosagem , Fármacos Dermatológicos/administração & dosagem , Etanercepte/administração & dosagem , Infecções por HIV/imunologia , Psoríase/tratamento farmacológico , Fator de Necrose Tumoral alfa/antagonistas & inibidores , Ustekinumab/administração & dosagem , Adalimumab/efeitos adversos , Adulto , Idoso , Produtos Biológicos/efeitos adversos , Contagem de Linfócito CD4 , Fármacos Dermatológicos/efeitos adversos , Etanercepte/efeitos adversos , Feminino , Infecções por HIV/diagnóstico , Humanos , Hospedeiro Imunocomprometido , Itália , Masculino , Pessoa de Meia-Idade , Psoríase/diagnóstico , Psoríase/imunologia , Recidiva , Indução de Remissão , Índice de Gravidade de Doença , Fatores de Tempo , Resultado do Tratamento , Fator de Necrose Tumoral alfa/imunologia , Ustekinumab/efeitos adversos , Carga ViralAssuntos
Fatores Biológicos/uso terapêutico , Fármacos Dermatológicos/uso terapêutico , Neoplasias/epidemiologia , Psoríase/tratamento farmacológico , Idoso , Fatores Biológicos/efeitos adversos , Fármacos Dermatológicos/efeitos adversos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias/etiologia , Estudos RetrospectivosAssuntos
Tonsila Faríngea/patologia , Budesonida/efeitos adversos , Dermatite Alérgica de Contato , Eritema Multiforme/diagnóstico , Face/patologia , Hidrocortisona/análogos & derivados , Antialérgicos/administração & dosagem , Antialérgicos/efeitos adversos , Budesonida/administração & dosagem , Pré-Escolar , Dermatite Alérgica de Contato/diagnóstico , Dermatite Alérgica de Contato/etiologia , Dermatite Alérgica de Contato/fisiopatologia , Diagnóstico Diferencial , Glucocorticoides/administração & dosagem , Glucocorticoides/efeitos adversos , Humanos , Hidrocortisona/administração & dosagem , Hidrocortisona/efeitos adversos , Hipertrofia/tratamento farmacológico , Masculino , Nebulizadores e Vaporizadores , Testes Cutâneos/métodosRESUMO
Bullous pemphigoid (BP) is an autoimmune subepidermal blistering disorder that has been reported to be associated with malignancies. Some authors described several cases of pemphigoid associated with malignancies (PAM); however, the evidence of this correlation still remains controversial. Several theories have been postulated to explain the relationship between malignant neoplasms and BP; the main theory suggests that antibodies directed against tumor-specific antigens of malignant cells may cross-react with antigens (like BP antigens) in the basement membrane zone leading to the formation of blisters. We performed an extensive review of the English published work focusing on the epidemiology, the pathogenetic theories and the clinical and histological aspects of the disease. We identified 40 cases of PAM: of these, seven cases were associated with hematological malignancies and 33 with solid tumors. Physicians should be aware of the existence of PAM and we suggest an oncological screening in early-onset pemphigoid, in patients with a former oncological history, in those with signs and symptoms that could be related to a neoplasm and in BP refractory to common immunosuppressive therapy.
Assuntos
Neoplasias/complicações , Penfigoide Bolhoso/complicações , Feminino , Neoplasias Hematológicas/complicações , Humanos , Masculino , Modelos Biológicos , Neoplasias/epidemiologia , Neoplasias/etiologia , Síndromes Paraneoplásicas/complicações , Penfigoide Bolhoso/imunologiaRESUMO
IMPORTANCE: Differential diagnosis between congenital Volkmann ischemic contracture (CVIC) and unilateral aplasia cutis congenita (ACC) type VII of the forearm presents a clinical challenge. Both diseases share the same clinical presentation characterized by a stellate ulceration over the upper extremities and reported association with neuromuscular defects, but the diagnostic criteria to differentiate these 2 entities remain unclear. OBSERVATIONS: A newborn girl presented with an ulceration of the left forearm associated with an apparent neurological impairment. On the basis of the suspected neurological involvement, a diagnosis of CVIC was initially considered, but because the neurological evaluation excluded paralysis, our final diagnosis was ACC type VII. CONCLUSIONS AND RELEVANCE: In our opinion, CVIC should be considered a particular form of ACC in which an external noxa affects the forearm, increasing the intracompartmental pressure and leading to muscle and nerve ischemia. Therefore, we propose that the definition of Volkmann ischemic contracture should be maintained only for the acquired forms with an evident etiology and that Frieden's classification scheme for ACC type VII needs to be reformulated.
Assuntos
Displasia Ectodérmica/diagnóstico , Contratura Isquêmica/congênito , Contratura Isquêmica/diagnóstico , Diagnóstico Diferencial , Feminino , Antebraço , Humanos , Recém-NascidoRESUMO
Extramammary Paget's disease is a rare neoplasm of apocrine gland-bearing areas of the skin. The most common site of presentation is the vulva. Surgery is the most frequently reported therapy so far; however, it is invasive and it is complicated by a high rate of recurrence. For this reason, several less-invasive treatments have been recently proposed, including photodynamic therapy. We describe in this article the case of an 84-year-old patient with a noninvasive vulvar extramammary Paget's disease successfully treated with methyl-aminolevulinic acid photodynamic therapy associated with topical tretinoin.