RESUMO
Neutrophilic eccrine hidradenitis (NEH) is a rare neutrophilic dermatosis, first described in patients undergoing chemotherapy for a malignant haemopathy. It has polymorphous clinical features and the association of both clinical and histological features is necessary to make a diagnosis. We report the first two cases of NEH in patients treated with a BRAF inhibitor (BRAFi), either dabrafenib or vemurafenib, for a stage IV metastatic melanoma. Disseminated erythematous plaques associated with fever and polyarthralgia occurred early after the initiation of treatment and were badly tolerated. Histological analyses confirmed the diagnosis of NEH. Symptoms disappeared a few days after the cessation of treatment and introduction of topical steroids. The replacement of one BRAFi with another is a therapeutic alternative as it is not necessarily associated with a relapse of NEH. NEH can be added to the spectrum of neutrophilic dermatoses induced by BRAFis. It occurs earlier (3-4 days) than previously described drug-induced NEH (9-12 days) and may be an earlier stage of eccrine squamous syringometaplasia, which has already been reported in the context of BRAFi-treated patients.
Assuntos
Antineoplásicos/efeitos adversos , Toxidermias/etiologia , Hidradenite/induzido quimicamente , Imidazóis/efeitos adversos , Indóis/efeitos adversos , Oximas/efeitos adversos , Sulfonamidas/efeitos adversos , Adulto , Feminino , Humanos , Masculino , Melanoma/tratamento farmacológico , Neoplasias Cutâneas/tratamento farmacológico , Vemurafenib , Adulto JovemRESUMO
BACKGROUND: Although varicelliform Kaposi eruption is a well-known complication of dermatoses, it has not been widely investigated. AIM: To investigate features of dermatoses and herpes superinfection in patients hospitalized in a dermatology department. PATIENTS AND METHODS: We performed a single-centre, retrospective study between 2008 and 2014 that included cases of Kaposi varicelliform eruptions defined by positive PCR of an unconventional site of herpetic recurrence in a setting of active dermatitis. A record was compiled of each case giving details of the history, clinical and laboratory findings, therapeutic data and outcome. RESULTS: Thirty-four cases of Kaposi varicelliform eruptions in 30 subjects were studied. Mean age at diagnosis was 63.3±24.2 years. The underlying dermatoses were as follows: 7 pemphigus, 6 bullous pemphigoid, 3 cicatricial pemphigoid, 3 atopic dermatitis, 1 Darier disease, and 14 other dermatoses. Patients presented with skin (94.1 %) or mucous membrane lesions (62 %), mostly erosive (79 %), vesicular (27 %) or bullous (41 %), often painful (56 %) or pruritic (29 %). At diagnosis, 41.2 % were undergoing systemic immunotherapy and 24 % were on topical corticosteroids. PCR was positive for HSV1 in 20 cases and for HSV2 in 4 cases, and indeterminate in 10 cases. Lymphocytopenia was seen in 59 % of cases. The majority of patients received treatment. Nine patients experienced at least one relapse. CONCLUSION: Our study confirms the over-representation not only of the expected dermatoses (pemphigus and atopic dermatitis), but also of others such as pemphigoid and acute dermatoses; these results should be investigated in a more systematic prospective study.
Assuntos
Hospedeiro Imunocomprometido , Pacientes Internados , Erupção Variceliforme de Kaposi/diagnóstico , Dermatopatias/diagnóstico , Superinfecção , Administração Cutânea , Adulto , Idoso , Idoso de 80 Anos ou mais , Antivirais/administração & dosagem , Diagnóstico Diferencial , Quimioterapia Combinada , Feminino , Glucocorticoides/administração & dosagem , Humanos , Erupção Variceliforme de Kaposi/complicações , Erupção Variceliforme de Kaposi/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Dermatopatias/complicações , Dermatopatias/tratamento farmacológico , Resultado do TratamentoAssuntos
Antivirais/efeitos adversos , Síndrome de Hipersensibilidade a Medicamentos/etiologia , Valaciclovir/efeitos adversos , Idoso , Clobetasol/uso terapêutico , Síndrome de Hipersensibilidade a Medicamentos/diagnóstico , Síndrome de Hipersensibilidade a Medicamentos/tratamento farmacológico , Feminino , Infecções por Herpesviridae/tratamento farmacológico , Infecções por Herpesviridae/prevenção & controle , Humanos , Masculino , Pessoa de Meia-Idade , Testes do EmplastroRESUMO
Nail-fold capillaroscopy is a non-invasive tool to study the microcirculation and is increasingly being used in dermatology, angiology and rheumatology. More recently, the use of video-capillaroscopy has allowed computer storage of capillaroscopic images (video-capillaroscopy), enabling evaluation of changes in capillaroscopic abnormalities during the follow-up of patients with systemic sclerosis or mixed connective tissue disease. Qualitative and quantitative assessment of the nail-fold dermal capillaries and of their organization can readily distinguish between a normal capillaroscopic pattern in primary Raynaud phenomenon and a specific sclerodermic pattern in secondary Raynaud phenomenon carrying a very high risk of systemic sclerosis. Apart from its important role as a diagnostic tool for distinguishing between primary and secondary Raynaud phenomenon, capillaroscopy is now used to predict the risk of development of digital ulcers and of future visceral complications in patients with systemic sclerosis. Moreover, nail-fold capillaroscopy is essential for differential diagnosis between connective tissue diseases, for the etiologic diagnosis of digital necrosis and diffuse interstitial lung disease, and in sclerodermiform syndromes.
Assuntos
Dermatologia/métodos , Dedos/irrigação sanguínea , Angioscopia Microscópica/métodos , Escleroderma Sistêmico/diagnóstico , Capilares/ultraestrutura , Cianose/diagnóstico , Cianose/patologia , Diagnóstico Precoce , Hemorragia/diagnóstico , Hemorragia/patologia , Humanos , Microcirculação , Doenças da Unha/diagnóstico , Doenças da Unha/patologia , Unhas , Razão de Chances , Doença de Raynaud/diagnóstico , Doença de Raynaud/patologia , Risco , Escleroderma Sistêmico/patologia , Úlcera Cutânea/diagnóstico , Úlcera Cutânea/patologiaRESUMO
BACKGROUND: Herein, we report the first case of kaposiform haemangioendothelioma (KHE) associated with acute B-lymphoblastic leukemia (B-ALL). PATIENTS AND METHODS: A five-month-old infant presented a plaque of angiomatous appearance on the forearm that had increased in volume since birth, as well as pallor and cutaneous haematomas. Kasabach-Merritt syndrome (KMS) was evoked despite hepatomegaly and considerable splenomegaly. Laboratory tests revealed severe anaemia and thrombocytopenia as well as major hyperleukocytosis with 90% blasts. Skin biopsy revealed vast vascular lobules containing cohesive fusiform endothelial cells not expressing Glut1, bound up in a dense infiltrate of B-lymphoblast cells. It was in fact KHE associated with B-ALL confirmed by the myelogram. The child was treated with the INTERFANT 2006 protocol followed by allograft of haematopoietic stem cells, which resulted in complete haematological remission. At the same time, almost total regression of KHE was noted. DISCUSSION: In this infant, KHE had an inflammatory appearance and was associated with thrombocytopenia, evocative of KMS. Analysis of blood and marrow samples resulted in a diagnosis of B-ALL. Histopathological examination of the angioma revealed a typical appearance of KHE associated with dense lymphoblastic proliferation. This appearance could have resulted either from passive contamination by circulating blast cells or from active recruitment of tumor cells at the KHE site. CONCLUSION: HK mimicking KMS may reveal B-ALL.