RESUMO
A 23-year-old woman presented with a hyperpigmented intertriginous eruption unresponsive to topical steroids and to topical and systemic antifungals. Histopathological assessment demonstrated patchy inflammatory infiltrate at the demoepidermal junction with pigment incontinence. Click here for the corresponding questions to this CME article.
Assuntos
Exantema , Hiperpigmentação , Feminino , Humanos , Adulto Jovem , Adulto , Hiperpigmentação/diagnósticoRESUMO
We present a case of cyclical periorbital angio-oedema, and highlight the appropriate investigations to support the clinical diagnosis of this rare dermatosis.
Assuntos
Angioedema , Dermatite Atópica , Angioedema/diagnóstico , HumanosRESUMO
Intravenous immunoglobulin (IVIg) is increasingly used across multiple specialties for the treatment of inflammatory and autoimmune diseases. Cutaneous reactions to IVIg are generally minor. Pompholyx is a common eruption of small vesicles on the palms, soles, and/or lateral aspects of the fingers. It has a multifactorial aetiology but is rarely attributed to being a drug-related side effect. We describe a 43-year-old woman presenting with peripheral sensory neuropathy who developed pompholyx eczema on both palms following treatment with IVIg.
Assuntos
Doenças Autoimunes , Efeitos Colaterais e Reações Adversas Relacionados a Medicamentos , Eczema Disidrótico , Doenças do Sistema Nervoso Periférico , Adulto , Doenças Autoimunes/tratamento farmacológico , Eczema Disidrótico/induzido quimicamente , Eczema Disidrótico/tratamento farmacológico , Feminino , Humanos , Imunoglobulinas Intravenosas/efeitos adversos , Doenças do Sistema Nervoso Periférico/induzido quimicamenteRESUMO
Clear cell acanthoma (CCA) is a rare, benign epidermal lesion of clear glycogen-containing keratinocytes. The exact etiology is unknown. Although CCA usually appear as solitary lesions, cases of multiple CCAs have also been described. The rare eruptive CCA variant describes cases with more than 30 lesions.Current therapeutic evidence for the management of multiple CCAs is limited. CO2 laser remains the most widely used laser in clinical practice. However, literature describing its effectiveness on CCA is sparse, and previous reports have described its use for a maximum of three lesions. To our knowledge, this is the first report of successful CO2 laser treatment of the multiple eruptive CCA variant with an excellent cosmetic outcome.Our case adds to the evidence that CO2 laser is an effective treatment for this condition. Particularly in those patients with refractory or multiple eruptive CCAs, or for those who may not tolerate repeated courses of cryotherapy, or who are concerned about scarring and cosmetic outcome.
Assuntos
Acantoma , Exantema , Terapia a Laser , Lasers de Gás , Neoplasias Cutâneas , Acantoma/patologia , Acantoma/cirurgia , Dióxido de Carbono , Humanos , Lasers de Gás/uso terapêutico , Neoplasias Cutâneas/patologia , Neoplasias Cutâneas/cirurgiaRESUMO
Omenn syndrome is a rare combined immunodeficiency mostly associated with RAG1 and RAG2 mutations; the clinical manifestations are well-described and include neonatal erythroderma. Mortality due to opportunistic infections is a serious risk, and a timely diagnosis with a skin biopsy is an important part of the diagnostic workup. We wish to highlight key clinical features of Omenn syndrome and discuss the relevance of a skin biopsy.
Assuntos
Dermatite Esfoliativa , Imunodeficiência Combinada Severa , Proteínas de Homeodomínio/genética , Humanos , Recém-Nascido , Mutação , Imunodeficiência Combinada Severa/diagnóstico , SíndromeAssuntos
Dapsona/uso terapêutico , Vasculite Leucocitoclástica Cutânea/tratamento farmacológico , Vasculite Leucocitoclástica Cutânea/patologia , Biópsia por Agulha , Humanos , Imuno-Histoquímica , Dermatoses da Perna/diagnóstico , Dermatoses da Perna/etiologia , Masculino , Pessoa de Meia-Idade , Doenças Raras , Índice de Gravidade de Doença , Resultado do Tratamento , Vasculite Leucocitoclástica Cutânea/diagnósticoRESUMO
A neonatal boy presented with a persistent urticarial rash. Initial investigations showed raised inflammatory markers and evidence of systemic inflammation. A working diagnosis of cryopyrin-associated periodic syndrome (CAPS) was made, and the patient responded extremely well to Anakinra. Molecular genetic testing revealed a somatic mutation (affecting 12.5% of cells) in the NLRP3 gene, accounting for the persistent inflammatory state but milder phenotype as seen in our patient.