RESUMO
Chronic granulomatous disease (CGD) is a primaryimmunodeficiency disorder that affects the phagocyticcells of the innate immune system. It is characterizedby recurrent or persistent infections with granulomaformation. Lupus-like lesions have been reported incarriers of CGD and less frequently, in patients withCGD. Immunological study in these patients areusually negative. We describe the case of an 8-yearoldboy with CGD who developed chronic and acutecutaneous lupus erythematous with angular cheilitis,oral ulcers, Raynaud phenomenon, and positiveserologies for antinuclear, anticentromere, and anti-Saccharomyces cerevisiae antibodies.
Assuntos
Dermatoses Faciais/diagnóstico , Dermatoses do Pé/diagnóstico , Doença Granulomatosa Crônica/imunologia , Lúpus Eritematoso Cutâneo/diagnóstico , Anticorpos Antinucleares/imunologia , Anticorpos Antifúngicos/imunologia , Queilite/complicações , Queilite/diagnóstico , Queilite/imunologia , Criança , Dermatoses Faciais/complicações , Dermatoses Faciais/imunologia , Dermatoses Faciais/patologia , Dermatoses do Pé/complicações , Dermatoses do Pé/imunologia , Dermatoses do Pé/patologia , Doença Granulomatosa Crônica/complicações , Humanos , Lúpus Eritematoso Cutâneo/complicações , Lúpus Eritematoso Cutâneo/imunologia , Lúpus Eritematoso Cutâneo/patologia , Masculino , Úlceras Orais/complicações , Úlceras Orais/diagnóstico , Úlceras Orais/imunologia , Doença de Raynaud/complicações , Doença de Raynaud/diagnóstico , Doença de Raynaud/imunologia , Fatores de Risco , Saccharomyces cerevisiae/imunologiaRESUMO
Pyodermatitis vegetans (PV) is a rare inflammatory dermatosis of unknown etiology and difficult diagnosis characterized by vesiculopustular, exudative, and vegetating plaques usually localized in the axillary, genital, and oral region. It bears similarities to pemphigus vegetans but clinical history, histology, and immunofluorescence help confirm the diagnosis. Pyodermatitis vegetans is often associated with an underlying gastrointestinal disorder, especially ulcerative colitis. No standardized treatment plan is available for PV. Its evolution tends to follow the progression of the underlying disorder. We report a patient with vulvar PV with good response to systemic antibiotics and corticosteroids.
Assuntos
Combinação Amoxicilina e Clavulanato de Potássio/uso terapêutico , Antibacterianos/uso terapêutico , Anti-Inflamatórios/uso terapêutico , Prednisolona/uso terapêutico , Pioderma/tratamento farmacológico , Doenças da Vulva/tratamento farmacológico , Idoso , Quimioterapia Combinada , Feminino , Humanos , Pioderma/patologia , Doenças da Vulva/patologiaRESUMO
Neurofibromatosis is extremely variable in its presentation. Segmental neurofibromatosis (SNF), which corresponds to NF-type 5 in the Riccardi classification, is a rare disorder. It may go unrecognized if few lesions are observed. We present a case of segmental neurofibromatosis in a 28 year-old patient who presented with multiple papules and nodules distributed in dermatomal fashion on the trunk. The histopathological examination of the lesions revealed a non-encapsulated, well-circumscribed spindle cell neoplasm, which was consistent with neurofibromas.
Assuntos
Neurofibromatoses/patologia , Neoplasias Cutâneas/patologia , Adulto , Humanos , Masculino , Doenças Raras , Pele/patologia , SíndromeRESUMO
Abstract Neurofibromatosis is extremely variable in its presentation. Segmental neurofibromatosis (SNF), which corresponds to NF-type 5 in the Riccardi classification, is a rare disorder. It may go unrecognized if few lesions are observed. We present a case of segmental neurofibromatosis in a 28 year-old patient who presented with multiple papules and nodules distributed in dermatomal fashion on the trunk. The histopathological examination of the lesions revealed a non-encapsulated, well-circumscribed spindle cell neoplasm, which was consistent with neurofibromas.
Assuntos
Humanos , Masculino , Adulto , Neoplasias Cutâneas/patologia , Neurofibromatoses/patologia , Pele/patologia , Síndrome , Doenças RarasRESUMO
Pityriasis rubra pilaris is a chronic inflammatory dermatosis of unknown etiology and great clinical variability. It has been divided into six categories. Types III, IV, and V occur in childhood and are distinguished by their clinical presentation, age of onset, and course. We report a 19-year-old male patient with a 2-week history of pruritic, scaling dermatosis of the hands, feet, elbows, and knees. He had no family history of skin disease. On physical examination, we observed circumscribed, reddish-orange, scaling plaques affecting the elbows and knees and a waxy palmoplantar keratoderma. The skin biopsy showed acanthosis, alternating orthokeratosis, parakeratosis, and follicular plugging suggestive of pityriasis rubra pilaris. The patient started treatment with oral acitretin, 25 mg every other day. The treatment was tolerated well, and after 6 months the lesions had resolved completely. Pityriasis rubra pilaris is a chronic papulosquamous disorder of unknown pathogenesis, characterized by reddish-orange scaly plaques, palmoplantar keratoderma, and keratotic follicular papules. There is still no consensus regarding the treatment, but therapeutic options include systemic retinoids, particularly acitretin in the recommended dose of 0.5 to 0.75 mg/kg/day. In our case, the patient was treated with a low-dose regimen of acitretin, which was effective and well tolerated.
Assuntos
Acitretina/uso terapêutico , Ceratolíticos/uso terapêutico , Pitiríase Rubra Pilar/tratamento farmacológico , Humanos , Masculino , Pitiríase Rubra Pilar/patologia , Adulto JovemAssuntos
Anormalidades Linfáticas/diagnóstico , Criança , Diagnóstico Diferencial , Exantema/etiologia , Humanos , Masculino , PescoçoRESUMO
Brachioradial pruritus is a chronic sensory neuropathy of unknown etiology which affects the skin of the shoulders, arms and forearms on the insertion of the brachioradialis muscle. We describe the case of a 60-year old woman recently diagnosed with multiple myeloma who refers paresis, severe pruritus and itching lesions on the right arm with 6 months of evolution. Investigation led to a diagnosis of Brachioradial pruritus consequent to the presence of cervical disc herniation and Parsonage-Turner syndrome. The patient started gabapentin 900 mg/day with good control of itching. Corticosteroids and antihistamines are often ineffective in the treatment of BP. Gabapentin has been used with encouraging results. All patients with Brachioradial pruritus should be evaluated for cervical spine injuries.
Assuntos
Neurite do Plexo Braquial , Vértebras Cervicais , Deslocamento do Disco Intervertebral/diagnóstico , Prurido/patologia , Biópsia , Neurite do Plexo Braquial/complicações , Neurite do Plexo Braquial/diagnóstico , Feminino , Humanos , Deslocamento do Disco Intervertebral/complicações , Pessoa de Meia-Idade , Prurido/tratamento farmacológico , Pele/patologiaRESUMO
Abstract Brachioradial pruritus is a chronic sensory neuropathy of unknown etiology which affects the skin of the shoulders, arms and forearms on the insertion of the brachioradialis muscle. We describe the case of a 60-yearold woman recently diagnosed with multiple myeloma who refers paresis, severe pruritus and itching lesions on the right arm with 6 months of evolution. Investigation led to a diagnosis of Brachioradial pruritus consequent to the presence of cervical disc herniation and Parsonage-Turner syndrome. The patient started gabapentin 900mg/day with good control of itching. Corticosteroids and antihistamines are often ineffective in the treatment of BP. Gabapentin has been used with encouraging results. All patients with Brachioradial pruritus should be evaluated for cervical spine injuries.
Assuntos
Feminino , Humanos , Pessoa de Meia-Idade , Neurite do Plexo Braquial , Vértebras Cervicais , Deslocamento do Disco Intervertebral/diagnóstico , Prurido/patologia , Biópsia , Neurite do Plexo Braquial/complicações , Neurite do Plexo Braquial/diagnóstico , Deslocamento do Disco Intervertebral/complicações , Prurido/tratamento farmacológico , Pele/patologiaRESUMO
Calcinosis cutis is a rare condition characterized by the deposition of insoluble calcium salts in the skin and subcutaneous tissue. Dystrophic calcinosis cutis appears as a result of local tissue damage or abnormalities, such as alterations in extra-cellular matrix proteins or subcutaneous tissue with normal calcium and phosphate serum levels. It has been rarely described as a late complication of burns. Latency periods of 15-54 years have been reported. We describe the case of a 57-year-old man with dystrophic calcinosis cutis in a burn scar, which developed 42 years after the skin injury. The condition was successfully treated with surgical excision.