RESUMEN
BACKGROUND: Scleredema adultorum of Buschke is a rare disease characterized by firm and non-pitting edema of the skin. The condition is rare with unknown etiology. Diagnosis is made on the basis of clinical findings and skin biopsy. CASE PRESENTATION: Here, we describe a 14-year-old Iranian girl presenting with non-pitting edema and woody thickening of the skin that progressed within a month. She was evaluated for possible underlying malignancy or connective tissue disorders, which were excluded by multiple laboratory workups. She underwent a skin biopsy which confirmed the diagnosis of scleredema, and she was successfully treated with intravenous immunoglobulin and mycophenolate mofetil. CONCLUSION: While scleredema adultorum of Buschke is a rare disease with no definite treatment, our effort through this report was to highlight the possible benefits of treatment by intravenous immunoglobulin and mycophenolate mofetil.
Asunto(s)
Escleredema del Adulto , Femenino , Humanos , Adolescente , Escleredema del Adulto/tratamiento farmacológico , Inmunoglobulinas Intravenosas/uso terapéutico , Ácido Micofenólico/uso terapéutico , Irán , Enfermedades Raras , EdemaRESUMEN
Scleredema adultorum of Buschke is an important differential diagnosis in sclerosing diseases. Diagnosis is based on the typical histology with mucin deposits and grossly increased dermal width and a clinical presentation of diffuse non-pitting induration of the skin starting at the nape of the neck and interscapular region extending to shoulders and upper thorax, causing dysmobility due to dermal stiffness. Even though the pathogenesis remains unclear, three subtypes can be distinguished: association with infections, paraproteins, or most frequently with diabetes mellitus. Management of the disease includes physiotherapy, physical therapies such as ultraviolet (UV) or ionizing irradiation, intravenous immunoglobulins and interdisciplinary treatment directed at associated diseases. Optimizing diabetes therapy and thereby decreasing insulin use may confer significant improvement.
Asunto(s)
Escleredema del Adulto , Enfermedades de la Piel , Humanos , Escleredema del Adulto/diagnóstico , Diagnóstico Diferencial , Enfermedades de la Piel/diagnóstico , Piel/patología , Inmunoglobulinas Intravenosas/uso terapéuticoAsunto(s)
Leucemia Linfocítica Crónica de Células B , Escleredema del Adulto , Escleromixedema , Humanos , Leucemia Linfocítica Crónica de Células B/complicaciones , Leucemia Linfocítica Crónica de Células B/diagnóstico , Escleromixedema/complicaciones , Escleromixedema/diagnóstico , Anciano , MasculinoRESUMEN
cleredema of Buschke is a rare pathological disorder of connective tissue, which is characterized by a woody, diffuse induration of the skin, most often in the upper extremities. We report an extremely rare complication of post-streptococcal infection in a six-year-old male complaining of gradually progressing, painless skin thickening and tightness which was preceded by a one-month history of fever, cough, and tonsillitis. By reporting this case, we hope to contribute to the creation of a database for future research aimed at better understanding the incidence, pathophysiology, and management of this extremely rare complication.
Asunto(s)
Escleredema del Adulto , Masculino , Humanos , Niño , Escleredema del Adulto/etiología , Escleredema del Adulto/complicaciones , Piel/patologíaRESUMEN
Cutaneous mucinoses are a heterogenous group of conditions, characterized by the deposition of glycosaminoglycans (mucin) in the dermis, follicles, or in the epidermis. Major cutaneous mucinoses include lichen myxedematosus, scleredema, mucinoses associated with thyroid disease, reticular erythematous mucinosis, papulonodular mucinosis associated with connective tissue diseases, and cutaneous focal mucinosis. The aim of this review is to provide an update of what has currently been reported in the last 30-year literature about several new or emerging conditions of acquired cutaneous mucinoses in adults. Two new clinico-pathologic entities have been described: (i) Obesity-associated lymphedematous mucinosis and pretibial stasis mucinosis; (OACM) (ii) Nodular mucinosis of the breast (NMB). Two relatively new disease categories encompassing cutaneous mucinoses with a common pathogenetic mechanism have been identified: (i) Cutaneous mucinoses associated with drug exposure including biologic therapy, anti-colony-stimulating factor 1 receptor (CSF1R) and subcutaneous intralesional interferons (toxic dermal mucinoses); (ii) Cutaneous mucinosis following physical agents including mechanical traumas and after knee replacement.
Asunto(s)
Enfermedades del Tejido Conjuntivo , Mucinosis , Escleredema del Adulto , Escleromixedema , Enfermedades de la Piel , Adulto , Mama , Humanos , Mucinosis/etiología , Mucinosis/patología , Enfermedades de la Piel/complicaciones , Enfermedades de la Piel/patologíaRESUMEN
Scleredema adultorum of Buschke is a rare disorder of the connective tissue, involving the skin. Here, we present a 61-year-old male, who is a known case of compensated liver cirrhosis with a past history of being treated for autoimmune thyrotoxicosis, who presented with complaints of alopecia, skin tightening, dry skin, pruritus, and woody indurated plaques on the skin of the upper back, shoulder, and arms. Skin biopsy of the arm revealed the characteristic features of scleredema. He was extensively evaluated for known literature-cited causes of scleredema, and the work up revealed a negative result. He was also found to be hypothyroid on presentation. Hence, we present a case of scleredema occurring in a patient with hypothyroidism and chronic liver disease, which to our knowledge is being described for the first time in literature.
Asunto(s)
Hipotiroidismo , Cirrosis Hepática , Escleredema del Adulto , Humanos , Masculino , Persona de Mediana Edad , Alopecia/etiología , Betametasona/uso terapéutico , Ácido Fusídico/uso terapéutico , Glucocorticoides/uso terapéutico , Hipotiroidismo/complicaciones , Cirrosis Hepática/complicaciones , Prurito/etiología , Escleredema del Adulto/diagnóstico , Tirotoxicosis/complicacionesRESUMEN
Diabetes mellitus is a significant worldwide health concern and cutaneous manifestations are common. This review describes characteristic skin findings of diabetes, general skin findings related to diabetes, and findings related to diabetes treatment with a focus on clinical presentation, diagnosis, pathophysiology, epidemiology, and treatment. As the prevalence of diabetes continues to rise, cutaneous manifestations of diabetes mellitus likely will be encountered more frequently by physicians in all disciplines including dermatologists and primary care physicians. Accordingly, knowledge regarding the prevention, diagnosis, and management of cutaneous manifestations is an important aspect in the care of patients with diabetes.
Asunto(s)
Complicaciones de la Diabetes/patología , Diabetes Mellitus/epidemiología , Enfermedades de la Piel/diagnóstico , Enfermedades de la Piel/prevención & control , Enfermedades de la Piel/fisiopatología , Acantosis Nigricans/etiología , Acantosis Nigricans/patología , Acantosis Nigricans/terapia , Dermatólogos/estadística & datos numéricos , Pie Diabético/etiología , Pie Diabético/patología , Pie Diabético/terapia , Salud Global/estadística & datos numéricos , Humanos , Conocimiento , Lipodistrofia/etiología , Lipodistrofia/patología , Lipodistrofia/terapia , Persona de Mediana Edad , Necrobiosis Lipoidea/etiología , Necrobiosis Lipoidea/patología , Necrobiosis Lipoidea/terapia , Médicos de Atención Primaria/estadística & datos numéricos , Prevalencia , Escleredema del Adulto/etiología , Escleredema del Adulto/patología , Escleredema del Adulto/terapia , Enfermedades de la Piel/epidemiologíaRESUMEN
BACKGROUND: Scleroedema adultorum of Buschke is a rare disorder characterized by fibromucinous thickening of the dermis that manifests mainly at the nape of the neck and on the upper back and shoulders. This study screened patients with diabetes mellitus for skin hardening caused by scleroedema adultorum of Buschke and characterized the clinical and laboratory findings in patients with newly identified cases, with a focus on lipid metabolism abnormalities and vascular complications. METHODS: Out of 113 consecutive patients with diabetes, 11 (9.7%) new scleroedema patients, all with type 2 diabetes, were found. Their clinical and laboratory data were compared to those of the rest of the screened patients and to those of a cohort of 15 patients with scleroedema and diabetes who were already being treated in a tertiary clinical centre at the University of Pécs. RESULTS: Higher proportions of patients with dyslipidaemia, hypertriglyceridemia (P < 0.05) and increased mean levels of non-high-density lipoprotein cholesterol (non-HDL-C) were found (P < 0.01) in both scleroedema groups than in the group without scleroedema. Stroke and venous thromboembolism (VTE) were more frequently found in the histories of both the newly identified scleroedema group (each 3/11; 27.3%) and the treated cohort (each 6/15; 40.0%) than in the group without scleroedema (6/102; 5.9% in cases of stroke P = 0.021, P < 0.001; and 14/102; 13.7%; P < 0.05 in cases of VTE, respectively). Based on binary logistic regression, a high non-HDL-C level (odds ratio (OD): 3.338, confidence interval (CI): 1.77-6.28; P < 0.001) and insulin treatment (OR 7.64, CI 1.9-29.3; P = 0.003) were independent predictors of scleroedema in patients with diabetes mellitus. CONCLUSIONS: Diabetes patients with scleroedema had more severe dyslipidaemia and higher occurrence of vascular complications compared to those without scleroedema. In addition to poorly controlled type 2 diabetes mellitus requiring insulin treatment, high non-HDL-C levels may be another contributing factor to the development of scleroedema. TRIAL REGISTRATION: NCT04335396 .
Asunto(s)
Diabetes Mellitus Tipo 2/complicaciones , Dislipidemias/epidemiología , Escleredema del Adulto/diagnóstico , Dislipidemias/etiología , Femenino , Humanos , Inhibidores de Hidroximetilglutaril-CoA Reductasas/uso terapéutico , Lípidos/sangre , Modelos Logísticos , Masculino , Tamizaje Masivo , Persona de Mediana Edad , Prevalencia , Factores de Riesgo , Escleredema del Adulto/etiología , Escleredema del Adulto/patología , Piel/patologíaRESUMEN
Hyperglycemia is defined by excess blood glucose and, when persistent, may lead to prediabetic and diabetic states. Insulin is a hormone produced by the beta cells of the pancreas in response to elevated blood glucose. Dysregulated insulin secretion or clearance results in hyperinsulinemia, which also is closely associated with type 2 diabetes mellitus (T2DM) and metabolic disturbances. Hyperglycemia and hyperinsulinemia are endemic within the United States and impart considerable morbidity and mortality. Cutaneous manifestations of chronic hyperglycemia and hyperinsulinemia include acanthosis nigricans (AN), diabetic dermopathy (DD), scleredema diabeticorum (SD), ichthyosiform skin changes, acrochordons, and keratosis pilaris (KP). Necrobiosis lipoidica (NL), bullosis diabeticorum (BD), and generalized granuloma annulare (GA) are more rarely reported in association with hyperglycemia and hyperinsulinemia; however, the strength of these associations remains unclear. It is crucial for dermatologists to recognize these cutaneous manifestations, as they may be the first signs of metabolic syndrome and insulin resistance. Early identification and management of these conditions is key to improving patient health outcomes and reducing health care costs. Herein, we review the clinical presentations of these conditions and their underlying pathophysiologic mechanisms.
Asunto(s)
Diabetes Mellitus Tipo 2 , Hiperglucemia , Hiperinsulinismo , Escleredema del Adulto , Enfermedades de la Piel , Diabetes Mellitus Tipo 2/complicaciones , Humanos , Hiperglucemia/complicaciones , Hiperinsulinismo/etiología , Enfermedades de la Piel/etiologíaRESUMEN
Scleredema adultorum of Buschke is a rare skin disease characterized by skin thickening and tightening typically at the neck and the upper part of the body. This thickening results from increased mucin deposition in the reticular dermis. Three variants are recognized. Scleredema diabeticorum is one subtype associated with diabetes mellitus. We report a man with a history of poorly controlled diabetes presenting with extensive scleredema adultorum of the trunk in a "cuirasse" pattern associated with restrictive lung disease. Cutaneous ultrasonography revealed a marked thickening of the dermis as well as various echogenic spots in the dermis. Cutaneous hardness secondary to scleredema may cause limited mobility. Therefore, respiratory manifestations may be associated and assessed, mainly in cases of extensive scleredema involving trunk and shoulders. Moreover, cutaneous ultrasonography may be useful to monitor the evolution of scleredema and confirm the diagnosis.
Asunto(s)
Complicaciones de la Diabetes/patología , Trastornos Respiratorios/etiología , Escleredema del Adulto/complicaciones , Escleredema del Adulto/patología , Humanos , Masculino , Persona de Mediana Edad , Escleredema del Adulto/diagnóstico por imagen , Torso/patología , UltrasonografíaAsunto(s)
Heparina/administración & dosificación , Escleredema del Adulto/tratamiento farmacológico , Esclerema Neonatal/tratamiento farmacológico , Vitamina E/uso terapéutico , Vitaminas/uso terapéutico , Administración Tópica , Antioxidantes , Preescolar , Femenino , Heparina/uso terapéutico , Humanos , Masculino , Crema para la Piel , Vitamina E/administración & dosificación , Vitaminas/administración & dosificaciónRESUMEN
Scleroedema is a rare clinical condition characterised by diffuse woody induration of skin commonly associated with diabetes mellitus, infections and monoclonal gammopathy. Its association with ovarian malignancy has not been reported. We report a case of a 56-year-old female with rapidly progressing skin thickening of limbs, face and trunk for 1 year and abdominal distension for 3 months. Patient had thickened skin, mask-like facies and ascites on examination. Atypical cells were seen in ascitic fluid. Contrast-enhanced computerised axial tomography scan of abdomen was suggestive of ovarian malignancy. Markers for autoimmune disorders were negative. CA 125 was elevated. Other causes of sclerodermiform-like syndrome were ruled out. Histopathology of skin biopsy was definitive of scleroedema. Diagnosis of scleroedema associated with ovarian malignancy was made based on temporal association, exclusion of other causes and histopathological findings. To our knowledge this is the first reported case of scleroedema associated with ovarian tumour.