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1.
Reumatismo ; 75(1)2023 May 08.
Artículo en Inglés | MEDLINE | ID: mdl-37154255

RESUMEN

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/patología
3.
Dermatol Online J ; 27(3)2021 Mar 15.
Artículo en Inglés | MEDLINE | ID: mdl-33865279

RESUMEN

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 , Ultrasonografía
4.
Indian J Dermatol Venereol Leprol ; 86(3): 272-277, 2020.
Artículo en Inglés | MEDLINE | ID: mdl-30289118

RESUMEN

Scleredema adultorum of Buschke is a rare fibromucinous, scleroderma-like connective tissue disease most commonly found in a post-infectious setting or linked to hematological disorders or diabetes. Lichen sclerosus et atrophicus is an autoimmune condition only in 2.5% of cases localized exclusively at an extragenital site, occurring in up to 34% of patients in association with other autoimmune diseases such as vitiligo, thyroid disorders, alopecia areata, lichen planus, morphea, pernicious anemia and systemic lupus erythematosus. In particular, a stronger link with an autoimmune background in lichen sclerosus et atrophicus has been observed in women who showed higher prevalence for autoimmune conditions and circulating autoantibodies. Literature reveals a genetic susceptibility linked to specific HLA types. We report three patients who developed lichen sclerosus et atrophicus superimposed on skin involved by scleredema adultorum of Buschke. Although the association of lichen sclerosus et atrophicus with scleredema adultorum of Buschke could be coincidental, both diseases could be considered part of the spectrum of sclerodermoid disorders with common underlying pathogenetic mechanisms; which could explain the sequential or simultaneous occurrence of both lesions in our patients.


Asunto(s)
Liquen Escleroso y Atrófico/complicaciones , Liquen Escleroso y Atrófico/diagnóstico , Escleredema del Adulto/complicaciones , Escleredema del Adulto/diagnóstico , Anciano , Femenino , Humanos , Persona de Mediana Edad
7.
Clin Dermatol ; 36(1): 94-100, 2018.
Artículo en Inglés | MEDLINE | ID: mdl-29241760

RESUMEN

The link between the metabolic syndrome (MetS) and skin diseases is increasingly important, with new associations being discovered. The association between MetS and psoriasis or MetS and hidradenitis suppurativa is well known, although the relationship between MetS and various autoimmune or inflammatory diseases has only recently attracted interest. Some inflammatory skin diseases, such as vitiligo, scleredema, recurrent aphthous stomatitis, Behçet disease, rosacea, necrobiosis lipoidica, granuloma annulare, skin tags, knuckle pads, and eruptive xanthomas, have possible associations with MetS. In this review, we examine the state of knowledge involving the relationship between MetS and these dermatologic diseases.


Asunto(s)
Síndrome Metabólico/complicaciones , Enfermedades de la Piel/complicaciones , Síndrome de Behçet/complicaciones , Granuloma Anular/complicaciones , Humanos , Necrobiosis Lipoidea/complicaciones , Rosácea/complicaciones , Escleredema del Adulto/complicaciones , Estomatitis Aftosa/complicaciones , Vitíligo/complicaciones , Xantomatosis/complicaciones
8.
Disabil Rehabil ; 40(23): 2833-2835, 2018 11.
Artículo en Inglés | MEDLINE | ID: mdl-28738698

RESUMEN

PURPOSE: Scleredema Adultorum Buschke is a disorder manifesting indurations of the skin mostly followed by musculoskeletal impairment. Data regarding this fact are seldom found and documentation of functional outcome of physical therapies and modalities related to Scleredema Adultorum Buschke is fragmentary. The aim of this case report is to demonstrate and to document an effective concept of rehabilitation in a patient suffering from Scleredema Adultorum Buschke. METHODS: A treatment plan was developed containing therapeutic ultrasound, manual lymphatic drainage, and physiotherapy. Assessments were performed at baseline and after therapy. RESULTS: Treatment by physical therapies of presented patient resulted in an improved functionality. Five out of eight Short Form-36 questionaire sections increased in terms of enhanced general health and level of activity. CONCLUSIONS: Musculoskeletal impairment in a patient suffering from Scleredema Adultorum Buschke can be reduced by a multimodal concept of rehabilitation. Implications for Rehabilitation Rehabilitation professional should suspect scleredema in patients with diffuse skin thickening where hands and feet are spared Essential reactivating physical activity should be supported by skin softening physical modalities irrespective of etiology or primary therapy. There is a need for functional outcome measures and documentation in the rehabilitation of Scleredema Adultorum Buschke.


Asunto(s)
Cifosis/rehabilitación , Escleredema del Adulto/rehabilitación , Vértebras Cervicales/fisiopatología , Humanos , Cifosis/fisiopatología , Masculino , Persona de Mediana Edad , Modalidades de Fisioterapia , Rango del Movimiento Articular/fisiología , Escleredema del Adulto/complicaciones , Articulación del Hombro/fisiopatología , Terapia por Ultrasonido
11.
An Bras Dermatol ; 90(3 Suppl 1): 81-3, 2015.
Artículo en Inglés | MEDLINE | ID: mdl-26312681

RESUMEN

Scleredema adultorum of Buschke is a rare disorder characterized by diffuse swelling and non-pitting induration of the skin usually involving the face, neck, arms and upper trunk. It has been associated with previous infectious diseases, diabetes, paraproteinemia and, more rarely, malignant neoplasms or autoimmune disorders. We report the case of a 30-year-old man who presented with a 2-year history of scleredema. Further investigation led to the diagnosis of primary Sjögren's syndrome. The association between scleredema and autoimmune disorders has been rarely seen. To our knowledge, there are no other reports describing the association between primary Sjögren's syndrome and scleredema adultorum of Buschke.


Asunto(s)
Escleredema del Adulto/complicaciones , Escleredema del Adulto/patología , Síndrome de Sjögren/complicaciones , Síndrome de Sjögren/patología , Adulto , Biopsia , Humanos , Masculino , Piel/patología
12.
An. bras. dermatol ; 90(3,supl.1): 81-83, May-June 2015. ilus
Artículo en Inglés | LILACS | ID: lil-755765

RESUMEN

Abstract

Scleredema adultorum of Buschke is a rare disorder characterized by diffuse swelling and non-pitting induration of the skin usually involving the face, neck, arms and upper trunk. It has been associated with previous infectious diseases, diabetes, paraproteinemia and, more rarely, malignant neoplasms or autoimmune disorders. We report the case of a 30-year-old man who presented with a 2-year history of scleredema. Further investigation led to the diagnosis of primary Sjögren’s syndrome. The association between scleredema and autoimmune disorders has been rarely seen. To our knowledge, there are no other reports describing the association between primary Sjögren’s syndrome and scleredema adultorum of Buschke.

.


Asunto(s)
Adulto , Humanos , Masculino , Escleredema del Adulto/complicaciones , Escleredema del Adulto/patología , Síndrome de Sjögren/complicaciones , Síndrome de Sjögren/patología , Biopsia , Piel/patología
17.
Acta Dermatovenerol Croat ; 19(3): 191-4, 2011.
Artículo en Inglés | MEDLINE | ID: mdl-21933647

RESUMEN

Scleredema adultorum (SA) is a rare sclerotic disorder characterized by non-pitting induration of the neck with acral progression, sparing hands and feet. We report on a 57-year-old male with severe SA associated with paraproteinemia, treated with methotrexate. Such widespread skin thickening followed by severe movement restriction and inability to function on daily basis, as in our patient, has never been described. Severe osteoarthritis and finding of HLA-B39 allele in association with SA has not been previously described either. To the best of our knowledge, up to 40 patients with SA associated with paraproteinemia has been reported so far, and currently, there is no established effective treatment protocol. In our patient, low-dose methotrexate resulted in stiffness reduction, increased motility of the trunk and extremities, and ability to function on daily basis. We believe that any information about treatment outcome in SA patients should be disseminated in order to establish consensual treatment protocol for this rare disease.


Asunto(s)
Antígeno HLA-B39/análisis , Osteoartritis/complicaciones , Paraproteinemias/complicaciones , Escleredema del Adulto/complicaciones , Humanos , Masculino , Metotrexato/uso terapéutico , Persona de Mediana Edad , Osteoartritis/inmunología , Paraproteinemias/tratamiento farmacológico , Paraproteinemias/inmunología , Escleredema del Adulto/tratamiento farmacológico , Escleredema del Adulto/inmunología
19.
Pediatr Dermatol ; 27(3): 315-7, 2010.
Artículo en Inglés | MEDLINE | ID: mdl-20609163

RESUMEN

A few descriptions of scleredema of Bushke associated with cardiac involvement in children have been reported. We report a case of an 11-year-old boy with diffuse nonpitting edema of the trunk and face associated with cardiac involvement.


Asunto(s)
Insuficiencia Cardíaca/diagnóstico , Escleredema del Adulto/diagnóstico , Captopril/uso terapéutico , Niño , Colágeno/análisis , Digoxina/uso terapéutico , Furosemida/uso terapéutico , Insuficiencia Cardíaca/tratamiento farmacológico , Insuficiencia Cardíaca/etiología , Humanos , Imagen por Resonancia Magnética , Masculino , Mucinas/análisis , Escleredema del Adulto/complicaciones , Piel/química , Privación de Agua
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