Scleredema Research Articles

Scleredema Diabeticorum in a Patient with the Normal Range of the Hemoglobin A1c Level and Impaired Glucose Tolerance

Scleredema diabeticorum is an uncommon skin disorder showing a diffuse, symmetrical, and non-pitting persistent in-duration developing initially on the nape. It usually occurs in patients with poorly controlled or insulin-resistant diabetes mellitus. We present a 57-year-old woman with scleredema diabeticorum with hemoglobin A1c 6.1% (normal, 4.7% - 6.2%) and impaired glucose tolerance. Alcian blue staining at pH 7.0 illustrated deposits of acid mucopolysaccharides between the collagen bundles in the reticular dermis, but the staining at pH 1.5 did not show the deposits. This case indicates that scleredema diabeticorum may be an early indicator of undiagnosed diabetes mellitus in some cases, and that impaired glucose tolerance test should be performed for individuals with scleredema even though the hemoglobin A1c level is within the normal range.

Scleredema adultorum of Buschke: With unusual manifestations in a young female

The proposed mechanisms of pathogenesis are analogous to the ossifying enthesopathies of seronegative arthritis. The inflammation is supposedly transmitted from the involved joint cartilage to the insertions of ligaments or tendons. The close anatomical relationships between nail and terminal phalanx may result in spread of inflammation of the psoriatic process from the subungual dermis to the terminal phalanx through the fibrous septae, which usually directly join them and are then only deeply inserted into bone.Treatment of POPP is not easy. Therapy is usually similar to that of psoriatic arthritis. Response to both non-steroidal anti-inflammatory drugs and sulfasalazine is not consistent. Methotrexate 4 mg weekly has been reportedly found to be effective in one patient.

ESCLEREDEMA ADULTORUM DE BUSCHKE – CASO CLÍNICO

O Escleredema adultorum de Buschke (EAB) é uma doença rara do tecido conjuntivo caracterizada pelo endurecimento não depressível da pele. Histologicamente há espessamento da derme com fibras de colagénio espes- sadas separadas por mucopolissacáridos. Apresentamos um doente de 51 anos de idade, diabético insulinotratado com mau controlo metabólico, que referia um quadro com 5 anos de evolução de agravamento progressivo, caracte- rizado por infiltração cutânea difusa de limites mal definidos na região cervical posterior, metade superior do tronco e raiz dos membros, marcada diminuição da mobilidade cutânea e limitação funcional da charneira cervical e ombros. A hipótese de EAB foi confirmada pela histologia. Os restantes exames complementares de diagnóstico não revelaram alterações significativas, permitindo estabelecer o diagnóstico do subtipo III – diabeticorum. Iniciou fototerapia com radiação UVA1 sem qualquer benefício, e posteriormente uma terapêutica experimental com PUVA1, com melhoria clínica significativa, objectivável pela maior facilidade na realização de tarefas básicas como o vestir.PALAVRAS-CHAVE – Escleredema Adultorum; Escleredema Diabeticorum; Diabetes Mellitus; Fototerapia; PUVA.

Treatment of acquired reactive perforating collagenosis with allopurinol incidentally improves scleredema diabeticorum

To the Editor: Acquired reactive perforating collagenosis is a condition characterized by altered collagen eliminated through the epidermis. It is usually associated with diabetes mellitus and chronic renal disease. Oral allopurinol has been described to be effective in treating acquired reactive perforating collagenosis. 1 Querings K. Balda B.R. Bachter D. Treatment of acquired reactive perforating collagenosis with allopurinol. Br J Dermatol. 2001; 145: 174-176 Crossref PubMed Scopus (33) Google Scholar Scleredema diabeticorum (SD) is also a dermatological complication of diabetes mellitus. It is characterized by diffuse swelling and nonpitting induration of the skin, especially on the upper trunk and neck. The histologic findings include a thickened broad dermis with mucin between thickened collagen bundles. SD is difficult to treat. Many therapies, including ultraviolet (UV) A1 phototherapy, bath psoralen UVA, narrowband UVB, extracorporeal photopheresis, radiation, electron beam, cyclosporine, methotrexate and high-dose penicillin, have been reported to be effective in separate cases. We report a case of acquired reactive perforating collagenosis treated with allopurinol treatment. Surprisingly, the concomitant SD improved simultaneously. This case implied the therapeutic effect of allopurinol in SD.

Scleroedema diabeticorum

Scleredema adultorum is characterized by induration of the skin on the neck, shoulders and upper back caused by increased accumulation of collagen and aminoglycans in the dermis. The induration may progress and lead to pronounced restriction of mobility. Scleredema diabeticorum is one type of scleredema adultorum associated with diabetes mellitus. Multiple therapies have been tried, but most of them have not proven to be consistently effective. We report two cases of scleredema diabeticorum treated successfully with UVA-1- as well as physiotherapy and topical corticosteroids; this approach led to improvement in skin changes and mobility.

Scleredema of Buschke following Mycoplasma pneumoniae respiratory infection

Scleredema of Buschke following <i>Mycoplasma pneumoniae</i> respiratory infection

Treatment of Scleredema Diabeticorum with Tamoxifen: Figure 1.

To the Editor: Scleredema, originally described by Buschke in 19021, is a rare sclerodermatosis of unknown etiology, characterized by nonpitting induration of the skin. In general, scleredema first affects the face and neck, and then may spread symmetrically to the shoulders, trunk, arms, and legs. Cardiac and other organ involvement is rare but restrictive lung disease can be a manifestation. Three clinical groups of scleredema have been described by Graff2. In the first group, the disease starts abruptly after an acute upper respiratory tract infection, often with streptococcal pyogenes and having a tendency to resolve after a period of months to years. The second group begins insidiously without a preceding respiratory tract infection, is of longer duration, and persisting over a period of several years. The third group, known as scleredema diabeticorum (SD), is a chronic form of scleredema associated with severe, often complicated, diabetes mellitus (DM). SD is characterized by an insidious onset of skin thickening, occurring diffusely over the posterior neck and upper back and occasionally extending to the deltoid and lumbar regions (Figure 1)3. It has been reported to occur in 2.5% to 14% of all patients with diabetes. Although numerous treatments have been tried, none have been reported to be effective. We describe 2 cases of SD that showed marked clinical regression following treatment with tamoxifen (tamoxifen citrate). Figure 1. Skin thickening and hyperpigmentation on the back and extending forward to involve the shoulders and chest in a patient with scleredema diabeticorum not treated with tamoxifen. A 61-year-old Chinese woman presented to our Scleroderma Clinic in April 2002 with a 2-year history of progressive skin thickening involving her back, chest, and shoulders but sparing her extremities. There was tightness of her chest with restricted movement of her shoulders, especially with reaching … Address correspondence to Dr. S. AlSaeedi, Mount Sinai Hospital, The Rebecca MacDonald Centre for Arthritis and Autoimmune Disease, 60 Murray Street, Box 9, Rm 2-004, Toronto, Ontario M5T 3L9, Canada. E-mail: sa2005saa{at}hotmail.com

Escleredema de Buschke associado ao diabetes melito tipo 2: relato de caso e revisão da literatura

Scleredema of Buschke (SB) is a rare disorder of connective tissue, characterized by hardening of the skin. Of unknown etiology, it may be associated with viral or bacterial infections, hematological alterations and diabetes mellitus (DM). In half of the reported cases of SB, there is association with DM. The most common characteristics among these patients are: being male adults, having long-term DM, bad metabolic control and presence of specific complications of DM. The histopathological examination shows dermal thickening, with widened collagen fibers separated by non-colored spots, which correspond to mucopolysaccharides deposits. The clinical consequences are: decrease in motility of the shoulders and an impairment of respiratory function. Several treatments are suggested in the literature, but with inconstant results. A case of SB is reported in a type 2 diabetes patient.

Scleredema diabeticorum successfully treated with ultraviolet A1 phototherapy

Scleredema diabeticorum is an uncommon condition. It is one of the cutaneous manifestations in diabetes mellitus that mainly occurs in obese middle-aged men with insulin-resistant diabetes. This condition is generally recalcitrant to therapy. Various treatments have been tried with inconsistent results. Here, we describe two cases of scleredema diabeticorum with substantial clinical improvement from a course of medium dose (60 J/cm²) ultraviolet A1 radiation therapy.

Scleredema of Buschke in a Child with Cardiac Involvement

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.

Indolente Schwellung des Halses

Scleredema adultorum Buschke is a rare disease of unknown etiology characterized by an immovable edematous swelling and hardening of the skin which typically begins in the neck and spreads to the face, head, shoulders and thorax. No standardized therapy for scleredema adultorum Buschke has yet been established. As in the case of our patient, a systemic cortisone therapy is normally administered combined with high-dose penicillin and physiotherapy.

Scleredema diabeticorum: an under recognised complication?

AbstractWe report the case of a 41‐year‐old man with type 1 diabetes who presented with scleredema diabeticorum.Scleredema diabeticorum is an uncommon skin condition. It is a poorly known (recognised) complication of diabetes.The aetiopathogenesis of the clinical condition is not well understood. No single form of treatment has been proven to be consistently beneficial. In most cases it is a self‐limiting, benign disease. Optimisation of metabolic control and physiotherapy are recommended for all cases. Copyright © 2009 John Wiley &amp; Sons.

Analysis of neonatal death in NICU during the latest 5 years

Objective To analyze the cause and change of neonatal death during the latest 5 years,to discuss the ways to decrease the mortality.Methods A retrospective analysis of 69 cases in NICU were performed from Jan 2004 to Dec 2008.Results(1)The sex ratio of male to female was 1.7:1.The preterm infants accounted for 34.8%,preterm infants with gestational ages of less than 32 weeks accounted for 70.8% of the total premature deaths.(2)59.4% of the deaths in newborn infants occurred in the first 7days of life.69.6% occurred within 3 days after admission.(3)The three leading causes of premature infant death were RDS,sepsis and scleredema neonatorum,accounted for 75% of premature death.The three leading canses of full term death were sepsis,neonate severe asphyxia and pneumonia,accounted for 71.1% of all full term death.(4)The newborn who died of RDS were gradually decreasing.Those who died of sepsis and severe asphyxia weren't changing.Condusion Heath care during the perinatal period should be strengthened to decrease the premature birth and asphyxia.The neonatal new resuscitation technique should be widely adovocated and neonatal sepsis should be treated positively. Key words: Infant; newborn; Neonate; premature; Death

Scleredema adultorum of Buschke associated with a carcinoid tumor

Scleredema adultorum of Buschke associated with a carcinoid tumor

Scleredema Diabeticorum Case Series: Successful Treatment With UV-A1

Scleredema Diabeticorum Case Series: Successful Treatment With UV-A1

Scleredema diabeticorum with unusual presentation and fatal outcome

We present a case of Scleredema Diabeticorum (SD) in a patient with diabetic neuropathy and restrictive respiratory disease with unusual skin lesion distribution. The onset of dermatologic symptoms heralded a progressive respiratory disease with constrictive component. Painful diabetic neuropathy was noteworthy and difficult to relieve. Predominantly, distribution of the skin lesions on the thighs makes the case exceptional. T2-weighted MRI showed abnormal hyperintensities along the muscles of the thighs in correspondence with the skin lesions. Gait and respiratory symptoms progressively worsened. After a transient remitting period, he developed sudden shortening of breath, arrested and expired at home.The atypical distribution of the skin lesions with further involvement of underlying muscles plus concomitant polyneuropathy and respiratory constrictive disease with sudden death is quite unusual and aggressive presentation of SD.

Amiloidose manifestando-se com pseudo-hipertrofia muscular

Amiloidose tipo AL e uma doenca rara causada pela deposicao extracelular de fragmentos de cadeias leves monoclonais em orgaos e tecidos. Pode apresentar-se com uma ampla variedade de sinais e sintomas, e o acometimento cutâneo-muscular, simulando pseudo-hipertrofia muscular, e um achado muito raro. Sao descritos dois casos que apresentaram tal manifestacao. CASO 1 - Mulher, 61 anos, ha quatro meses com historia de mialgia e aumento da massa muscular nas cinturas pelvica, escapular e regiao cervical. Nao havia alteracoes significativas ao exame fisico, exceto aparente hipertrofia muscular difusa e discreta macroglossia. CASO 2 - Homem, 51 anos, ha dois anos com cansaco e espessamento cutâneo progressivo do dorso, pescoco e bracos. Em outros servicos levantou suspeitas diagnosticas de esclerodermia ou de escleredema de Buschke; desde fevereiro de 2007 passou a ser acompanhado nesse servico e referia, havia cerca de um ano, disfagia para solidos, disartria e dificuldade para movimentar a lingua. Chamava atencao em seu exame o porte fisico atletico com musculatura toracica proeminente, porem referia nao fazer exercicios fisicos. Em ambos os casos, a biopsia cutânea foi realizada com identificacao do deposito amiloide por meio da coloracao de vermelho congo.

Poster 113: Functional Capacity and Buschke’s Scleredema Adultorum Associated With Diabetes Mellitus: A Case Report

Setting: Rehabilitation unit in a tertiary public hospital. Patient: A 52-year-old woman presented with Buschke’s scleredema adultorum associated with diabetes mellitus. Case Description: Patient with a 2-year history of scleredema adultorum type 3 and pulmonary hypertension as a systemic manifestation of the disease. She was referred to our rehabilitation unit because her functional capacity was getting worse, pain, a bilateral temporo-mandibular joint dysfunction, and important shoulder, elbow, and cervical spine range of motion (ROM) limitations that affected her activities of daily life and quality of life (QOL).

The Family Survy to a Case of Patient with Children Scleredema and the Literature Reviewed

目的 探讨小儿硬肿病及家族聚集现象.方法 报告1例小儿硬肿病的临床特点及其家族聚集现象,并通过复习文献,分析该病的临床特点、预后及治疗方法.结果 8岁患儿,因尿少、尿频1个月,皮肤发硬肿胀5天入院.经皮肤病理活检等检查后确诊为硬肿病,予改善微循环等治疗后8天症状消失,进一步家系调查发现该家系四代30人中共11人类似发病.结论 硬肿病的临床特点是对称性、非凹陷性皮肤硬肿,无痛感或触痛,最初发生在面部皮肤及颈后两侧,逐渐向肩、臂、上胸发展,最后波及腹、臀、腿,也可局限于大腿,阿辛蓝染色呈蓝色,可有家族聚集现象。

Scleredema adultorum treated with narrow‐band ultraviolet B phototherapy

The Journal of DermatologyVolume 34, Issue 4 p. 270-272 Scleredema adultorum treated with narrow-band ultraviolet B phototherapy Ting XIAO, Ting XIAO Department of Dermatology, No. 1 Hospital of China Medical University, Shenyang, ChinaSearch for more papers by this authorZhen-Hai YANG, Zhen-Hai YANG Department of Dermatology, No. 1 Hospital of China Medical University, Shenyang, ChinaSearch for more papers by this authorChun-Di HE, Chun-Di HE Department of Dermatology, No. 1 Hospital of China Medical University, Shenyang, ChinaSearch for more papers by this authorHong-Duo CHEN, Hong-Duo CHEN Department of Dermatology, No. 1 Hospital of China Medical University, Shenyang, ChinaSearch for more papers by this author Ting XIAO, Ting XIAO Department of Dermatology, No. 1 Hospital of China Medical University, Shenyang, ChinaSearch for more papers by this authorZhen-Hai YANG, Zhen-Hai YANG Department of Dermatology, No. 1 Hospital of China Medical University, Shenyang, ChinaSearch for more papers by this authorChun-Di HE, Chun-Di HE Department of Dermatology, No. 1 Hospital of China Medical University, Shenyang, ChinaSearch for more papers by this authorHong-Duo CHEN, Hong-Duo CHEN Department of Dermatology, No. 1 Hospital of China Medical University, Shenyang, ChinaSearch for more papers by this author First published: 09 March 2007 https://doi.org/10.1111/j.1346-8138.2007.00268.xCitations: 8 Professor Hong-Duo Chen, M.D., Department of Dermatology, No. 1 Hospital of China Medical University, 155 North Nanjing Street, Shenyang 110001, China. Email: [email protected] Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat Citing Literature Volume34, Issue4April 2007Pages 270-272 RelatedInformation