Scleroderma-like Disease Research Articles

Photo gallery. Rare dermatoses. Part I

Orphan dermatoses are caused by both genetic causes and a number of autoimmune, inflammatory and infectious processes in the body. Congenital giant nevus is associated with serious complications: malignant melanoma, CNS lesions (neurocutaneous melanosis). Multiple epidermoid cysts are often a component of Gardner's syndrome. Angiolymphoid hyperplasia with eosinophilia, a vascular tumor formation with proliferation of histiocytoid endothelial cells, with severe lymphocytic and eosinophilic infiltration, recurs in 1/3 of cases. Morbigan's disease is persistent erythema and swelling of the middle third and upper part of the face. Skin mastocytosis in children is prone to spontaneous resolution during puberty. Squamous cell skin cancer accounts for 20% of all skin cancers and has a high risk of recurrence, metastasis, and death. Buschke's scleroderma is a scleroderma-like disease that causes progressive fibrous-mucinous thickening of the skin affecting the neck, shoulders, proximal upper extremities, and face. Fulminant (lightning) acne is a sharply exacerbated form of acne vulgaris, characterized by an acute onset and the appearance of confluent abscesses. Cases of acne fulminans have been observed during treatment with isotretinoin. Norwegian (crustose) scabies is associated with a compromised immune response, which allows the mites to multiply and can number in the millions. Pretibial myxedema (thyroid dermopathy) is a rare extrathyroid manifestation of Graves' disease along with thyrotoxicosis, thyroid hypertrophy, ophthalmopathy, and acropachy. Porokeratosis Mibelli predisposing factors of development are immunosuppressive conditions, malignancy is possible. Osteomas are formations from bone tissue in the dermis or subcutaneous fat as a result of metaplasia of fibroblasts into osteoblasts, or differentiation of primitive mesenchymal cells into osteoblasts and their migration to an ectopic site. Sarcoidosis of the skin is characterized by a variety of clinical forms, rarely observed before 40 years. Xanthomas with histiocytosis X ― a violation of lipid metabolism. The rural type of leishmaniasis (acute necrotizing) has a short incubation period, a significant spread of rashes and healing with the formation of scar tissue. The giant condyloma of BuschkeLevenshtein is characterized by progressive growth, large size and recurrent course, it can transform into squamous cell carcinoma. Degos disease ― malignant atrophic papulosis ― a rare disease caused by blockage of the arteries and veins.

Scleroderma-like Disease Due to Diffuse Monosodium Urate Crystal Deposition.

Scleroderma-like disease is defined as fibrosis of the skin caused by certain chemicals, drugs, environmental factors, or metabolic abnormalities, which manifests as scleroderma-like changes.1,2 However, large deposits of urate crystals in the skin and subcutaneous fascial tissue causing scleroderma-like disease have not been reported in the literature.

Examination of the Validity of Skin Ultrasound to Quantitate Skin Involvement for Multicenter Clinical Trials in Patients with Systemic Sclerosis (SSc): A Systematic Literature Review (SLR).

ObjectiveThe study objective was to conduct a systematic literature review (SLR) of ultrasound of the skin in patients with systemic sclerosis (SSc) to establish the degree to which ultrasound of the skin has been validated, using the Outcome Measures in Rheumatology (OMERACT) Filter.MethodsWe conducted an SLR of publications between 1950 and 2018, using PubMed and Cochrane library, to examine ultrasound validity to quantitate SSc skin involvement. Inclusion criteria were as follows: (1) in English; (2) used the 1980 or 2013 classification criteria for SSc criteria; (3) either a randomized controlled trial, an observational study, or a case study including more than 15 patients; (4) subjects 18 years of age or older; (5) for mixed patient populations, SSc results were separable; and (6) the ultrasound machine was clearly described. Exclusion criteria were as follows: (1) not in English; (2) data did not record at least one of the validation criteria; (3) subjects aged less than 18 years; (4) subjects had disease other than SSc (eg, localized scleroderma or scleroderma‐like disease); (5) a letter to the editor or an editorial; and (6) involved a modified Rodnan skin score of less than 2. Descriptive statistics were generated for each criterion.ResultsFrom an initial 292 citations, 14 articles (1,055 patients) met inclusion and exclusion criteria. The status of validation for ultrasound was evaluated by using the OMERACT criteria of truth, discrimination, and feasibility (in turn divided into nine different criteria). Face, criterion, content, construct, reliability, and responsiveness criteria were met, and the feasibility criterion was partially met, whereas discrimination and reproducibility criteria were not met.ConclusionBased on an SLR through December 31, 2018, ultrasound of the skin met some but not all validation criteria for use in clinical trials.

The place of <sup>18</sup>F FDG PET/CT in the management of patients with eosinophilic fasciitis: a case report

Eosinophilic fasciitis is a rare connective tissue disease with a clinical presentation of scleroderma-like disease. We report a case of a 36-year-old female patient with a 6-month history of progressive stiffness involving her forearms and legs with joint pain. Laboratory examinations showed hypereosinophilia and elevated C-reactive protein. 18F FDG PET/CT showed diffuse and symmetrical increased uptake in the fasciae of the upper and lower limbs, sparing both muscles and fat tissues. Guided biopsy and histologic examination confirmed the diagnosis of eosinophilic fasciitis. 18F FDG PET/CT is of great help in the diagnosis of eosinophilic fasciitis, as it can guide the biopsy where FDG uptake is strongest and also help rule out possible associated neoplasms.

Pulmonary involvement in juvenile eosinophilic fasciitis: A case report

Introduction: Eosinophilic fasciitis (EF) is a rare scleroderma-like disease, characterized by acute onset of symmetrical swelling, induration and thickness of the skin. Laboratory findings include peripheral eosinophilia, elevated inflammatory markers and increased gammaglobulemia. Although, the diagnosis is mainly clinically, a full thickness skin/fascia/muscle biopsy remains the gold standard for the definite diagnosis. Unlike systemic sclerosis, visceral involvement, such as pulmonary affection, is rare in EF. Although, few cases of systemic involvement in adult EF have been presented, we report a rare case of juvenile EF associated with pulmonary involvement. Case report: A 12-years old boy presented with symmetrical skin thickening of bilateral upper extremity, back, trunk, neck, face, and scalp of 2-weeks duration. The laboratory tests revealed marked peripheral eosinophilia 61.9% (normal 1–3%) and elevation of the acute phase reactants (erythrocyte sedimentation rate 40 mm/1st hour and C-reactive protein 15 mg/dL). Lung imaging study showed bilateral extensive pulmonary nodules. A full thickness skin/fascia/muscle biopsy revealed an inflammatory infiltration, fibrosis in the fascial and muscle tissues. These findings were concordant with EF. An initial treatment of intravenous (IV) methylprednisolone 30 mg/kg/day for 3 successive days was started followed by oral steroid (2 mg/kg/day) plus methotrexate (20 mg/week). Follow up revealed complete improvement in the skin thickening, pulmonary affection and systemic inflammation. Conclusion: To the best of our knowledge this is the first reported co-existence of pediatric eosinophilic fasciitis with pulmonary affection. Systemic involvement should be screened in EF cases, as it may have consequences in the management and outcome.

Next Generation Sequencing identifies mutations in GNPTG gene as a cause of familial form of scleroderma-like disease

BackgroundScleroderma is a multisystem disease, characterized by fibrosis of skin and internal organs, immune dysregulation, and vasculopathy. The etiology of the disease remains unknown, but it is likely multifactorial. However, the genetic basis for this condition is defined by multiple genes that have only modest effect on disease susceptibility.MethodsThree Moroccan siblings, born from non-consanguineous Moroccan healthy parents were referred for genetic evaluation of familial scleroderma. Whole Exome Sequencing was performed in the proband and his parents, in addition to Sanger sequencing that was carried out to confirm the results obtained.ResultsMutation analysis showed two compound heterozygous mutations c.196C>T in exon 4 and c.635_636delTT in exon 9 of GNPTG gene. Sanger sequencing confirmed these mutations in the affected patient and demonstrated that their parents are heterozygous carriers.ConclusionOur findings expand the mutation spectrum of the GNPTG gene and extend the knowledge of the phenotype–genotype correlation of Mucolipidosis Type III gamma. This report also highlights the diagnostic utility of Next Generation Sequencing particularly when the clinical presentation did not point to specific genes.

Systemic sclerosis and silicone breast implant: a case report and review of the literature.

Environmentally induced systemic sclerosis is a well-recognized condition, which is correlated with exposure to various chemical compounds or drugs. However, development of scleroderma-like disease after exposure to silicone has always been a controversial issue and, over time, it has triggered spirited debate whether there is a certain association or not. Herein, we report the case of a 35-year-old female who developed Raynaud's phenomenon and, finally, systemic sclerosis shortly after silicone breast implantation surgery.

Nephrogenic Systemic Fibrosis-Like Effects of Magnetic Resonance Imaging Contrast Agents in Rats with Adenine-Induced Renal Failure

Nephrogenic systemic fibrosis (NSF) is a scleroderma-like disease associated with prior administration of certain gadolinium chelates (GCs). NSF occurs in patients with severe renal failure. The purpose of this study was to set up a rat model of GC-associated NSF to elucidate the mechanism of this devastating disease. Firstly, after characterization of the model, male Wistar rats received a 0.75% adenine-enriched diet for 8, 14, or 16 days to obtain various degrees of renal failure. Rats received five consecutive daily iv injections of saline or gadodiamide (2.5 mmol/kg/day). Secondly, the safety profile and in vivo propensity to dissociate of all categories of marketed GCs (gadoterate, gadobutrol, gadobenate, gadopentetate, and gadodiamide) were compared in rats receiving adenine-enriched diet for 16 days. Serial skin biopsies were performed for blinded histopathological study. Total Gd concentration in tissues was measured by Inductively Coupled Plasma Mass Spectrometry. Relaxometry was used to evaluate the presence of dissociated Gd in skin and bone. Gadodiamide-induced high mortality and skin lesions (dermal fibrosis, calcification, and inflammation) were related to adenine diet duration. No skin lesions were observed with other molecules. Unlike macrocyclic GCs, gadodiamide, gadopentetate, and gadobenate gradually increased the r(1) relaxivity value, consistent with in vivo dissociation and release of soluble Gd (or, in the case of gadobenate, the consequence of protein binding). Gadodiamide-induced cutaneous and systemic toxicity depended on baseline renal function. We demonstrate in vivo dissociation of linear GCs, gadodiamide, and gadopentetate, whereas macrocyclic agents remained stable over the study period.

Role of UVA1 phototherapy in treatment of scleroderma and scleroderma-like disorders”

ENWEndNote BIBJabRef, Mendeley RISPapers, Reference Manager, RefWorks, Zotero AMA Osmola-Mańkowska A, Dańczak-Pazdrowska A, Olek-Hrab K, Silny W. Review papersRole of UVA1 phototherapy in treatment of scleroderma and scleroderma-like disorders”. Central European Journal of Immunology. 2012;37(4):391-398. doi:10.5114/ceji.2012.32731. APA Osmola-Mańkowska, A., Dańczak-Pazdrowska, A., Olek-Hrab, K., & Silny, W. (2012). Review papersRole of UVA1 phototherapy in treatment of scleroderma and scleroderma-like disorders”. Central European Journal of Immunology, 37(4), 391-398. https://doi.org/10.5114/ceji.2012.32731 Chicago Osmola-Mańkowska, Agnieszka, Aleksandra Dańczak-Pazdrowska, Karolina Olek-Hrab, and Wojciech Silny. 2012. "Review papersRole of UVA1 phototherapy in treatment of scleroderma and scleroderma-like disorders”". Central European Journal of Immunology 37 (4): 391-398. doi:10.5114/ceji.2012.32731. Harvard Osmola-Mańkowska, A., Dańczak-Pazdrowska, A., Olek-Hrab, K., and Silny, W. (2012). Review papersRole of UVA1 phototherapy in treatment of scleroderma and scleroderma-like disorders”. Central European Journal of Immunology, 37(4), pp.391-398. https://doi.org/10.5114/ceji.2012.32731 MLA Osmola-Mańkowska, Agnieszka et al. "Review papersRole of UVA1 phototherapy in treatment of scleroderma and scleroderma-like disorders”." Central European Journal of Immunology, vol. 37, no. 4, 2012, pp. 391-398. doi:10.5114/ceji.2012.32731. Vancouver Osmola-Mańkowska A, Dańczak-Pazdrowska A, Olek-Hrab K, Silny W. Review papersRole of UVA1 phototherapy in treatment of scleroderma and scleroderma-like disorders”. Central European Journal of Immunology. 2012;37(4):391-398. doi:10.5114/ceji.2012.32731.

Cells from a vanished twin as a source of microchimerism 40 years later in a male with a scleroderma-like condition.

We report the case of a 40-year-old man diagnosed with a scleroderma-like disease. Clinical similarities with graft versus host disease prompted initial testing for chimerism employing fluorescence in situ hybridization (FISH). Female cells were observed within peripheral blood mononuclear cells from the patient.Because maternal cells have been detected in healthy immunologically competent adults and patients with autoimmune conditions, we hypothesized that these cells were of maternal origin. Contrary to our expectations, HLA-specific quantitative PCR (QPCR) ruled out maternal microchimerism. However, HLA-specific QPCR testing was positive for the paternal HLA haplotype that the patient did not inherit. We reasoned that the most likely origin of chimerism with non-inherited paternal HLA alleles was from an unrecognized "vanished" twin. The patient had never received a blood transfusion.This report suggests that cells from a vanished twin are a possible source of chimerism. The frequency of chimerism from this source is not yet known and whether the scleroderma-like disease observed in the patient is anecdotal or implies a potential association with autoimmune disease remains to be elucidated.

Novel Anti-Fibrotic Therapies for Experimental Chronic Graft-Versus-Host Disease.

Abstract 4490 BackgroundChronic graft-versus host disease (cGvHD) can result in a scleroderma-like disease with dermal, pulmonary and gastrointestinal fibrosis. The histopathological hallmark of sclerodermatous chronic GvHD is accumulation of extracellular matrix proteins by pathologically activated fibroblasts, caused at least in part by the profibrotic cytokines TGF-β and PDGF. It was shown recently that the family of abl kinases plays an important role in the activation of matrix synthesis. Here we investigated whether imatinib and nilotinib, both targeting specifically TGF-β and PDGF signaling pathways by inhibiting the tyrosine kinase activity of c-abl and PDGF receptors, could reduce cGvHD in a murine model. Material and MethodsRecipient mice (BALB/c, H2d) received total body irradiation (2×6 Gy), followed by 1×10∧6 bone marrow cells and 2×10∧6 splenocytes from B10.D2 donors (H2d, multiple minor mismatches). Recipients receiving syngeneic bone marrow and spleen cells served as control. The treatment of transplanted mice started 12 to 14 days post transplantation. Imatinib (150 mg/kg) was given per os once daily and nilotinib (37.5 mg/kg) was administrated per os twice daily until 6 weeks after bone marrow transplantation. Observed skin changes were documented using a clinical score. After 6 weeks dermal thickness was determined by staining with hematoxylin and eosin and activated fibroblasts by using immunohistochemistry for alpha smooth muscle actin. Collagen concentration in afflicted skin was analyzed with the hydroxyproline assay. ResultsUntreated mice receiving an allogeneic transplant, in contrast to syngeneic controls, showed clinical features of cutaneous cGvHD, including skin lesions with alopecia and a lower mobility, in addition to diarrhea and weight loss until 6 weeks after alloBMT. Treatment of recipients with imatinib and nilotinib resulted in serum concentrations comparable to those achieved in humans by standard dosing. Application of imatinib or nilotinib completely prevented the development of cutaneous cGvHD including alopecia and skin ulceration, and was effective in preventing weight loss. Compared to untreated recipients dermal thickness was decreased by 100 ± 8 % (p = 0.001) with imatinib and by 54 ± 14 % (p = 0.016) with nilotinib. The numbers of myofibroblasts were reduced by 84 ± 16 % (p = 0.001) with imatinib and by 87 ± 18 % (p = 0.002) with nilotinib. Furthermore, the collagen content in afflicted skin was reduced by 83 ± 15 % with imatinib and by 94 ± 14 % with nilotinib. ConclusionsThe combined inhibition of c-abl and PDGFR by imatinib or nilotinib was effective for the prevention of sclerodermatous chronic GvHD. The data support recent clinical observations of the potential effect of imatinib in fibrotic chronic graft-versus-host disease. Disclosures:Spriewald:Novartis: Research Funding. Off Label Use: Imatinib and Nilotinib as anti-fibrotic drug in the prevention and treatment of chronic graft-versus-host disease.. Distler:Novartis: Research Funding.

Detection of Clonal T Cells in the Circulation of Patients With Nephrogenic Systemic Fibrosis

Nephrogenic systemic fibrosis is a sclerodermalike disease in patients with acute or chronic renal insuffiency related to administration of gadolinium-containing contrast agents. Previous studies have demonstrated clonal T-cell populations in the blood of patients with systemic sclerosis, suggesting that these cells may be involved in the pathogenesis of the disease. Facing the clinical similarities of both diseases, we hypothesized that clonal expansion of T cells could be present in nephrogenic systemic fibrosis as well. Findings from polymerase chain reaction and high-resolution capillary electrophoresis for T-cell receptor gamma gene rearrangement analysis showed that all 6 prospectively evaluated patients (100%) with nephrogenic systemic fibrosis had detectable clonal T cells in their peripheral blood. In contrast, only 4 of the 15 control patients (27%) with chronic renal failure and none of the 12 healthy individuals analyzed in this study had evidence for T-cell clonality using the same type of examination. Clonal T-cell-positive patients with systemic sclerosis have previously been reported to better respond to extracorporeal photopheresis. However, this was not the case in 2 of our patients with nephrogenic systemic fibrosis. Conclusion As in systemic sclerosis, clonally expanded T-cell populations could play a critical role in the pathogenesis of nephrogenic systemic fibrosis, probably as an in vivo-activated inflammatory response to gadolinium exposure.

Painful ANA-Positive Scleroderma-Like Disease With Acral Ulcerations: A Case of Chronic Gangrenous Ergotism

Chronic ergotism is a rare cause of limb ischemia. In this case report, the authors present a 62-year-old woman with history of long-term use of ergotamine alkaloids for the treatment of menstrual pain, who developed a severe painful disease initially misdiagnosed as systemic sclerosis (scleroderma) for 3 decades. She presented with a combination of acral gangrene, foot ulcer, renal obstruction, mild pulmonary fibrosis, and reduced esophageal motility. Right-sided renal obstruction was evident. The condition was extremely painful and had led to muscular contractions and immobility, drug abuse, and anemia. After establishing the diagnosis of chronic gangrenous ergotism, changing drug therapy, mobilization, and treatment of chronic wounds, she showed a remarkable recovery. Eventually the foot ulcer was closed successfully using a mesh graft transplantation, and the patient was able to walk alone. Chronic ergotism is rare but has to be taken into account when presented with painful chronic digital and foot ulcers.

Nephrogene systemische Fibrose nach Anwendung gadoliniumhaltiger Kontrastmittel - ein Statuspapier zum aktuellen Stand des Wissens

Recently the association of a rare disease named "nephrogenic systemic fibrosis" (NSF) with the administration of gadolinium-containing contrast media, especially gadodiamide (Omniscan, GE-Healthcare), was described. NSF is a scleroderma-like disease characterised by widespread tissue fibrosis. Until now, NSF cases were observed only in patients with kidney disease. Almost all patients were suffering from chronic renal insufficiency, 90 % of them required renal replacement therapy. The true incidence of the disease is unknown. First retrospective analyses of selected collectives of patients with end-stage renal disease showed 2-5 % cases of NSF after administration of Gadolinium-containing contrast agents with an odds ratio of 20-50 in comparison to non-exposed controls. NSF is a serious adverse reaction, which may result in severe disabilities and even death. Therefore all radiologists applying gadolinium-based contrast agents should be informed about this disease and the recent recommendations for its prevention. On the basis of the published data, Omniscan should not be used in patients with severe renal impairment (GFR < 30 ml/min/1.73 m(2)) and those who have had or are undergoing liver transplantation. In neonates and infants up to 1 year of age, Omniscan should only be used after careful consideration. Also the other gadolinium-based contrast agents should be used in high-risk patients only after careful consideration using the lowest dose possible.

Diminished transforming growth factor β2 production leads to increased expression of a profibrotic procollagen α2 type I messenger RNA variant in embryonic fibroblasts of UCD‐200 chickens, a model for systemic sclerosis

A procollagen alpha2(I) messenger RNA (mRNA) variant, with a 115-bp band and an expected band of 180 bp, was found to be increased during early, acute scleroderma-like disease in UCD-200 chickens. The present study investigated the influence of cytokines on the expression of these 2 proalpha2(I) mRNA variants. Embryonic fibroblasts of UCD-200 chickens (UCD-200-CEF) and normal white leghorns (NWL-CEF) were grown in 3-dimensional collagen gels. Procollagen mRNA expression was analyzed by RNase protection assay, and proliferation was determined by (3)H-thymidine incorporation. Transforming growth factor beta1 (TGFbeta1) and TGFbeta2 were measured in culture supernatants by enzyme-linked immunosorbent assay. Compared with NWL-CEF, UCD-200-CEF expressed 7.2 times more of the smaller profibrotic proalpha2(I) mRNA variant. TGFbeta1 stimulated the proliferation of UCD-200-CEF, but not NWL-CEF. The 115 bp:180 bp ratio was increased by TGFbeta1 in both NWL-CEF and UCD-CEF. TGFbeta2 and TGFbeta3 reduced the expression of the profibrotic proalpha2(I) mRNA in UCD-200-CEF to the same levels observed in healthy control NWL-CEF. In culture supernatants, NWL-CEF produced 4.1 times more TGFbeta2 than that produced by UCD-CEF. Inhibition of endogenous TGFbeta2 in NWL-CEF resulted in the same 115 bp:180 bp ratio as seen in untreated UCD-CEF. TGFbeta2 reduces the expression of a profibrotic proalpha2(I) mRNA variant in UCD-200-CEF. The constitutive overproduction of this proalpha2(I) mRNA variant and the diminished synthesis of TGFbeta2 in untreated UCD-200-CEF suggest that TGFbeta2 can act as an antifibrotic cytokine and might be a key player during fibrosis onset. These results shed light on the contradictory observations regarding the role of TGFbeta2 in human systemic sclerosis.

Chemotherapy-induced scleroderma: a pleiomorphic syndrome

A scleroderma-like disease has recently been described in association with taxanes. We present the first case of diffuse scleroderma occurring in a woman treated with doxorubicin and cyclophosphamide for breast cancer. The clinical pattern of skin involvement and histological alterations were identical to those found in the classical form of scleroderma. Skin involvement progressed to affect 80% of total body area, and subsequently remained unchanged despite progression of the underlying cancer, making a paraneoplastic aetiology of the scleroderma unlikely. Specific chemotherapeutic agents might be directly responsible for the clinical manifestations and the parameters of progression. Analysis of all similar case reports defines the particular features and clinical course of this phenomenon.

Eosinophilic Fasciitis Associated with Autoimmune Thyroiditis

Eosinophilic fasciitis (EF) is scleroderma-like disease without Raynaud's phenomenon or visceral involvement. It is characterized by painful swelling of the extremities, accompanied by rapid weight gain, fever and myalgia. The acute state of disease is associated with significant peripheral blood eosinophilia, an elevated erythrocyte sedimentation rate and hypergammaglobulinemia. EF is also frequently associated with hematological abnormalities, including malignant lymphoproliferative diseases, but rarely associated with autoimmune thyroiditis. In the present study we report a case of eosinophilic fasciitis associated with autoimmune thyroiditis.

Fascitis eosinofílica: experiencia en tres casos

Eosinophilic fasciitis (EF) is a scleroderma-like disease of unknown etiology characterized by cutaneous swelling and induration that affects predominantly the extremities, elevated immune globulins, and peripheral eosinophilia. We report three patients with clinical, laboratory and pathologic characteristics of EF. The main symptoms were cutaneous and included peau d' orange, groove signs and induration of the affected extremities. Two patients had skin changes after exercising and one had a hypothyroidism. None had extra-cutaneous manifestations. All patients had peripheral eosinophilia. Pathologic findings were thickening and inflammation of the fascia and normal epidermis. Only one patient was treated with prednisone 30 mg daily and showed only moderate improvement. There is no follow up information about the other two cases. EF is a rare disorder classified at times as scleroderma variants. However, its clinical picture, capillary microscopy findings, pathology and prognosis differentiate it from systemic sclerosis. (Rev Med Chile 2002; 130: 209-14)

Effect of Halofuginone on the Development of Tight Skin (TSK) Syndrome

The end point of pathogenic events in scleroderma is fibrosis of the skin and internal organs. Fibrosis in scleroderma results from the over synthesis and deposition of collagen in the connective tissue. The morbidity and mortality of the scleroderm is very high and presently there is no specific treatment. Halofuginone is a drug with great potential for the treatment of scleroderma since it inhibits the synthesis of collagen type I by fibroblasts. We have studied the in vivo effect of halofuginone in tight skin (TSK) mice that spontaneously develop a scleroderma-like disease due to a genetic defect. Our results demonstrate that halofuginone prevented the occurrence of skin sclerosis when administered to newborn mice and reduced cutaneous hyperplasia when administered in adult TSK mice. These effects correlated with a decreased number of cells synthesizing collagen gene transcripts and a reduction in the level of autoantibodies specific for human target antigens. These results indicate that halofuginone may have use as a therapeutic in the treatment of fibrotic disease.

Cost-Effectiveness Analyses: A Basic Overview for Dermatologists

Background: Eosinophilic fasciitis is a rare, scleroderma-like disease that usually affects the extremities of young to middle-aged males. The disease may cause flexion contractures and limit joint mobility and is associated with peripheral eosinophilia. The fascia, by definition, is infiltrated with mononuclear cells and typically with eosinophils. Eosinophilic fasciitis may be separated from another sclerodermatous disorder, linear scleroderma, by its response to systemic corticosteroids. The etiology is unclear but eosinophilic fasciitis has numerous disease associations. However, it has not previously been associated with renal failure and hemodialysis. Objective: This article reports a case of eosinophilic fasciitis occurring four weeks following the onset of hemodialysis. Methods: The clinical and histologic features confirmed the diagnosis of eosinophilic fasciitis. He was treated with systemic corticosteroids with good response. Conclusion: This is the first reported patient who developed eosinophilic fasciitis in close temporal relationship with the start of hemodialysis. While eosinophilic fasciitis may be coincidental with a common disorder, namely, renal failure, it is interesting to note that hemodialysis patients often have immune-regulation abnormalities and peripheral eosinophilia.