| Skin Diseases Similar to Scleroderma (Main Menu) | | | |
| About Dermatology |
| Scleroderma-like Fibrosing Disorders. Many conditions presenting with clinical hard skin and tissue fibrosis can be confused with systemic sclerosis (scleroderma). (ScienceDirect) Rheumatic Disease Clinics of North America Vol 34:1, Feb 2008, Pp 199-220. |
| Dermatology DermWeb. |
| Skin and Connective Tissue Diseases Karolinska Institutet, Sweden. |
| Acrokeratoelastoidosis |
| Acrokeratoelastoidosis (AKE) is a rare genodermatosis characterized by small, firm papules or plaques on the sides of the hands and feet. These nodules may result from an abnormality in the secretion or excretion of elastic material by fibroblasts in the dermis. Emedicine. |
| Acrokeratoelastoidosis associated with nodular scleroderma. Because there is a paper describing the association of acrokeratoelastoidosis with systemic scleroderma, coexistence of acrokeratoelastoidosis and nodular scleroderma in the patient suggests that acrokeratoelastoidosis is related to localized nodular scleroderma as well as systemic scleroderma. PubMed. Eur J Dermatol. 2003 Sep-Oct;13(5):490-2. (Also see: Types of Scleroderma: Nodular) |
| Atrophoderma of Pierini and Pasini |
| Atrophoderma of Pierini and Pasini APP) is similar to Morphea Scleroderma. Some doctors think it represents a late-stage Morphea. ISN. |
| Bullous Pemphigoid |
| Bullous Pemphigoid. American Osteopathic College of Dermatology. |
| Bullous Pemphigoid. Bullous pemphigoid is a skin disorder characterized by large blisters. Medline Plus. |
| Antiphospholipid antibodies (aPL) in patients with autoimmune blistering disease (ABD). aPLs are frequently detected in patients with ABD. Careful examination and follow-up for thromboembolism may be necessary in ABD patients with aPLs. Journal of the American Academy of Dermatology Vol 57, Issue 3, September 2007, Pages 397-400. (Also see: Pemphigus vulgaris ) |
| Bullous Pemphigoid in a Patient with Systemic Sclerosis (Scleroderma). Diagnosis of blistering skin disease like bullous pemphigoid can be overlooked in patients with scleroderma because significant pruritus and ulcerations are commonly part of the scleroderma skin process. J Rheumatol 2006 October;33:2098. Letters. |
| Dermatitis Artefacta or Factitial Dermatitis |
| Dermatitis Artefacta means that somebody has injured their own skin, by any method. They may injure their skin by scratching it, with their fingernails or a knife or other sharp instrument; burning it with fire such as cigarettes, matches, or candles; burning it with caustic chemicals, such as bleach. They may or may not be aware that they caused the damage themselves, but they typically deny having intentionally inflicted the injury. ISN. |
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| Eosinophilic Fasciitis (Shulman's Syndrome) |
| Eosinophilic Fasciitis The debate is ongoing as to whether or not eosinophilic fasciitis is a form of scleroderma. ISN. |
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| Eosinophilia-Myalgia Syndrome |
| Eosinophilia-Myalgia Syndrome (EMS) is a multi-systemic disease caused by ingestion of impure L-tryptophan, an amino acid dietary supplement. |
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| Graft-versus-Host Disease (GVHD) |
| Graft-versus-Host Disease (GVHD) is an autoimmune response to foreign cells, such as from skin grafting, bone marrow transplants, stem cell transplants, blood transfusions, or organ transplants. |
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| Grzybowski Syndrome |
| Grzybowski generalized eruptive keratoacanthoma. Grzybowski syndrome, or generalized eruptive keratoacanthoma, refers to a very rare disease in which hundreds of keratoacanthoma-like papules appear. Grzybowski syndrome, or generalized eruptive keratoacanthoma, refers to a very rare disease in which hundreds of keratoacanthoma-like papules appear. DermNet NZ. Dec 2006. |
| Keloidal Scleroderma |
| Keloids are smooth, shiny, flesh-colored, raised growths of fibrous tissue that form over areas of injury or surgical wounds. Keloidal Scleroderma is a rare, disfiguring variant of scleroderma. |
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| Lichen Sclerosus or Lichen Sclerosus et Atrophicus |
| Lichen Sclerosus (aka Lichen Sclerosis or Lichen Sclerosus et Atrophicus) is a skin condition that affects the vulva (or penis) and anus. ISN. |
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| Lipodermatosclerosis |
| Lipodermatosclerosis (LDS) in patients with diffuse connective tissue diseases (DCTD). LDS is a clinical condition characterized by the appearance of hardened, painful, and hyperchromic plaques on the legs. Its recognition is very relevant since it is characterized by thickening of the skin, similar to scleroderma.PubMed. Eur J Intern Med. 2006 Jul;17(4):288-9. |
| Lipodystrophy, Lipoatrophy |
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| Morphea Scleroderma |
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| Myeloma (Bone Marrow Cancer) |
| Familial characteristics of autoimmune and hematologic disorders in 8,406 multiple myeloma patients. There was generally no increase in risk of multiple myeloma in probands whose relatives had hematologic malignancies other than multiple myeloma. PubMed. InterScience Weekly. 06/15/06. |
| Multiple myeloma with scleroderma-like changes. We present a case of a patient who presented concomitantly with generalized pruritus, brownish sclerodermatous plaques, sclerodactyly and a monoclonal band for IgG-kappa. The patient was diagnosed as having multiple myeloma by bone marrow examination. PubMed. J Eur Acad Dermatol Venereol. 2005 Jul;19(4):500-2. (Also see: Scleroderma and Cancer) |
| Nephrogenic Fibrosing Dermopathy |
| Nephrogenic fibrosing dermopathy/nephrogenic systemic fibrosis (NFD/NSF) in 2 patients with end-stage renal disease on hemodialysis. NFD/NSF can pose many diagnostic challenges. A team of experienced rheumatologists, nephrologists, dermatologists, and dermatopathologists is essential in identifying this disorder. PubMed. J Clin Rheumatol. 2006 Jun;12(3):134-6. (Also see: Renal Involvement) |
| Nephrogenic systemic fibrosis (NSF): an emerging threat among renal patients. NSF is a scleroderma-like skin disease observed in patients with renal insufficiency. Recent observations indicate that specific cells normally involved in wound repair and tissue remodeling may be aberrantly recruited to the skin and soft tissues of NSF patients. PubMed. Semin Dial. 2006 May-Jun;19(3):191-4. (Also see: Renal Involvement) |
| Nephrogenic fibrosing dermopathy, a new mimicker of systemic sclerosis. This is a unique fibrosing disorder recently identified to occur exclusively among patients with renal disease. The cutaneous findings are similar to those of systemic sclerosis, but it is important to differentiate between these two disorders because of significant prognostic and therapeutic implications. PubMed. Am J Med Sci. 2005 Oct;330(4):192-4. (Also see: Renal Involvement) |
| Involvement of skeletal muscle in dialysis-associated systemic fibrosis (nephrogenic fibrosing dermopathy). Nephrogenic fibrosing dermopathy (NFD), a newly recognized scleroderma-like disease, was originally described as a purely cutaneous disorder. More widespread involvement, including fibrosis of pulmonary and cardiac tissues, has been documented only recently, and it has been suggested that a more appropriate designation is dialysis-associated systemic fibrosis. PubMed. Muscle Nerve. 2004 Sep 7. (Also see: Dialysis) |
| Dialysis-associated systemic fibrosis (nephrogenic fibrosing dermopathy): Study of inflammatory cells and transforming growth factor beta1 expression in affected skin. Our findings indicate that the fibrotic process of NFD affects not only the dermis, but also the subcutaneous tissues, fascia, and other organs, including striated muscles, heart, and lungs. PubMed. Arthritis Rheum. 2004 Aug;50(8):2660-6. (Also see: Dialysis) |
| Progressive Fibrosis with Features of Systemic Sclerosis and Diffuse Fasciitis in Patients with Chronic Dialysis for End-Stage Renal Disease: Clinical Description of Eight New Cases. The fibrotic process is not confined to the dermis but involves the subcutaneous fascia and the connective tissue of striated muscles, myocardium and pulmonary vasculature. Sergio A. Jimenez. ACR Conference Oct. 2003. |
| Nephrogenic fibrosing dermopathy: the first 6 years. Nephrogenic fibrosing dermopathy (NFD) is a newly recognized scleroderma-like fibrosing skin condition. It develops in patients with renal insufficiency. Recent studies have contributed to a clearer definition of the clinical spectrum, epidemiology, and pathogenesis of NFD. PubMed. Curr Opin Rheumatol. 2003 Nov;15(6):785-90. (Also see: Kidney Involvement) |
| Nephrogenic fibrosing dermopathy: an unusual skin condition associated with kidney disease. We report the case of a patient who, while on long-term hemodialysis (HD), developed nephrogenic fibrosing dermopathy, a newly described sclerosing skin disorder. This disorder is characterized by thickened, hardened skin with brawny hyperpigmentation and raised plaques. In contrast to scleroderma, inflammatory cells are generally absent. PubMed. Semin Dial 2003 May-Jun;16(3):276-80 (Also see: Kidney Involvement) |
| Parry-Rombergs Syndrome |
| Parry-Rombergs Syndrome, aka Progressive Hemifacial Atrophy or HFA. This may be the same as Linear Scleroderma, affecting the facial area. It usually affects the left half of the face and the facial atrophy may be accompanied by other symptoms, including migraine headaches and trigeminal neuralgia. ISN. |
| Pemphigus Vulgaris |
| What is Pemphigus Vulgaris? Pemphigus vulgaris is an autoimmune skin disorder characterized by blistering of the skin and mucous membrane. Medline Plus. |
| Antiphospholipid antibodies (aPL) in patients with autoimmune blistering disease (ABD). aPLs are frequently detected in patients with ABD. Careful examination and follow-up for thromboembolism may be necessary in ABD patients with aPLs. Journal of the American Academy of Dermatology Vol 57, Issue 3, September 2007, Pages 397-400. (Also see: Bullous pemphigoid) |
| Autoimmune Disease Yields to Single Rituximab Cycle. In a small open-label study, 86% of patients with severe pemphigus had complete healing of their skin and mucosal lesions three months after receiving one four-week cycle of rituximab. Medpage Today. 08/08/07. |
| Treatment of Pemphigus Vulgaris with Rituximab and Intravenous Immune Globulin. The combination of rituximab and intravenous immune globulin is effective in patients with refractory pemphigus vulgaris. The New England Journal of Medicine Vol 355:1772-1779, No 17 Oct 26 2006. |
| Scientists stop autoimmune disease without shutting off immune system. The development of drugs that completely suppress the immune system offered a lifeline to patients with pemphigus vulgaris (PV) and other autoimmune disorders, but the drugs themselves can be lethal and often cause serious side effects. Now researchers have found a safer, more effective way to treat PV patients. EurekAlert! 08/22/06. |
| POEMS |
| What is POEMS? Common symptoms include progressive weakness of the nerves in the arms and legs, an abnormally enlarged liver and/or spleen (hepatosplenomegaly), abnormally darkening of the skin (hyperpigmentation) and excessive hair growth (hypertrichosis). WebMD. |
| POEMS stands for Polyneuropathy, Organomegaly, Endocrinopathy, M-proteins, and Skin changes. The skin changes may be similar to Scleroderma. It is also known as Crow-Fukase Syndrome, PEP Syndrome, Polyeuropathy-organomegaly-endocrinopathy-M protein-skin lesions, Shimpo syndrome, Takatsuki syndrome, and Polyneuropathy-organomegaly-endocrinopathy-M protein-skin lesions. |
| Porphyria Cutanea Tarda (PCT) |
| A 63 year old man developed generalized scleroderma with massive sclerotic areas, particularly in the abdominal region, four years after being diagnosed with porphyria cutanea tarda (PCT). He had almost daily exposure to organic solvents (benzene, trichlorethylene) for many years. The pansclerotic PCT was differentiated from a systemic sclerosis, a disabling pansclerotic morphea and a generalized morphea by means of histological examinations, the absence of a Raynaud phenomenon and the non-involvement of additional organs. Auto-antibodies typical for systemic sclerosis were negative. Using a medium dosage of UVA1 phototherapy and intensive physiotherapy, the progression of the skin disease was stopped and the sclerosis improved. PubMed. Hautarzt 2003 May;54(5):448-52(Also see: Causes of Scleroderma) |
| Progeria, Hutchinson-Gilford Progeria |
| What is Progeria? Progeria syndromes give the appearance of premature aging. Many people know progeria from seeing children who look very old on TV talk shows and documentaries. Those children have the more rare form of progeria; there is another more common type that affects adolescents and adults. About.com. |
| What is Hutchinson-Gilford Progeria Syndrome? It is a form of progeria that begins to show around 6 to 12 months of age, when the child begins to look like an elderly person. The life expectancy is about 13 years. About.com. |
| Hutchinson-Gilford progeria syndrome (HGPS): Review of the phenotype. HGPS is a rare but well known entity characterized by extreme short stature, low body weight, early loss of hair, lipodystrophy, scleroderma, decreased joint mobility, osteolysis, and facial features that resemble aged persons. Cardiovascular compromise leads to early demise. Cognitive development is normal. PubMed. Am J Med Genet A. 2006 Jul 12. |
| Tight skin and limited joint movements as early presentation of Hutchinson-Gilford progeria in a 7-week-old infant. Hutchinson-Gilford syndrome is an extremely rare disorder of which the full clinical spectrum becomes evident with time. Sclerodermatous changes in the infant can be the first manifestation. PubMed. Eur J Pediatr. 2005 Feb 22. |
| Pseudoscleroderma |
| Pseudoscleroderma refers to skin diseases that are similar to either systemic or localized scleroderma. A broad spectrum of unrelated disorders is included in this category, such as scleredema, diffuse fasciitis with blood eosinophilia, progeria, Werner's disease, carcinoid syndrome, chronic graft-versus-host disease, porphyria cutanea tarda, phenylketonuria, scleromyxoedema, scleroderma-like lesions due to bleomycin therapy, occupational sclerodermas and melorheostosis with linear scleroderma. |
| Pseudoscleroderma associated with cancer. In 71 patients with sclerotic skin changes; 66 were diagnosed with systemic sclerosis (SSc), five were diagnosed with pseudoscleroderma associated with various malignancies. The mean duration of disease in the five patients was significantly shorter than that of the SSc patients. PubMed. Clin Exp Dermatol. 2006 May;31(3):381-3. (Also see: Scleroderma and Cancer) |
| A case of pseudoscleroderma as paraneoplastic syndrome due to carcinoma of cervical uteri. We immunochemically investigated the pathogenesis of paraneoplastic syndrome and found that connective tissue growth factor (CTGF) might be involved and transforming growth factor-beta (TGF-beta) might not be involved in this case. PubMed. Nihon Rinsho Meneki Gakkai Kaishi. 2003 Oct;26(5):293-8. |
| Radiation Port Scleroderma |
| Causes of Scleroderma: Radiation There is an increased rate of cancer among scleroderma patients. Furthermore, scleroderma patients have an increased sensitivity to radiation treatments. In addition, radiation can cause scleroderma including Radiation Port Scleroderma. |
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| Schnitzler Syndrome |
| Schnitzler Syndrome: Beyond the Case Reports: Review and Follow-Up of 94 Patients with an Emphasis on Prognosis and Treatment. Schnitzler syndrome is a disabling disorder which affects multiple systems and which can be considered as an autoinflammatory syndrome. There are new, effective treatment options, but close monitoring remains warranted because of the increased risk of lymphoproliferative disease. Science Direct. j.semarthrit.2007.04.001. |
| Scleredema adultorum Buschke |
| Scleredema is an illness similar to scleroderma. It is also known as Scleredema Adultorum, Scleredema Adultorum of Buschke, Scleredema Diabeticorum, and Scleredema Diabeticorum of Buschke. |
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| Scleroderma-Like |
| Unilateral limited scleroderma-like changes following formation of an arteriovenous fistula. The arteriovenous fistula for haemodialysis had been formed on the same arm 2 years previously. Tissue hypoxia is believed to be a contributing factor in the pathogenesis of scleroderma, and this case demonstrates scleroderma-like changes in the setting of limb ischaemia. PubMed. Australas J Dermatol. 2007 Feb;48(1):37-9. (Also see: Renal Involvement ) |
| Taxane-induced scleroderma. Although the mechanisms have not been clarified, it should be noted that taxane is causally involved in the formation of scleroderma-like skin conditions. PubMed Br J Dermatol. 2007 Feb;156(2):363-7. (Also see: Causes of Scleroderma: Drugs and Medications) |
| Reticulate hyperpigmented scleroderma: a new pigmentary manifestation. We report another unrecognized pigmentary abnormality in a 48-year-old patient who presented with a reticulate hyper-pigmentation affecting the trunk, upper and lower limbs. To our knowledge, cutaneous hyperpigmented reticulate scleroderma has not been reported previously. PubMed. Clin Exp Dermatol. 2005 Mar;30(2):131-3. |
| Gemcitabine-associated scleroderma-like changes of the lower extremities. Gemcitabine is a nucleosid analog approved for use in the treatment of metastatic urothelial carcinoma of the bladder. This is the first case report of a scleroderma-like reaction associated with gemcitabine. This antineoplastic agent must be added to the very limited number of cytostatic agents capable of giving rise to scleroderma-like features. PubMed. J Am Acad Dermatol. 2004 Aug;51(2 Suppl):S73-6. (Also see: Causes of Scleroderma: Medications) |
| A case of scleroderma spectrum disorder with anticentriole antibody and pulmonary hypertension. A 54-year-old woman had noticed Raynaud's phenomenon and digital ulcers during the winter for the past 10 years. Although sclerodactyly was not present, digital ulcers, swelling of her hands, and phalangeal contracture were observed. This is the first case of SSD with anticentriole antibody to develop pulmonary hypertension. PubMed. Clin Rheumatol. 2004 Jun;23(3):266-8. (Also see: Pulmonary Hypertension) |
| Donna S: Odd Version of Scleroderma My dermatologist has spoken with numerous dermatologists across the country, and they have not heard of my particular case of scleroderma. They also have not heard of blisters being associated with scleroderma in any form... |
| Scleromyxedema aka Lichen Myxedematosus or Papular Mucinosis |
| Diseases Similar to Scleroderma: Scleromyxedema is a scleroderma-like condition. However, it causes thickening and hardening that makes the skin look too ample, rather than too tight, as in scleroderma. It is also referred to as Scleromyxedema of Groton, lichen myxedematosus, and papular mucinosis. Overview, symptoms, diagnosis, treatments, support, and personal stories. ISN. |
| Stiff Skin Syndrome (SSS) |
| Stiff skin syndrome: a case report. Stiff skin syndrome (SSS) is a disease similar to scleroderma with an unknown etiology. Stone-hard areas of skin are observed from birth or in early childhood. PubMed. Pediatr Dermatol. 2003 Jul-Aug;20(4):339-41. |
| Vitiligo |
| Diseases Similar to Scleroderma: Vitiligo Includes overview, symptoms, causes, treatments, research and personal stories. ISN. |
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| Werner's Syndrome |
| Werner's Syndrome: A Rare Cause of Hoarseness. A 31-year-old man presented with a 12-year history of hoarseness. Also noted were diabetes mellitus, cataracts, scleroderma-like skin atrophy, osteoporosis, and hypogonadism. A clinical diagnosis of WS was made. PubMed. J Voice. 2007 Jan 20. |
| Werner's syndrome and endocrine disorders. Werner's syndrome is a rare autosomal recessive disease caused by the mutation of DNA helicase gene (WRN), characterized by the premature onset of multiple age-related disorders and skin changes similar to those observed in scleroderma. PubMed. Ann Endocrinol (Paris). 2003 Jun;64(3):205-9. |
| Soft-tissue mineralization in Werner syndrome. A review of previous descriptions of the radiological abnormalities of Werner syndrome indicates that the presence of soft-tissue calcifications has either not been noted or been mentioned only briefly. Moreover, there is no mention of bony masses associated with Werner syndrome in the world literature, and this would appear to be the first report of this kind. PubMed. Skeletal Radiol. 2004 May 11. (Also see: Diseases Similar to Scleroderma: Werner's Syndrome and Calcinosis) |
| Neurological complications of Werner's syndrome. Some of the neurological complications are secondary to premature cerebrovascular disease, but the pathogenesis of peripheral neuropathy and myelopathy in patients with Werner's syndrome is uncertain. PubMed. J Neurol. 2003 Oct;250(10):1174-8. |
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