En Coup de Sabre
Radiation Port Sclero
Stiff Skin Syndrome
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. Medscape.
(Case Report) 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, . (Also see Types of Scleroderma: Nodular)
Bullous Pemphigoid. American Osteopathic College of Dermatology.
Bullous Pemphigoid. Bullous pemphigoid is a skin disorder characterized by large blisters. Medline Plus.
Autoimmune blistering diseases of the skin. In this review, the focus is on autoimmune bullous diseases with the skin as the major target which involve antigens of epidermis, basal membrane or dermis. Sticherling M. (PubMed) Autoimmun Rev, 2011 May 27.
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 means that somebody has injured their own skin, by any method. ISN.
Causes of Dermatitis Artefacta
Types of Dermatitis Artefacta
Diagnosis of Dermatitis Artefacta
|Differential Diagnosis |
— Medical Causes of Itching
Treatment of Dermatitis Artefacta
Case Histories of Dermatitis Artefacta
Eosinophilic Fasciitis The debate is ongoing as to whether or not eosinophilic fasciitis is a form of scleroderma. ISN.
| What is Eosinophilic Fasciitis? |
Scleroderma and EF
Symptoms of Eosinophilic Fasciits
Diagnosis of Eosinophilic Fasciitis
| Research about Eosinophilic Fasciitis |
Personal Stories of Eosinophilic Fasciitis
Eosinophilia-Myalgia Syndrome (EMS) is a multi-systemic disease caused by ingestion of impure L-tryptophan, an amino acid dietary supplement.
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.
|Overview of GVHD
Causes of GVHD
Diagnosis of GVHD
|Treatments for Graft-versus-Host Disease (GVHD)
GVHD Patient Stories
Research for GVHD
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.
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. ISN.
Lichen Sclerosus (aka Lichen Sclerosis or Lichen Sclerosus et Atrophicus) is a skin condition that affects the vulva (or penis) and anus. ISN.
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.
Semicircular Localized Lipodystrophy
Types of Morphea
Causes of Morphea
Complications of Morphea
Patient and Caregiver Stories
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 Systemic Fibrosis (NSF). Nephrogenic systemic fibrosis resembles scleroderma and eosinophilic fasciitis clinically and scleromyxedema histopathologically. Patients with NSF may develop large areas of indurated skin with fibrotic nodules and plaques. Medscape.
Sclerotic bodies beyond nephrogenic systemic fibrosis. Sclerotic bodies may be found in skin biopsies from patients with nephrogenic systemic fibrosis (NSF). Journal of Cutaneous Pathology, 29 Jun 2013.
Scleroderma in Overlap. When Systemic Sclerosis (in either the Limited or the Diffuse form) appears in conjunction with features of one or more other connective tissue diseases (such as Systemic Lupus Erythematosus, Polymyositis, Dermatomyositis) it is called "overlap." ISN.
|Overview of Polyautoimmunity
Scleroderma in Overlap
Connective Tissue Disease (CTD)
Mixed Connective Tissue Disease (MCTD)
|Multiple Autoimmune Syndrome
Undifferentiated Connective Tissue Disease (UCTD)
Overlap Patient and Caregiver Stories
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.
What is Pemphigus Vulgaris? Pemphigus vulgaris is an autoimmune skin disorder characterized by blistering of the skin and mucous membrane. Medline Plus.
Allogenic hematopoietic stem cell transplantation (HSCT) in pemphigus vulgaris: a single-center experience. Drug-resistant Pemphigus Vulgaris can be successfully and safely treated by allogeneic HSCT. Indian J Dermatol, 2012 Jan;57(1):9-11.
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.
Perioral Dermatitis (Also called periorificial dermatitis) is a facial rash that tends to occur around the mouth and/or nose and occasionally, the eyes. ISN.
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.
The effects of alcohol and drug abuse on the skin. Skin changes associated with alcohol and drug abuse can be the earliest clinical manifestation of several disorders such as psoriasis, porphyria cutanea tarda, and hyperpigmentation. Liu SW. (PubMed) Clin Dermatol, 2010 Jul-Aug;28(4):391-9. (Also see Psoriasis and Porphyria Cutanea Tarda)
A 63-year-old man developed generalized scleroderma 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)
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.
Progeria syndromes and ageing: what is the connection? One of the many debated topics in ageing research is whether progeroid syndromes are really accelerated forms of human ageing. Burtner CR. (PubMed) Nat Rev Mol Cell Biol, 2010 Aug;11(8):567-78.
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.
Psoriasis and Psoriatic Arthritis. Psoriasis is an autoimmune skin disease that causes scaly red patches of skin. Sometimes people with psoriasis develop joint pain, which is called psoriatic arthritis. ISN.
Mortality and Prognosis
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.
What is Radiation?
Immune System and Radiation
What is Scleroderma?
Radiation and Scleroderma
|Radiation Complications in Scleroderma
Radiation Port Scleroderma
Personal Stories About Radiation
Schnitzler syndrome (SS), first reported in 1972,1 is characterized by chronic, nonpruritic urticaria in association with recurrent fever, bone pain, arthralgia or arthritis, and a monoclonal immunoglobulin M (IgM) gammopathy in a concentration that is usually less than 10 g/L. Since 1972, approximately 100 cases of Schnitzler syndrome have been reported. Medscape.
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.
|Overview of Scleredema |
Treatments for Scleredema
Scleredema Patient Stories
|Scleredema Resources |
Phaeochromocytoma Mimicking Scleroderma. Phaeochromocytoma should be suspected in patients with features of scleroderma who are negative for auto-antibodies. (Hindawi) Joan Joo-Ching Khoo. International Journal of Endocrinology Volume 2011 (2011), Article ID 917453. (Also see Scleroderma-Like)
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)
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...
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.
Mutations in Fibrillin-1 Cause Congenital Scleroderma: Stiff Skin Syndrome (SSS). We report that stiff skin syndrome (SSS), an autosomal dominant congenital form of scleroderma, is caused by mutations in the sole Arg-Gly-Asp sequence–encoding domain of fibrillin-1 that mediates integrin binding. B. L. Loeys. Sci Transl Med 17 March 2010: Vol. 2, Issue 23, p. 23ra20.
Determination of dermatology life quality index, and serum C-reactive protein (CRP) and plasma interleukin-6 (IL-6) levels in patients with chronic urticaria (CU). These findings support the relationship between the inflammatory process in chronic urticaria and the clinical findings. Ter Media, March 2013.
Urticaria. Urticaria may be confused with a variety of other dermatologic diseases that are similar in appearance and are pruritic including atopic dermatitis (eczema), maculopapular drug eruptions, contact dermatitis, insect bites, erythema multiforme, pityriasis rosea, and others. Medscape.
Diseases Similar to Scleroderma: Vitiligo Includes overview, symptoms, causes, treatments, research and personal stories. ISN.
|What is Vitiligo?
Causes of Vitiligo
|Diagnosis of Vitiligo
Treatments for Vitiligo
Warts. People with immune deficiencies are more susceptible to contracting the virus that causes warts. Common warts, genital warts, and plantar warts are all caused by different strains of the human papillomavirus. Warts.org.
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.
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