| Skin
Diseases Similar to Scleroderma (Main Menu) |
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| 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. |
| Mandibuloacral Dysplasia |
| What
is Mandibuloacral Dysplasia? Mandibuloacral dysplasia
(MAD) is a rare autosomal recessive disorder, characterized
by postnatal growth retardation, craniofacial anomalies,
skeletal malformations, and mottled cutaneous pigmentation. Am
J Hum Genet. 2002 August; 71(2): 426–431. |
| A
case of mandibuloacral dysplasia presenting with features
of scleroderma. Mandibuloacral dysplasia should be considered
in the differential diagnosis of juvenile scleroderma in
the presence of atypical features such as negative serological
studies, absence of Raynaud's phenomenon, sparse hair and
micrognathia. PubMed. Int J Clin Pract. 2004 Jun;58(6):635-8.
(Also see: Types
of Scleroderma) |
| 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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