View source for Morphea

{{short description|Form of scleroderma involving isolated patches of hardened skin}}
{{see also|Morphia (disambiguation)|Morphine}}
{{Infobox medical condition (new)
| name            = Morphea
| synonyms        = '''Localized scleroderma''' or '''Circumscribed scleroderma'''
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'''Morphea''' is a form of [[scleroderma]] that mainly involves isolated patches of hardened skin on the face, hands, and feet, or anywhere else on the body, usually with no internal organ involvement.<ref name=FITZPATRICK2005>{{cite book|first=Thomas B. |last=Fitzpatrick |title=Fitzpatrick's color atlas and synopsis of clinical dermatology|year=2005|publisher=McGraw-Hill Medical Pub. Division|location=New York|isbn=978-0-07-144019-6|edition=5th}}</ref> However, in Deep Morphea inflammation and sclerosis can be found in the deep [[dermis]], [[panniculus]], [[fascia]], superficial muscle and bone.<ref>{{Cite journal |last=Bielsa |first=Isabel |last2=Ariza |first2=Aurelio |date=June 2007 |title=Deep morphea |url=https://pubmed.ncbi.nlm.nih.gov/17544960 |journal=Seminars in Cutaneous Medicine and Surgery |volume=26 |issue=2 |pages=90–95 |doi=10.1016/j.sder.2007.02.005 |issn=1085-5629 |pmid=17544960}}</ref>{{rp|130}}
==Signs and symptoms==
[[Image:Frontal linear scleroderma 1.JPEG|thumb|upright|Frontal linear scleroderma|alt=]]
Morphea most often presents as [[Skin condition|macules]] or plaques a few centimeters in diameter, but also may occur as bands or in [[Guttation|guttate]] lesions or nodules.<ref name="ANDREWS2005"/>{{rp|171}}
Morphea is a thickening and hardening of the skin and [[subcutaneous tissue]]s from excessive [[collagen]] deposition.  Morphea includes specific conditions ranging from very small [[Skin plaque|plaques]] only involving the skin to widespread [[disease]] causing functional and cosmetic [[Deformity|deformities]].  Morphea discriminates from [[Systemic scleroderma|systemic sclerosis]] by its supposed lack of [[Organ (biology)|internal organ]] involvement.<ref>{{cite journal |vauthors=Peterson LS, Nelson AM, Su WP |title=Classification of morphea (localized scleroderma) |journal=Mayo Clin. Proc. |volume=70 |issue=11 |pages=1068–76 |year=1995 |pmid=7475336 |doi= 10.4065/70.11.1068}}</ref>  This classification scheme does not include the mixed form of morphea in which different morphologies of [[Skin condition|skin lesions]] are present in the same individual.  Up to 15% of morphea [[patient]]s may fall into this previously unrecognized category.<ref name=Zulian>{{cite journal |vauthors=Zulian F, Athreya BH, Laxer R |title=Juvenile localized scleroderma: clinical and epidemiological features in 750 children. An international study |journal=Rheumatology (Oxford) |volume=45 |issue=5 |pages=614–20 |year=2006 |pmid=16368732 |doi=10.1093/rheumatology/kei251|doi-access= }}</ref>
==Cause==
[[Physician]]s and scientists do not know what causes morphea. Case reports and observational studies suggest there is a higher frequency of family history of autoimmune diseases in patients with morphea.<ref name=Zulian/> Tests for [[Autoantibody|autoantibodies]] associated with morphea have shown results in higher frequencies of anti-histone and anti-topoisomerase IIa antibodies.<ref>{{cite journal |vauthors=Hayakawa I, Hasegawa M, Takehara K, Sato S |title=Anti-DNA topoisomerase IIalpha autoantibodies in localized scleroderma |journal=Arthritis Rheum. |volume=50 |issue=1 |pages=227–32 |year=2004 |pmid=14730620 |doi=10.1002/art.11432 |doi-access= }}</ref> Case reports of morphea co-existing with other systemic autoimmune diseases such as primary biliary cirrhosis, vitiligo, and systemic lupus erythematosus lend support to morphea as an autoimmune disease.<ref>{{cite journal |vauthors=Majeed M, Al-Mayouf SM, Al-Sabban E, Bahabri S |title=Coexistent linear scleroderma and juvenile systemic lupus erythematosus |journal=Pediatr Dermatol |volume=17 |issue=6 |pages=456–9 |year=2000 |pmid=11123778 |doi=10.1046/j.1525-1470.2000.01820.x |s2cid=30359530 }}</ref><ref>{{cite journal |vauthors=Bonifati C, Impara G, Morrone A, Pietrangeli A, Carducci M |title=Simultaneous occurrence of linear scleroderma and homolateral segmental vitiligo |journal=J Eur Acad Dermatol Venereol |volume=20 |issue=1 |pages=63–5 |year=2006 |pmid=16405610 |doi=10.1111/j.1468-3083.2005.01336.x |s2cid=11523611 }}</ref><ref>{{cite journal |vauthors=González-López MA, Drake M, González-Vela MC, Armesto S, Llaca HF, Val-Bernal JF |title=Generalized morphea and primary biliary cirrhosis coexisting in a male patient |journal=J. Dermatol. |volume=33 |issue=10 |pages=709–13 |year=2006 |pmid=17040502 |doi=10.1111/j.1346-8138.2006.00165.x |s2cid=29859708 }}</ref>
''[[Borrelia burgdorferi]]'' infection may be relevant for the induction of a distinct autoimmune type of scleroderma; it may be called "''Borrelia''-associated early onset morphea" and is characterized by the combination of disease onset at younger age, infection with ''B. burgdorferi'', and evident autoimmune phenomena as reflected by high-titer antinuclear antibodies.<ref>{{cite journal |vauthors=Prinz JC, Kutasi Z, Weisenseel P, Pótó L, Battyáni Z, Ruzicka T|title="Borrelia-associated early-onset morphea": a particular type of scleroderma in childhood and adolescence with high titer antinuclear antibodies? Results of a cohort analysis and presentation of three cases.|journal=J Am Acad Dermatol |pmid=19022534|doi=10.1016/j.jaad.2008.09.023|volume=60|issue=2|year=2009|pages=248–55}}</ref>
==Diagnosis==
===Classification===
* '''Morphea–lichen sclerosus et atrophicus overlap''' is characterized by both lesions of morphea and lichen sclerosus et atrophicus, most commonly seen in women.<ref name=ANDREWS2005/>{{rp|171}}
* '''Generalized morphea''' is characterized by widespread indurated plaques and pigmentary changes, sometimes associated with muscle atrophy, but without visceral involvement.<ref name=ANDREWS2005/>{{rp|171}}
* '''Morphea profunda''' involves deep [[subcutaneous tissue]], including [[fascia]], and there is a clinical overlap with [[eosinophilic fasciitis]], [[eosinophilia-myalgia syndrome]], and the Spanish [[toxic oil syndrome]].<ref name=ANDREWS2005/>{{rp|171}}  Morphea profunda shows little response to [[corticosteroids]] and tends to run a more chronic debilitating course.<ref name=ANDREWS2005/>{{rp|171}}
* '''Pansclerotic morphea''' is manifested by sclerosis of the dermis, panniculus, fascia, muscle, and at times, the bone, all causing disabling limitation of motion of joints.<ref name=ANDREWS2005/>{{rp|171}}
* '''Linear [[scleroderma]]''' is a type of localised scleroderma<ref>{{DorlandsDict|seven/000095147|linear scleroderma}}</ref> which is an [[autoimmune disease]] characterized by a line of thickened skin which can affect the bones and muscles underneath it. It most often occurs in the arms, legs, or forehead, and may occur in more than one area.  It is also most likely to be on just one side of the body. Linear scleroderma generally first appears in young children.<ref name="ANDREWS2005">James, William; Berger, Timothy; Elston, Dirk (2005). ''Andrews' Diseases of the Skin: Clinical Dermatology''. (10th ed.). Saunders. Page 171. {{ISBN|0-7216-2921-0}}.</ref>
* '''Frontal linear scleroderma''' (also known as '''en coup de sabre''' or '''morphea en coup de sabre''') is a type of [[linear scleroderma]] characterized by a linear band of atrophy and a furrow in the skin that occurs in the frontal or frontoparietal [[scalp]].<ref name=Bolognia>{{cite book|author1=Rapini, Ronald P. |author2=Bolognia, Jean L. |author3=Jorizzo, Joseph L. |title=Dermatology: 2-Volume Set|publisher=Mosby|location=St. Louis|year=2007|isbn=978-1-4160-2999-1}}</ref><ref name=KATZ2003>{{cite journal|last=Katz|first=KA|title=Frontal linear scleroderma (en coup de sabre).|journal=Dermatology Online Journal|date=October 2003|volume=9|issue=4|pages=10|pmid=14594583}}</ref>  Multiple lesions of ''en coup de sabre'' may coexist in a single patient, with one report suggesting that the lesions followed [[Blaschko's lines]].<ref name=KATZ2003 /> It gets its name from the perceived similarity to a [[sabre]] wound.<ref>{{DorlandsDict|nine/000951723|coup de sabre}}</ref>
[[Image:Frontal linear scleroderma 2.JPEG|thumb|upright|Frontal linear scleroderma|alt=A young girl with facial palsy, complete ptosis, and marked atrophy of subcutaneous and bony structures on the left upper side of the face]]
* '''Atrophoderma of Pasini and Pierini''' (also known as "Dyschromic and atrophic variation of scleroderma,"<ref name="Bolognia" /> "Morphea plana atrophica,"<ref name="Bolognia" /> "Sclérodermie atrophique d'emblée"<ref name="Bolognia" />) is a disease characterized by large [[lesion]]s with a sharp peripheral border dropping into a depression with no outpouching, which, on [[biopsy]], elastin is normal, while collagen may be thickened.<ref name="Fitz2">Freedberg, et al. (2003). ''Fitzpatrick's Dermatology in General Medicine''. (6th ed.). Page 1029. McGraw-Hill. {{ISBN|0-07-138076-0}}.</ref> Atrophoderma of Pasini and Pierini affects less than 200,000 Americans and is classified as a rare disease by http://rarediseases.info.nih.gov.  The disease results in round or oval patches of hyper-pigmented skin. The darkened skin patches may sometimes have a bluish or purplish hue when they first appear and are often smooth to the touch and hairless.
==Treatment==
Throughout the years, many different treatments have been tried for morphea including topical, intra-lesional, and systemic corticosteroids.  Antimalarials such as hydroxychloroquine or chloroquine have been used.  Other immunomodulators such as [[methotrexate]], topical [[tacrolimus]], and [[penicillamine]] have been tried. Children and teenagers with active morphea (linear scleroderma, generalised morphea and mixed morphea: linear and circumscribed) may experience greater improvement of disease activity or damage with oral methotrexate plus [[prednisone]] than with placebo plus prednisone.<ref name=":0">{{Cite journal|last1=Albuquerque|first1=Julia V de|last2=Andriolo|first2=Brenda NG|last3=Vasconcellos|first3=Monica RA|last4=Civile|first4=Vinicius T|last5=Lyddiatt|first5=Anne|last6=Trevisani|first6=Virginia FM|date=2019-07-16|title=Interventions for morphea|journal=Cochrane Database of Systematic Reviews|volume=2019|issue=7 |pages=CD005027|doi=10.1002/14651858.cd005027.pub5|issn=1465-1858|pmc=6630193|pmid=31309547}}</ref>  Some have tried prescription vitamin-D with success. Ultraviolet A (UVA) light, with or without psoralens have also been tried.  UVA-1, a more specific wavelength of UVA light, is able to penetrate the deeper portions of the skin and thus, thought to soften the plaques in morphea by acting in two fashions: by causing a systemic immunosuppression from UV light, or by inducing enzymes that naturally degrade the collagen matrix in the skin as part of natural sun-aging of the skin. [http://www.menzilim.org/] {{Webarchive|url=https://web.archive.org/web/20190203072618/https://www.menzilim.org/ |date=2019-02-03 }} However, there is limited evidence that UVA‐1 (50 J/cm<sup>2</sup>), low‐dose UVA‐1 (20 J/cm<sup>2</sup>), and narrowband UVB differ from each other in effectiveness in treating children and adults with active morphea.<ref name=":0" />
==Epidemiology==
Morphea is a form of scleroderma that is more common in women than men, in a ratio 3:1.<ref>{{cite journal |vauthors=Laxer RM, Zulian F |title=Localized scleroderma |journal=Curr Opin Rheumatol |volume=18 |issue=6 |pages=606–13 |year=2006 |pmid=17053506 |doi=10.1097/01.bor.0000245727.40630.c3 |s2cid=42839634 }}</ref> Morphea occurs in childhood as well as in adult life.<ref name=ANDREWS2005 />
Morphea is an uncommon condition that is thought to affect 2 to 4 in 100,000 people.<ref>{{cite journal |vauthors=Peterson LS, Nelson AM, Su WP, Mason T, O'Fallon WM, Gabriel SE |title=The epidemiology of morphea (localized scleroderma) in Olmsted County 1960-1993 |journal=J. Rheumatol. |volume=24 |issue=1 |pages=73–80 |year=1997 |pmid=9002014 }}</ref>  Adequate studies on the incidence and prevalence have not been performed.  Morphea also may be under-reported, as physicians may be unaware of this disorder, and smaller morphea plaques may be less often referred to a dermatologist or rheumatologist.  {{citation needed|date=August 2013}}
==See also==
* [[List of cutaneous conditions]]
==References==
{{Reflist}}
==Further reading==
* [http://archderm.jamanetwork.com/article.aspx?articleID=1740691 JAMA Dermatology Patient Page.] Morphea (Localized Scleroderma. Nicole M. Fett, MD. JAMA Dermatol. 2013;149(9):1124. doi:10.1001/jamadermatol.2013.5079. September 2013
== External links ==
{{Medical resources
|  DiseasesDB      = 8351
|  ICD10           = {{ICD10|L|94|0|l|80}}
|  ICD9            = {{ICD9|701.0}}
|  ICDO            =
|  OMIM            =
|  MedlinePlus     =
|  eMedicineSubj   = derm
|  eMedicineTopic  = 272
|  MeshID          = D012594
}}
{{Diseases of the skin and appendages by morphology}}
{{Localized connective tissue diseases}}
[[Category:Autoimmune diseases]]
[[Category:Rheumatology]]
[[Category:Connective tissue diseases]]