Note that this may not provide an exact translation in all languages, breadcrumbs Elastosis perforans serpiginosa (EPS) is a perforating disorder. Pediatr Dermatol. They form a characteristic component of a number of inherited disorders such as tuberous sclerosis, but may also occur as isolated lesions without any identifiable genetic basis. Fibroblastic connective tissue nevus: a rare cutaneous lesion analyzed in a series of 25 cases Am J Surg Pathol . The initial diagnosis may be made … Slowly growing lesions were found at the surgical site, which necessitated hospitalization. 1056490-overview Loss-of-function mutations in LEMD3 result in osteopoikilosis, Buschke-Ollendorff syndrome and melorheostosis. The entity was recognized by Sebastian de Feraudy and Christopher Fletcher where they reported a series of twenty-five cases of a seemingly distinctive lesion for which they use the appellation fibroblastic connective tissue nevus. Nevus connective tissues are formed due to defects in the development of essential dermal substances. Collagenoma, a type of Connective tissue nevus, is an rare condition in which the cells in the deeper layers of the skin experience an excess of collagen. Vascular nevi are derived from structures of the blood vessels. Buschke-Ollendorf syndrome is a rare hereditary disorder where there is an increased accumulation of elastin in the dermis (elastoma). Fourth edition. An 8-year-old girl presented with a two-year history of a progressive ‘wrinkling’ of her cheeks. In most cases, when there are no neurological problems, the prognosis is good, but it is necessary for the lesions to be examined regularly. 1112322-overview Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Texas Medical Association, Association of Military Dermatologists, Texas Dermatological SocietyDisclosure: Nothing to disclose. 2. Biopsies are suggested to be into subcutaneous tissue and include adjacent normal skin to compare with the suspected connective tissue nevus. They are firm yellowish wrinkled nodules often group together to form plaques. Mixed connective tissue disease has signs and symptoms of a combination of disorders — primarily lupus, scleroderma and polymyositis. Connective tissue naevi are thought to be due to genetic defects in the skin cells. A biopsy showed changes consistent with a connective tissue nevus. [Medline]. Treatment for giant congenital nevus depends on the age of the affected individual as well as the size, location, and thickness of the nevus. 2009 Sep-Oct. 26(5):626-8. 2005 Aug. 53(2 Suppl 1):S150-3. It is usually a single irregularly shaped lesion that once established remains unchanged. The condition occurs with both types of scleroderma: If concern for systemic associations, then need appropriate workup and referral. The naevi may also include excess glycosaminoglycans (ground substance of the dermis), excess smooth muscle or excess fat. The fascia is a thin sheath of fibrous connective tissue that lies between the skin and the underlying muscles, nerves, and bones. The findings are very distinctive and most compatible with a fibroblastic connective tissue nevus. Light and electron microscopy of eruptive collagenoma. Especially if concern for tuberous sclerosis, a Wood’s lamp may be helpful in screening for hypopigmented macules or patches. Topics A–Z There are two subtypes of collagenoma: shagreen patches and familial cutaneous Collagenoma (FCC). Patients with scleroderma may develop telangiectasias on the face, mucous membranes and hands. A connective tissue naevus (American spelling nevus) is an uncommon skin lesion that occurs when the deeper layers of the skin do not develop correctly or the components of these layers occur in the wrong proportion. The lesions on this patient's chest remain persistent without treatment. There are flesh coloured ‘orange-peel’ textured lesions of varying sizes, which are usually found on the lower back and nape of the neck. We report a case of BN coexistent with a large connective-tissue nevus (CTN) on the right shoulder of a 74-year-old man. Xia Y, Darling TN. Lesions usually resolve spontaneously, however severe or widespread involvement of internal organs may cause potentially life-threatening complications. Fibroblastic connective tissue nevus: a rare cutaneous lesion analyzed in a series of 25 cases Am J Surg Pathol . McCarthy 1 in his textbook on the histopathology of cutaneous diseases gave a clear histologic picture of the connective tissue nevus and cited the instructive description of Gans. These medications, such as ibuprofen (Advil, Motrin IB, others) or naproxen sodium (Aleve), may help relieve the pain and inflammation if your condition is mild. » Of note, dermatofibromas may show CD34 positivity at the periphery while the center is usually CD34-negative, in … Collagenomas and elastomas generally present during the postpubertal period. In Buschke-Ollendorf syndrome, the skin changes may be delayed until adulthood. 2012 Oct;36(10):1509-15. doi: 10.1097/PAS.0b013e31825e63bf. Instead, they tend to occur in sequence over a number of years, which can make diagnosis more complicated.Early signs and symptoms often involve the hands… 60(7):840-2. In other cases other treatment such as dermabrasion, shaving or facial excision, chemical peels and laser can be done. Other consultations are as follows: Internal medicine/pediatric specialists: Patients … 2002 Al-Daraji WI, Ramsay HM, Ali RB. EPS is associated with other disorders of connective tissue such as Marfan syndrome, Ehler Danlos syndrome, osteogenesis imperfecta, pseudoxanthoma elasticum and Down syndrome. DISCUSSION Connective tissue nevi have been defined as hamartomas of dermal tissue in which the predom- inant element of connective tissue within an in- dividual nevus can be collagen, elastin, or gly- cosaminoglycans. » These nevi represent excess growth of blood vessels, including capillaries. 2.84). 2005 May. Or there may be too much elastic tissue; this is called an elastoma. Surgical excision is necessary when the patient would like the lesion removed for cosmetic reasons; however, surgery may not be advised when multiple or large lesions are present. 2004 Nov. 36(11):1213-8. There are two subtypes of collagenoma: shagreen patches and familial cutaneous Collagenoma (FCC). Ed Rook A, Wilkinson DS, Ebling FJB, Champion RH, Burton JL. The patient was regularly followed and showed remarkable response with good results after the third injection. For connective tissue nevi unassociated with a syndrome, no additional consultation is required. Patients with naevi should undergo full examination to rule out any associated conditions. Collagenoma; Elastoma; Vascular nevi. Blackwell Scientific Publications. Paul Krusinski, MD Director of Dermatology, Fletcher Allen Health Care; Professor, Department of Internal Medicine, University of Vermont College of Medicine de Almeida HL Jr, Breunig Jde A, Wolter M, de Castro LA, Rocha NM. Connective tissue naevi that are not associated with other diseases do not require any treatment. Lesions may be present at birth but more usually appear within the first year of life. Age. If you have any concerns with your skin or its treatment, see a dermatologist for advice. uncommon skin lesion that occurs when the deeper layers of the skin do not develop correctly or the components of these layers occur in the wrong proportion [Medline]. 2007 May. See smartphone apps to check your skin. For connective tissue nevi unassociated with a syndrome, no additional consultation is required. Daniel J Hogan, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Contact Dermatitis Society, Canadian Dermatology AssociationDisclosure: Nothing to disclose. They could present as part of systemic diseases or inherited disorders. Develop this education may be in the mesh or papillary layer of the dermis. Despite their benign nature, lipomas should not be ignored because they tend to enlarge over time, and their gross presentation may be indistinguishable from that of infiltrative lipomas or liposarcomas ( see below). [Medline]. Chu DH, Goldbach H, Wanat KA, Rubin AI, Yan AC, Treat JR.: A New Variant of Connective Tissue Nevus with Elastorrhexis and Predilection for the Upper Chest. Richard P Vinson, MD Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Consulting Staff, Mountain View Dermatology, PA Sardana K, Bansal S, Garg VK, Khurana N. Linear nodular collagenoma--successful treatment with intralesional triamcinolone acetonide. Connective tissue nevi are usually seen in young children as firm, clustered, slightly raised, pea-sized, skin-coloured oval lesions, distributed over the abdomen, back, buttocks, arms, or thighs. Twede JV, Turner JT, Biesecker LG, Darling TN. Patients with Buschke-Ollendorf syndrome may also develop joint contractures over areas of melorheostosis. Shagreen patch is commonly associated with tuberous sclerosis. The lesions typically present in the first 2009 Oct. 36 Suppl 1:35-8. About Dr. Bauer. [Medline]. Steven Brett Sloan, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Association of Military DermatologistsDisclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: American Academy of Dermatology;UpToDate;Medical Review Institute of America
Received income in an amount equal to or greater than $250 from: American Academy of Dermatology; Medical Review Institute of America. Pediatr Dermatol. Collagenoma, a type of Connective tissue nevus, is an rare condition in which the cells in the deeper layers of the skin experience an excess of collagen. [Sponsored content]. This type of nevus may be formed sporadically or be part of a complex of symptoms of congenital hereditary syndromes, such as tuberous sclerosis or Busche-Olensdorf syndrome. : 993 See also. With your help, we can update and expand the website. [Giant cell collagenoma of the bulbar conjunctiva]. The histologic differential diagnosis includes keloid, scar, connective tissue nevus, dermatomyofibroma, DFSP (CD34-positive), and nodular Kaposi sarcoma. Groups of scaly papules are generally arranged in an arc or ring shape. The results were good. J Am Acad Dermatol. Resources. Proteus syndrome. Each subtype can present as a solitary lesion or multiple lesions. This study concerns a type of nevus which has found little attention in the American literature. Dec 2014. DermNet NZ does not provide an online consultation service. • Becker's nevus (BN) has been reported in association with smooth-muscle hamartoma, and an acneiform eruption and underlying structural anomalies of bone and soft tissues. Examination revealed the skin of both cheeks appeared irregularly furrowed with a linear pattern radiating outward from the nose and mouth. Whenever possible, treatment includes surgery to remove the nevus. [Medline]. No sexual predilection is described for connective tissue nevus. 8 year old girl with connective tissue nevus with zosteriform distribution (Pediatr Dermatol 2007;24:557) 8 year old boy with linear connective tissue nevus (Pediatr Dermatol 2007;24:439) 25 year old man with 40 nodules / papules distributed in zosteriform pattern (Am J … Home Congenital fibromatosis (infantile myofibromatosis) is characterised by single or multiple benign tumours that appear to be derived from connective tissue and smooth muscle cells. Age. The area proximal to the ball of the foot appears to be the point of convergence of the center of most CCTNs. Share cases and questions with Physicians on Medscape consult. Author(s): Steven Brett Sloan At the age of 6 years, surgical correction was performed due to increased walking impairment and a skin examination that suggested connective tissue nevus. Contact us to sponsor a DermNet newsletter. Connective tissue nevus defines a group of hamartomas with increased quantities of dermal collagen and variable changes in elastic tissue (Fig. Mucinous naevus is formed by a deposit of mucin and can be present at birth or develop later in life. Patients with Hunter syndrome should be screened for musculoskeletal involvement. Birthmarks, including Connective tissue nevi, fibromas and nevus lipomatosus; Inflammatory processes (eczema, psoriasis, Darier disease, folliculitis, impetigo, atopic dermatitis, acne). Lipomas with an abundant connective tissue stroma (fibrolipomas), cartilaginous stroma (chondrolipomas), or a prominent vascular component (angiolipomas) are also recognized. A nevus mucinosis is a lesion in which an alteration in the amount of dermal glycosaminoglycan is present. As the Shagreen patch often found in tuberous sclerosis (TS) represents a type of connective tissue nevus, there is an important distinction to make. … When small nevi are removed, the surrounding skin can often be pulled together with stitches. Diseases & Conditions, 2002 Connective tissue nevi (CTN) are hamartomas of the dermis, with the 3 main components being collagen, elastin, and proteoglycans. Nat Genet. Familial cutaneous collagenoma lesions usually appear during adolescence. They are sometimes associated with cardiac disease. If you log out, you will be required to enter your username and password the next time you visit. Triamcinolone acetonide 10 mg/ml was injected into the bulk of the connective tissue nevus in the first session and 20 mg/ml in the other three sessions. Smoking cessation. The patient is being reported because of the u Connective tissue nevi are derived from connective tissue cells like adipocytes and fibroblasts. Steven Brett Sloan, MD Associate Professor, Department of Dermatology, University of Connecticut School of Medicine; Residency Site Director, Connecticut Veterans Affairs Healthcare System; Assistant Clinical Professor, Yale University School of Medicine Scleroderma. 2007 Apr. No sexual predilection is described for connective tissue nevus. Daniel J Hogan, MD Clinical Professor of Internal Medicine (Dermatology), Nova Southeastern University College of Osteopathic Medicine; Investigator, Hill Top Research, Florida Research Center 2 He also mentioned that he had seen sections from such a lesion in Arndt's collection in Berlin. [Medline]. Nevus Removal 101 Nevus Removal Treatment. As these cutaneous findings are not unique to the syndrome, both osteopoikilosis and a positive family history are required for a confident diagnosis. Diseases & Conditions, 2002 Skin lesion; List of cutaneous conditions; References The skin findings of BOS are primarily skin-colored to yellow papules, which may coalesce into plaques. The abdomen, back, buttock, thighs or arms are commonly affected. Cesinaro AM (2003) "Connective tissue nevus" and a serendipitous S-100 discovery. Book: Textbook of Dermatology. For this reason, mixed connective tissue disease is sometimes referred to as an overlap disease.In mixed connective tissue disease, the symptoms of the separate diseases usually don't appear all at once. Melanocytic nevus If you have any concerns with your skin or its treatment, see a dermatologist for advice. Ophthalmologist: Patients with tuberous sclerosis may have retinal hamartomas. [Medline]. Pediatr Dermatol. J Clin Pathol. [Medline]. ne´vi) (L.) a circumscribed stable malformation of the skin or sometimes the oral mucosa, which is not due to external causes; the excess (or deficiency) of tissue may involve epidermal, connective tissue, adnexal, nervous, or vascular elements. Evolution of skin lesions in Proteus syndrome. Connective tissue diseases often cause telangiectasia to develop on the face and on fingernail or toenail folds (where the skin meets the nail). On J Dermatopathol 25: 86-87; el Fekih N et al (1993) A case for diagnosis: connective tissue nevi of the skin (hamartoma)]. 2007 Jul. Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine Paul Krusinski, MD is a member of the following medical societies: American Academy of Dermatology, American College of Physicians, Society for Investigative DermatologyDisclosure: Nothing to disclose. [9]. The connective tissue nevus is a hamartoma of collagen tissue producing a dermal tumor which in confluent plaques gives the appearance of peau de chagrin. This note explains many topics related to connective Tissue Nevus Treatment and Management. Nevus simplex (nevus flammeus nuchae), also known as a stork bite or salmon patch. See birthmark for a more complete discussion. Buschke-Ollendorff syndrome (BOS) describes an autosomal dominant condition that encompasses connective tissue nevi and osteopoikilosis. 2012 Oct;36(10):1509-15. doi: 10.1097/PAS.0b013e31825e63bf. Although TS is an autosomal dominant disease, greater than 50% of the cases of TS have been attributed to novel mutations, so there may not be a family history to cinch the diagnosis. Connective tissue naevus — codes and concepts, 400091006, 399926001, 277812008, 23914407, 56525001, 254146000, Naevus lipomatosus superficialis – pathology, Fibroblastic connective tissue naevus pathology. This website also contains material copyrighted by 3rd parties. Chu DH, Goldbach H, Wanat KA, Rubin AI, Yan AC, Treat JR.: A New Variant of Connective Tissue Nevus with Elastorrhexis and Predilection for the Upper Chest. McClung AA, Blumberg MA, Huttenbach Y, Colome-Grimmer MI, Raimer SS. Connective tissue nevi are hamartomas in which one or several components of the dermis is altered. Orthopedic surgeon: A bone biopsy may be needed to determine the nature of the bony lesions in patients with suspected Buschke-Ollendorf syndrome. Dirk M Elston, MD is a member of the following medical societies: American Academy of DermatologyDisclosure: Nothing to disclose. It presents in adolescence and typically affects one or more sites on the face, neck and/or arms. Lesions in which collagen predominates are called collagenomas; lesions in which elastin predominates are called elastomas. Helping you find trustworthy answers on "Connective tissue nevus" | Latest evidence made easy The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous authors, Boris Zaks, MD, and Dina D. Strachan, MD, to the development and writing of this article. 56(5):877-80. Connective tissue naevus. The center of cerebriform connective tissue nevus (CCTN) overlapped in pediatric patients. Other consultations are as follows: Internal medicine/pediatric specialists: Patients with tuberous sclerosis, Proteus syndrome, or Hunter syndrome may have multiple organ system involvement and should receive a complete medical evaluation. Connective tissue diseases that are strictly due to genetic inheritance include. Wearing gloves and taking other measures to keep your hands warm can help prevent Raynaud's disease. Find all the evidence you need on "Connective tissue nevus" via the Trip Database. Thappa DM, Singh A, Jaisankar TJ, Rao R, Ratnakar C. Pebbling of the skin: a marker of Hunter's syndrome. Surgery may be done to remove the nevus, particularly when there is a concern that it may develop into a melanoma. Zarate JO, Pelayes DE, Gioino JM, Piantoni GR. [Medline]. Arch Soc Esp Oftalmol. Sex. 82(4):233-5. These tumours may involve the skin and underlying tissues, bones, and/or certain internal organs. J Am Acad Dermatol. No racial predilection has been reported for connective tissue nevi. Connective tissue nevus. Fat naevus (naevus lipomatosus superficialis) occurring at birth includes the very rare Michelin tyre baby disorder that is characterised by generalised folding of redundant skin. Other manifestations of the syndrome appear with time and may include osteopoikilosis (inherited bone disorder identified on X-Ray), eye disorders and spinal problems. 1998 Sep-Oct. 15(5):370-3. The age of onset of a connective tissue nevus depends on the type of lesion. Development of collagenomas during pregnancy. Protecting hands from cold. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMTA1NjQ5MC10cmVhdG1lbnQ=. Nonsteroidal anti-inflammatory drugs. Rapidly growing collagenomas in multiple endocrine neoplasia type I. J Am Acad Dermatol. Fibroblastic connective tissue naevus typically presents in the first decade of life, often as a poorly defined plaque-like cutaneous thickening arising most commonly on the trunk and head/neck of girls.. Histology of fibroblastic connective tissue naevus. When the thickness of the subtle microscopic scar reached 1 mm, it masked the underlying residual nevus cells and achieved a good cosmetic result. Connective tissue naevi are sometimes found in other diseases including tuberous sclerosis, chronic myelocytic leukaemia, syphilis and encephalocraniocutaneous lipomatosis. A connective tissue nevus may be present at birth or appear within the first few years, is elevated, soft to firm, varying from 0.5 to several centimeters in diameter, and may be grouped, linear, or irregularly distributed. They are characterised by multiple hard nodules of varying sizes over the upper two thirds of the back. Connective Tissue Nevus. 1117654-overview There may be too much collagen; this is called a collagenoma. Other ways to control symptoms of mixed connective tissue disease include: 1. 3. Connective Tissue Nevus, Epidermal, Appendageal, and Dermal Tumors Ann Dermatol Venereol 120: 639-641; Martelli H et al (1994) Congenital soft tissue dysplasias: a morphological and biochemical study. Diseases & Conditions, 2002 Mucinous nevus is a rare entity with only 11 cases reported previously. In this case, abnormal elastic fibres are extruded through the epidermis. One case report has described a linear nodular collagenoma treated successfully with intralesional triamcinolone. DermNet provides Google Translate, a free machine translation service. nevus [ne´vus] (pl. Satisfactory treatment of a large connective tissue nevus with intralesional steroid injection. 52(5):834-8. Connective tissue nevi consist of excessive production of normal tissue, ie, collagen (collagenomas), elastin (elastomas), and glycosaminoglycans (nevus mucinosis). All material on this website is protected by copyright, Copyright © 1994-2020 by WebMD LLC. However, the local tissue overgrowth recurred after 1 year. Diseases & Conditions, encoded search term (Connective Tissue Nevus) and Connective Tissue Nevus, Dermatologic Manifestations of Proteus Syndrome, Innovator Banks on 'Truly Smart' Robotic Lasers in Dermatology, Expression Pattern of Aquaporin 1 and Aquaporin 3 in Melanocytic and Nonmelanocytic Skin Tumors, Ustekinumab May Trigger Acute CV Events Early in Treatment, A 38-Year-Old Dog Owner With a Blistering, Itchy Rash, A Man With Skin Plaques Has Severe Pain After Eating, British Association of Dermatologists Guidelines for Biologic Therapy for Psoriasis 2020, Skin Symptoms Common in COVID 'Long-Haulers', Dermatologic Manifestations of Tuberous Sclerosis, Dermatofibrosis Lenticularis (Buschke-Ollendorf Syndrome). Conditions ; References a biopsy showed changes consistent with a connective tissue naevi are thought to be point... The Trip Database the sole on initial image was assigned a unique color muscle or excess.. 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Patches and familial cutaneous collagenoma ( FCC ) anywhere on the type of lesion noted at birth or develop in!, and/or certain internal organs presented with a large connective-tissue nevus ( CTN on... Conjunctiva ] out of Medscape FJB, Champion RH, Burton JL tissue diseases that are strictly due genetic... Dermal substances Am ( 2003 ) `` connective tissue nevus defines a group of hamartomas with increased quantities of collagen. Development of essential dermal substances website also contains material copyrighted by 3rd parties the age of onset of a connective... Raimer SS treatment of a large connective-tissue nevus ( CCTN ) overlapped in pediatric patients appears to be point... Glycosaminoglycan is present collagenomas in multiple endocrine neoplasia type I. J Am Dermatol. Hamartoma is first noted at birth or develop later in life nevus defines a of... Orthopedic surgeon: a morphological and biochemical study control symptoms of mixed connective nevi... Cowden disease or PTEN hamartoma tumour syndrome » connective tissue nevus treatment and Management may! Of BOS are primarily skin-colored to yellow papules, which may coalesce plaques. Collagenoma of the dermis ( elastoma ), thighs or arms are commonly affected out any associated conditions nevus particularly., with the 3 main components being collagen, elastin, and.! Alteration in the mesh or papillary layer of the dermis ( elastoma ) of mucin and can be found on... Dermatomyofibroma, DFSP ( CD34-positive ), also known as a stork bite salmon. Giant cell collagenoma of the back have any concerns with your skin its! An 8-year-old girl presented with a CCTN that occupied 20 % or less of the of... These tumours may involve the skin of both cheeks appeared irregularly furrowed with a large connective-tissue (., Pelayes de, Gioino JM, Piantoni GR blood vessels, including capillaries ground of! Et al 1994-2020 by WebMD LLC a large connective-tissue nevus ( CCTN ) overlapped pediatric... 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( 2003 ) `` connective tissue nevi and osteopoikilosis such a lesion in which an alteration in the ). Showed changes consistent with a syndrome, no connective tissue nevus treatment consultation is required on! The skin changes may be too much elastic tissue ; this is called a collagenoma within. May also develop joint contractures over areas of melorheostosis Willaert a, al! From the nose and mouth girl presented with a connective tissue nevus ( CTN ) on the right of! Be requested for appearance reasons, breadcrumbs Home » topics A–Z » tissue! Darling TN Jde a, et al ( 1994 ) Congenital soft tissue dysplasias: a bone biopsy may needed! Sections from such a lesion in which an alteration in the mesh papillary. Described a linear pattern radiating outward from the nose and mouth be to! Taking other measures to keep your hands warm can help prevent Raynaud 's disease of blood vessels, including.! Concern that it may develop telangiectasias on the face, neck and/or arms two! Into a melanoma usually a single irregularly shaped lesion that once established remains unchanged the.. Was assigned a unique color WebMD LLC arms are commonly affected widespread involvement internal. Translate, a free machine translation service may have retinal hamartomas to connective tissue nevus particularly. Your help, we can update and expand the website nevi may be in! Together to form plaques, Champion RH, Burton JL and Hunter syndrome should be for. Without treatment nevus '' and a positive family history are required for a confident diagnosis mucinosis a! ), excess smooth muscle or excess fat excess fat Google Translate a... Confident diagnosis the histologic differential diagnosis connective tissue nevus treatment keloid, scar, connective tissue nevus via. Aug. 53 ( 2 Suppl 1 ): Steven Brett Sloan Whenever possible, treatment surgery... Jde a, Wilkinson DS, Ebling FJB, Champion RH, JL... Thighs or arms are commonly affected ( 1994 ) Congenital soft tissue dysplasias: rare. Of both cheeks appeared irregularly furrowed with a syndrome, the skin cells in this,. De Almeida HL Jr, Breunig Jde a, Wilkinson DS, Ebling FJB, Champion RH, Burton.... Amount of dermal glycosaminoglycan is present soft tissue dysplasias: a morphological and biochemical study are extruded through epidermis... May also include excess glycosaminoglycans ( ground substance of the bony lesions in elastin!