• Scleroderma
• Scleroderma references

Scleroderma

Scleroderma is an autoimmune disease. In the early stages of scleroderma in the skin inflammatory cells can be seen in the dermis. However, this inflammation gradually diminishes over time as the skin becomes increasingly abnormal. Scleroderma involves a gradual hardening and tightening of the skin due to excessive collagen production. The excessive collagen stops the normal functioning of hair follicles in a way that might be described as "suffocation" or "strangulation". Although there is a lot of collagen in the skin and there is atrophy of affected hair follicles, there is very little actual scar tissue formation.

Excess collagen production occurs in patches and consequently hair loss also develops in distinct areas. The first symptoms of localized skin scleroderma in hair bearing areas may involve a premature graying of the hair shortly followed by hair loss. Hair loss may be progressive with patches gradually expanding in size over several years. When hair loss occurs on the scalp in a linear fashion it is given the description "en coup de sabre". It was apparently given this name because the strip of hair loss resembles the scar of a sabre cut. Treatment of en coup de sabre involves surgery to remove the affected area of skin.

There are several different classifications of scleroderma. The distinctions between them can be hazy but, in general, scleroderma is defined as either:

• Localized scleroderma limited to small regions of the skin. This form is most likely to induce patchy hair loss.
  
• “CREST” which is a more extensive form of scleroderma. The acronym “CREST” stands for: Calcinosis (calcium deposits in soft tissue), Raynaud's phenomenon (hypersensitivity of the digits to cold), Esophageal involvement (difficulty swallowing), Sclerodactyly (skin hardening on fingers), Telangiectasis (dilation of blood vessels around the mouth). CREST is a fairly benign form of scleroderma but very occasionally heart failure can occur.
  
• “PSS” - progressive systemic scleroderma.

PSS - progressive systemic sclerosis is the most serious form of scleroderma. As well as affecting the skin, often showing the phenomena seen in CREST, there is also adverse activity in other parts of the body. The joints, gut, lungs, kidneys, nerves and muscles including the heart may be affected. PSS is the result of a continued fibrosis in any or all these organs.

Lymphocyte cells from many patients with scleroderma produce cytokines - chemicals which stimulate fibroblast cells and promote collagen production. Overproduction of collagen results in fibrosis in the organs. In the heart fibrosis can lead to rhythm disturbances and heart failure. Scleroderma is a disease that develops spontaneously but it may be induced in people who work with silica, vinyl-chloride, after silicone implants or after injection of certain drugs. Bone marrow transplant recipients and some people who contract Hepatitis C can contract a similar condition. Onset is up to three time more common in women. It is believed that at least some of those affected are genetically susceptible to the condition.

Scleroderma references

• Patrizi A, Marzaduri S, Marini R. A familial case of scleroderma en coup de sabre. Acta Derm Venereol. 2000 May;80(3):237.
• Rai R, Handa S, Gupta S, Kumar B. Bilateral en coup de sabre-a rare entity. Pediatr Dermatol. 2000 May-Jun;17(3):222-4.
• Higashi Y, Kanekura T, Fukumaru K, Kanzaki T. Scleroderma en coup de sabre with central nervous system involvement. J Dermatol. 2000 Jul;27(7):486-8.
• Eguchi T, Harii K, Sugawara Y. Repair of a large "coup de sabre" with soft-tissue expansion and artificial bone graft. Ann Plast Surg. 1999 Feb;42(2):207-10.
• Blaszczyk M, Jablonska S. Linear scleroderma en Coup de Sabre. Relationship with progressive facial hemiatrophy (PFH). Adv Exp Med Biol. 1999;455:101-4.
• McKenna DB, Benton EC. A tri-linear pattern of scleroderma 'en coup de sabre' following Blaschko's lines. Clin Exp Dermatol. 1999 Nov;24(6):467-8.
• Itin PH, Schiller P. Double-lined frontoparietal scleroderma en coup de sabre. Dermatology. 1999;199(2):185-6.
• Soma Y, Fujimoto M. Frontoparietal scleroderma (en coup de sabre) following Blaschko's lines. J Am Acad Dermatol. 1998 Feb;38(2 Pt 2):366-8.
• Jackson JM, Callen JP. Scarring alopecia and sclerodermatous changes of the scalp in a patient with hepatitis C infection. J Am Acad Dermatol. 1998 Nov;39(5 Pt 2):824-6.
• Field T, Bridges AJ. Clinical and laboratory features of patients with scleroderma and silicone implants. Curr Top Microbiol Immunol. 1996;210:283-90.
• Sengezer M, Deveci M, Selmanpakoglu N. Repair of "coup de sabre," a linear form of scleroderma. Ann Plast Surg. 1996 Oct;37(4):428-32.
• Battafarano DF, Zimmerman GC, Older SA, Keeling JH, Burris HA. Docetaxel (Taxotere) associated scleroderma-like changes of the lower extremities. A report of three cases. Cancer. 1995 Jul 1;76(1):110-5.
• Ostertag JU, Hulsmans RF, Neumann HA. [Bilateral linear temporoparietal scleroderma en coup de sabre]. Hautarzt. 1994 Jun;45(6):398-401.
• Kahari VM. Activation of dermal connective tissue in scleroderma. Ann Med. 1993 Dec;25(6):511-8.
• Nakazawa A, Matsuo I, Ohkido M. Progressive facial hemiatrophy with localized scleroderma. Tokai J Exp Clin Med. 1992 Jul;17(2):85-7.
• Lehman TJ. The Parry Romberg syndrome of progressive facial hemiatrophy and linear scleroderma en coup de sabre. Mistaken diagnosis or overlapping conditions? J Rheumatol. 1992 Jun;19(6):844-5.
• Nakahara M, Harii K, Yamada A, Ueda K, Kajikawa A. Repair of 'en coup de sabre' with tissue expander. Br J Plast Surg. 1991 Nov-Dec;44(8):575-8.
• David J, Wilson J, Woo P. Scleroderma 'en coup de sabre'. Ann Rheum Dis. 1991 Apr;50(4):260-2.
• Goldenstein-Schainberg C, Pereira RM, Gusukuma MC, Messina WC, Cossermelli W. Childhood linear scleroderma "en coup de sabre" with uveitis. J Pediatr. 1990 Oct;117(4):581-4.
• Hulsmans RF, Asghar SS, Siddiqui AH, Cormane RH. Hereditary deficiency of C2 in association with linear scleroderma 'en coup de sabre'. Arch Dermatol. 1986 Jan;122(1):76-9.
• Serup J, Serup L, Sjo O. Localized scleroderma 'en coup de sabre' with external eye muscle involvement at the same line. Clin Exp Dermatol. 1984 Mar;9(2):196-200.
• Milan MF, Bennett JE. Scleroderma en coup de sabre. Ann Plast Surg. 1983 May;10(5):364-70.
• Long PR, Miller OF 3d. Linear scleroderma. Report of a case presenting as persistent unilateral eyelid edema. J Am Acad Dermatol. 1982 Oct;7(4):541-4.
• Thompson DM, Robinson TW, Lennard-Jones J. Alopecia areata, vitiligo, scleroderma and ulcerative colitis. Proc R Soc Med. 1974 Oct;67(10):1010-2.
• Hazen PG, Askari A. Localized scleroderma with cutaneous calcinosis. A distinctive variant. Arch Dermatol. 1979 Jul;115(7):871-2.
• Brenner W, Diem E, Gschnait F. Coincidence of vitiligo, alopecia areata, onychodystrophy, localized scleroderma and lichen planus. Dermatologica. 1979;159(4):356-60.
• Thompson DM, Robinson TW, Lennard-Jones J. Alopecia areata, vitiligo, scleroderma and ulcerative colitis. Proc R Soc Med. 1974 Oct;67(10):1010-2.
• Zamick P, Weiss RM. Repair of coup de sabre, a linear form of scleroderma. Case report. Plast Reconstr Surg. 1972 Nov;50(5):520-2.
• Moldenhauer E. [Surgical treatment of coup de sabre scleroderma]. Dermatol Monatsschr. 1969;155(12):973-6.
• Dilley JJ, Perry HO. Bilateral linear scleroderma en coup de sabre. Arch Dermatol. 1968 Jun;97(6):688-9.
• Chamberlain JL 3d, Bard JW. Localized scleroderma - en coup de sabre. South Med J. 1968 Feb;61(2):206.
• Grupper C. [Pseudopeladic state and generalised scleroderma]. Bull Soc Fr Dermatol Syphiligr. 1965 May-Jun;72(3):236-7.