D. A. Sieber et al.

Conclusion

Necrotizing and exfoliative diseases of the skin are commonly encountered by physicians across multiple medical specialties and remain a diagnostic challenge. When encountered, diagnosis needs to be made as rapidly as possible with swift transfer to burn centers for definitive care. Treatment modalities must be based around a multidisciplinary team approach to patient management. With prompt diagnosis, local wound care, and aggressive nutritional support patients have an increased likelihood of meaningful long-term survival and recovery.

References

[1]Howell ER, Phillips CM (2007) Cutaneous manifestations of Staphylococcus aureus disease. Skinmed 6(6): 274–279

[2]Bisno AL (1984) Cutaneous infections: microbiologic and epidemiologic considerations. Am J Med 76(5A): 172–179

[3]Chan HL (1983) Bacterial infections of the skin. I: primary and secondary infections. Ann Acad Med Singapore 12(1): 92–97

[4]Connor MP, Gamelli R (2009) Challenges of cellulitis in a lymphedematous extremity: a case report. Cases J 2: 9377

[5]Fontes RA, Jr, Ogilvie CM, Miclau T (2000) Necrotizing soft-tissue infections. J Am Acad Orthop Surg 8(3): 151– 158

[6]Bang RL, Gang RK, Sanyal SC, Mokaddas EM, Lari AR (1999) Beta-haemolytic Streptococcus infection in burns. Burns 25(3): 242–246

[7]Stevens DL (2009) Treatments for skin and soft-tissue and surgical site infections due to MDR Gram-positive bacteria. J Infect 59 [Suppl [1]: S32–39

[8]Mueller E, Haim M, Petnehazy T, Acham-Roschitz B, Trop M (2010) An innovative local treatment for staphylococcal scalded skin syndrome. Eur J Clin Microbiol Infect Dis 29(7): 893–897

[9]Satyapal S, Mehta J, Dhurat R, Jerajani H, Vaidya M (2002) Staphylococcal scalded skin syndrome. Indian J Pediatr 69(10): 899–901

[10]Simpson C (2003) The management of staphylococcal scalded skin syndrome in infants. Nurs Times 99(42): 59–61

[11]Elias PM, Fritsch P, Epstein EH (1977) Staphylococcal scalded skin syndrome. Clinical features, pathogenesis, and recent microbiological and biochemical developments. Arch Dermatol 113(2): 207–219

[12]Mirabile R, Weiser M, Barot LR, Brown AS (1986) Staphylococcal scalded-skin syndrome. Plast Reconstr Surg 77(5): 752–756

[13]Patel GK (2004) Treatment of staphylococcal scalded skin syndrome. Exp Rev Anti Infect Ther 2(4): 575–587

[14]Johnston GA (2004) Treatment of bullous impetigo and the staphylococcal scalded skin syndrome in infants. Exp Rev Anti Infect Ther 2(3): 439–446

[15]Patel GK, Finlay AY (2003) Staphylococcal scalded skin

syndrome: diagnosis and management. Am J Clin Dermatol 4(3): 165–175

[16] Decleire PY, Blondiaux G, Delaere B, Glupczynski Y (2004) Staphylococcal scalded skin syndrome in an adult. Acta Clin Belg 59(6): 365–368

[17]Oyake S, Oh-i T, Koga M (2001) Staphylococcal scalded skin syndrome in a healthy adult. J Dermatol 28(3): 145–148

[18]Ladhani S (2003) Understanding the mechanism of action of the exfoliative toxins of Staphylococcus aureus. FEMS Immunol Med Microbiol 39(2): 181–189

[19]Hanakawa Y, Schechter NM, Lin C et al (2002) Molecular mechanisms of blister formation in bullous impetigo and staphylococcal scalded skin syndrome. J Clin Invest 110(1): 53–60

[20]Amagai M, Matsuyoshi N, Wang ZH, Andl C, Stanley JR (2000) Toxin in bullous impetigo and staphylococcal scalded-skin syndrome targets desmoglein 1. Nat Med 6(11): 1275–1277

[21]Iwatsuki K, Yamasaki O, Morizane S, Oono T (2006) Staphylococcal cutaneous infections: invasion, evasion and aggression. J Dermatol Sci 42(3): 203–214

[22]Blyth M, Estela C, Young AE (2008) Severe staphylococcal scalded skin syndrome in children. Burns 34(1): 98–103

[23]Hedrick J (2003) Acute bacterial skin infections in pediatric medicine: current issues in presentation and treatment. Paediatr Drugs 5 [Suppl 1]: 35–46

[24]Sharma S, Verma KK (2001) Skin and soft tissue infection. Indian J Pediatr 68 [Suppl 3]: S46–50

[25]Kasperkiewicz M, Schmidt E (2009) Current treatment of autoimmune blistering diseases. Curr Drug Discov Technol 6(4): 270–280

[26]Patricio P, Ferreira C, Gomes MM, Filipe P (2009) Autoimmune bullous dermatoses: a review. Ann N Y Acad Sci 1173: 203–210

[27]Cunha PR, Barraviera SR (2009) Autoimmune bullous dermatoses. An Bras Dermatol 84(2): 111–124

[28]Kabir AK, Kamal M, Choudhury AM (2008) Clinicopathological correlation of blistering diseases of skin. Bangladesh Med Res Counc Bull 34(2): 48–53

[29]Olasz EB, Yancey KB (2008) Bullous pemphigoid and related subepidermal autoimmune blistering diseases. Curr Dir Autoimmun 10: 141–166

[30]Lessey E, Li N, Diaz L, Liu Z (2008) Complement and cutaneous autoimmune blistering diseases. Immunol Res 41(3): 223–232

[31]McCuin JB, Hanlon T, Mutasim DF (2006) Autoimmune bullous diseases: diagnosis and management. Dermatol Nurs 18(1): 20–25

474

Necrotizing and exfoliative diseases of the skin

[32]Xu L, Chen M, Peng J et al (1998) Molecular cloning and characterization of a cDNA encoding canine type VII collagen non-collagenous (NC1) domain, the target antigen of autoimmune disease epidermolysis bullosa acquisita (EBA). Biochim Biophys Acta 1408(1): 25–34

[33]Woodley DT, Gammon WR (1989) Epidermolysis bullosa acquista. Immunol Ser 46: 547–563

[34]Puvabanditsin S, Garrow E, Samransamraujkit R, Lopez LA, Lambert WC (1997) Epidermolysis bullosa associated with congenital localized absence of skin, fetal abdominal mass, and pyloric atresia. Pediatr Dermatol 14(5): 359–362

[35]Horvath B, Niedermeier A, Podstawa E et al (2010) IgA autoantibodies in the pemphigoids and linear IgA bullous dermatosis. Exp Dermatol 19(7): 648–653

[36]Kharfi M, Khaled A, Karaa A et al (2010) Linear IgA bullous dermatosis: the more frequent bullous dermatosis of children. Dermatol Online J 16(1): 2

[37]Colombo M, Volpini S, Orini S et al (2008) [Linear IgA bullous dermatosis: the importance of a correct differential diagnosis]. Minerva Pediatr 60(3): 351–353

[38]Akin MA, Gunes T, Akyn L et al (2009) A newborn with bullous pemphigoid associated with linear IgA bullous dermatosis. Acta Dermatovenerol Alp Panonica Adriat 18(2): 66–70

[39]Onodera H, Mihm MC, Jr, Yoshida A, Akasaka T (2005) Drug-induced linear IgA bullous dermatosis. J Dermatol 32(9): 759–764

[40]Khan I, Hughes R, Curran S, Marren P (2009) Drug-as- sociated linear IgA disease mimicking toxic epidermal necrolysis. Clin Exp Dermatol 34(6): 715–717

[41]Navi D, Michael DJ, Fazel N (2006) Drug-induced linear IgA bullous dermatosis. Dermatol Online J 12(5): 12

[42]Knudson RM, Kalaaji AN, Bruce AJ (2010) The management of mucous membrane pemphigoid and pemphigus. Dermatol Ther 23(3): 268–280

[43]Mahoney MG, Wang Z, Rothenberger K et al (1999) Explanations for the clinical and microscopic localization of lesions in pemphigus foliaceus and vulgaris. J Clin Invest 103(4): 461–468

[44]Mazzi G, Raineri A, Zanolli FA et al (2003) Plasmapheresis therapy in pemphigus vulgaris and bullous pemphigoid. Transfus Apher Sci 28(1): 13–18

[45]Bickle K, Roark TR, Hsu S (2002) Autoimmune bullous dermatoses: a review. Am Fam Physician 65(9): 1861– 1870

[46]Canoso JJ, Barza M (1993) Soft tissue infections. Rheum Dis Clin North Am 19(2): 293–309

[47]Ryssel H, Germann G, Kloeters O et al (2010) Necrotizing fasciitis of the extremities: 34 cases at a single centre over the past 5 years. Arch Orthop Trauma Surg 130(12): 1515–1522

[48]Patino JF, Castro D (1991) Necrotizing lesions of soft tissues: a review. World J Surg 15(2): 235–239

[49]Light TD, Choi KC, Thomsen TA et al (2010) Long-term outcomes of patients with necrotizing fasciitis. J Burn Care Res 31(1): 93–99

[50]Childers BJ, Potyondy LD, Nachreiner R et al (2002) Necrotizing fasciitis: a fourteen-year retrospective study of 163 consecutive patients. Am Surg 68(2): 109–116

[51]McGee EJ (2005) Necrotizing fasciitis: review of pathophysiology, diagnosis, and treatment. Crit Care Nurs Q 28(1): 80–84

[52]Edlich RF, Winters KL, Woodard CR, Britt LD, Long WB, 3rd (2005) Massive soft tissue infections: necrotizing fasciitis and purpura fulminans. J Long Term Eff Med Implants 15(1): 57–65

[53]Seal DV, Kingston D (1988) Streptococcal necrotizing fasciitis: development of an animal model to study its pathogenesis. Br J Exp Pathol 69(6): 813–831

[54]Wall DB, Klein SR, Black S, de Virgilio C (2000) A simple model to help distinguish necrotizing fasciitis from nonnecrotizing soft tissue infection. J Am Coll Surg 191(3): 227–231

[55]Endorf FW, Klein MB, Mack CD, Jurkovich GJ, Rivara FP (2008) Necrotizing soft-tissue infections: differences in patients treated at burn centers and non-burn centers. J Burn Care Res 29(6): 933–938

[56]Redman DP, Friedman B, Law E, Still JM (2003) Experience with necrotizing fasciitis at a burn care center. South Med J 96(9): 868–870

[57]Barillo DJ, McManus AT, Cancio LC, Sofer A, Goodwin CW (2003) Burn center management of necrotizing fasciitis. J Burn Care Rehabil 24(3): 127–132

[58]Endorf FW, Supple KG, Gamelli RL (2005) The evolving characteristics and care of necrotizing soft-tissue infections. Burns 31(3): 269–273

[59]Faucher LD, Morris SE, Edelman LS, Saffle JR (2001) Burn center management of necrotizing soft-tissue surgical infections in unburned patients. Am J Surg 182(6): 563–569

[60]Steinstraesser L, Sand M, Steinau HU (2009) Giant VAC in a patient with extensive necrotizing fasciitis. Int J Low Extrem Wounds 8(1): 28–30

[61]Hassan Z, Mullins RF, Friedman BC et al (2010) Treating necrotizing fasciitis with or without hyperbaric oxygen therapy. Undersea Hyperb Med 37(2): 115–123

[62]Graves C, Saffle J, Morris S, Stauffer T, Edelman L (2005) Caloric requirements in patients with necrotizing fasciitis. Burns 31(1): 55–59

[63]Saffle JR, Morris SE, Edelman L (2008) Fournier’s gangrene: management at a regional burn center. J Burn Care Res 29(1): 196–203

[64]Jeong HJ, Park SC, Seo IY, Rim JS (2005) Prognostic factors in Fournier gangrene. Int J Urol 12(12): 1041–1044

[65]Sada A, Misago N, Okawa T et al (2009) Necrotizing fasciitis and myonecrosis “synergistic necrotizing cellulitis” caused by Bacillus cereus. J Dermatol 36(7): 423–426

[66]Rozmaryn LM (1995) Synergistic necrotizing cellulitis in the hand of a renal dialysis patient: a case report. J Hand Surg Am 20(3): 500–501

[67]Stone HH, Martin JD, Jr (1972) Synergistic necrotizing cellulitis. Ann Surg 175(5): 702–711

475

D. A. Sieber et al.

[68]Adcock DM, Hicks MJ (1990) Dermatopathology of skin necrosis associated with purpura fulminans. Semin Thromb Hemost 16(4): 283–292

[69]Warner PM, Kagan RJ, Yakuboff KP et al (2003) Current management of purpura fulminans: a multicenter study. J Burn Care Rehabil 24(3): 119–126

[70]Chu DZ, Blaisdell FW (1982) Purpura fulminans. Am J Surg 143(3): 356–362

[71]Chasan PE, Hansbrough JF, Cooper ML (1992) Management of cutaneous manifestations of extensive purpura fulminans in a burn unit. J Burn Care Rehabil 13(4): 410–413

[72]Zenciroglu A, Ipek MS, Aydin M et al (2010) Purpura fulminans in a newborn infant with galactosemia. Eur J Pediatr 169(7): 903–906

[73]Demirel N, Bas AY, Okumus N, Zenciroglu A, Yarali N (2009) Severe purpura fulminans due to coexistence of homozygous protein C deficiency and homozygous methylenetetrahydrofolate reductase mutation. Pediatr Hematol Oncol 26(8): 597–600

[74]Lalitha AV, Aruna D, Prakash A, Nanjunda Swamy HM, Subba Rao SD (2009) Spectrum of purpura fulminans. Indian J Pediatr 76(1): 87–89

[75]Wharton SM, Reid CA (1998) Purpura fulminans localising to a recent burn injury. Burns 24(7): 680–682

[76]Brown DL, Greenhalgh DG, Warden GD (1998) Purpura fulminans: a disease best managed in a burn center. J Burn Care Rehabil 19(2): 119–123

[77]Gast T, Kowal-Vern A, An G, Hanumadass ML (2006) Purpura fulminans in an adult patient with Haemophilus influenzae sepsis: case report and review of the literature. J Burn Care Res 27(1): 102–107

[78]Jordan K, Kristensen K (2010) [Purpura fulminans]. Ugeskr Laeger 172(28): 2064–2065

[79]Kubo K, Chishiro T, Okamoto H, Matsushima S (2009) [Purpura fulminans (symmetric peripheral gangrene): 7-year consecutive case review in Japan]. Kansenshogaku Zasshi 83(6): 639–646

[80]Sheridan RL, Briggs SE, Remensnyder JP et al (1995) The burn unit as a resource for the management of acute nonburn conditions in children. J Burn Care Rehabil 16(1): 62–64

[81]Bichet JC, Mojallal A, Delay E, Ziad S, Foyatier JL (2003) [Surgical management of cutaneous necrosis in the purpura fulminans: report of 2 clinical cases]. Ann Chir Plast Esthet 48(4): 216–221

[82]Minhas KM, Bashir S, Sarwari AR, Parker J (2008) Pneumococcal purpura fulminans successfully treated with activated protein C. South Med J 101(10): 1046–1048

[83]Hassan Z, Mullins RF, Friedman BC et al (2008) Purpura fulminans: a case series managed at a regional burn center. J Burn Care Res 29(2): 411–415

[84]Arevalo JM, Lorente JA, Fonseca R (1998) Surgical treatment of extensive skin necrosis secondary to purpura fulminans in a patient with meningococcal sepsis. Burns 24(3): 272–274

[85]Lowery K, Shirley R, Shelley OP et al (2008) Purpura fulminans skin loss: surgical management protocols at a regional burns centre. J Plast Reconstr Aesthet Surg 61(12): 1520–1523

[86]Dalli RL, Kumar R, Kennedy P et al (2007) Toxic epidermal necrolysis/Stevens-Johnson syndrome: current trends in management. ANZ J Surg 77(8): 671–676

[87]Baby S, Doris S (1999) The Steven Johnson syndrome. A case study. Nurs J India 90(7): 149–150

[88]Wolkenstein P, Charue D, Laurent P et al (1995) Metabolic predisposition to cutaneous adverse drug reactions. Role in toxic epidermal necrolysis caused by sulfonamides and anticonvulsants. Arch Dermatol 131(5): 544–551

[89]Lissia M, Mulas P, Bulla A, Rubino C (2010) Toxic epidermal necrolysis (Lyell’s disease). Burns 36(2): 152–163

[90]Gravante G, Delogu D, Marianetti M et al (2007) Toxic epidermal necrolysis and Steven Johnson syndrome: 11-years experience and outcome. Eur Rev Med Pharmacol Sci 11(2): 119–127

[91]Crosby SS, Murray KM, Marvin JA, Heimbach DM, Tartaglione TA (1986) Management of Stevens-Johnson syndrome. Clin Pharm 5(8): 682–689

[92] Auquier-Dunant A, Mockenhaupt M, Naldi L et al (2002) Correlations between clinical patterns and causes of erythema multiforme majus, Stevens-John- son syndrome, and toxic epidermal necrolysis: results of an international prospective study. Arch Dermatol 138(8): 1019–1024

[93]Dolan P, Flowers F, Araujo O et al (1989) Toxic epidermal necrolysis. J Emerg Med (7): 65–69

[94]Lyell A (1956) Toxic epidermal necrolysis: an eruption resembling scalding of the skin. Br J Dermatol (68): 355–361

[95]Arnold HJ OR, James WD (1990) Contact dermatitis: drug eruptions. In: Andrew’s diseases of the skin: Clinical dermatology. WB Saunders, Philadelphia, pp 128–130

[96]Roujeau JC (1997) Stevens-Johnson syndrome and toxic epidermal necrolysis are severity variants of the same disease which differs from erythema multiforme. J Dermatol 24(11): 726–729

[97]Roujeau J, Guillaume J, Fabre J et al (1990) Toxic epider-

mal necrolysis (Lyell Syndrome): incidence and drugetiology in France, 1981–1985. Arch Dermatol

126: 37–42

[98]Guillaume JC, Roujeau JC, Revuz J, Penso D, Touraine R (1987) The culprit drugs in 87 cases of toxic epidermal necrolysis (Lyell’s syndrome). Arch Dermatol 123(9): 1166–1170

[99]Hawk RJ, Storer JS, Daum RS (1985) Toxic epidermal necrolysis in a 6-week-old infant. Pediatr Dermatol 2(3): 197–200

[100]Roujeau JC, Kelly JP, Naldi L et al (1995) Medication use and the risk of Stevens-Johnson syndrome or toxic epidermal necrolysis. N Engl J Med 333(24): 1600–1607

476

Necrotizing and exfoliative diseases of the skin

[101]Rzany B, Mockenhaupt M, Stocker U, Hamouda O, Schopf E (1993) Incidence of Stevens-Johnson syndrome and toxic epidermal necrolysis in patients with the acquired immunodeficiency syndrome in Germany. Arch Dermatol 129(8): 1059

[102]Fritsch P, Sidoroff A (2000) Drug-induced StevensJohnson syndrom/toxic epidermal necrolysis. Am J Clin Dermatol (1): 349–360

[103]Kaufmann D (1991) Epidemiologic approaches to the study of toxic epidermal necrolysis. J Invest Dermatol (102): 31S-33S

[104]Schopf E, Stuhmer A, Rzany B et al (1991) Toxic epidermal necrolysis and Stevens-Johnson syndrome. An epidemiologic study from West Germany. Arch Dermatol 127(6): 839–842

[105]Shear NH, Spielberg SP, Grant DM, Tang BK, Kalow W (1986) Differences in metabolism of sulfonamides predisposing to idiosyncratic toxicity. Ann Intern Med 105(2): 179–184

[106]Ruiz-Maldonado R (1985) Acute disseminated epidermal necrosis types 1,2 and 3: a study of sixty cases. J Am Acad Dermatol 13: 623–635

[107]Becker D (1998) Toxic epidermal necrolysis. Lancet 351: 1417–1419

[108]Oplatek A, Brown K, Sen S et al (2006) Long-term fol- low-up of patients treated for toxic epidermal necrolysis. J Burn Care Res 27(1): 26–33

[109]Paquet P, Pierard GE (1997) Erythema multiforme and toxic epidermal necrolysis: a comparative study. Am J Dermatopathol 19(2): 127–132

[110]Bastuji-Garin S, Fouchard N, Bertocchi M et al (2000) SCORTEN: a severity-of-illness score for toxic epidermal necrolysis. J Invest Dermatol 115(2): 149–153

[111]Palmieri TL, Greenhalgh DG, Saffle JR et al (2002) A multicenter review of toxic epidermal necrolysis treated in U. S. burn centers at the end of the twentieth century. J Burn Care Rehabil 23(2): 87–96

[112]Garcia-Doval I, LeCleach L, Bocquet H, Otero XL, Roujeau JC (2000) Toxic epidermal necrolysis and StevensJohnson syndrome: does early withdrawal of causative drugs decrease the risk of death? Arch Dermatol 136(3): 323–327

[113]Demling RH, Ellerbe S, Lowe NJ (1978) Burn unit management of toxic epidermal necrolysis. Arch Surg 113(6): 758–759

[114]Herndon DN (1995) Toxic epidermal necrolysis: a systemic and dermatologic disorder best treated with standard treatment protocols in burn intensive care

units without the prolonged use of corticosteroids. J Am Coll Surg 180(3): 340–342

[115]Kelemen JJ, 3rd, Cioffi WG, McManus WF, Mason AD, Jr, Pruitt BA, Jr (1995) Burn center care for patients with toxic epidermal necrolysis. J Am Coll Surg 180(3): 273–278

[116]Wolkenstein P, Latarjet J, Roujeau JC et al (1998) Randomised comparison of thalidomide versus placebo in toxic epidermal necrolysis. Lancet 352 (9140): 1586–1589

[117]Viard I, Wehrli P, Bullani R et al (1998) Inhibition of toxic epidermal necrolysis by blockade of CD95 with human intravenous immunoglobulin. Science 282 (5388): 490–493

[118]Prins C, Kerdel FA, Padilla RS et al (2003) Treatment of toxic epidermal necrolysis with high-dose intravenous immunoglobulins: multicenter retrospective analysis of 48 consecutive cases. Arch Dermatol 139(1): 26–32

[119]Stella M, Cassano P, Bollero D, Clemente A, Giorio G (2001) Toxic epidermal necrolysis treated with intravenous high-dose immunoglobulins: our experience. Dermatology 203(1): 45–49

[120]Trent JT, Kirsner RS, Romanelli P, Kerdel FA (2003) Analysis of intravenous immunoglobulin for the treatment of toxic epidermal necrolysis using SCORTEN: The University of Miami Experience. Arch Dermatol 139(1): 39–43

[121]Tristani-Firouzi P, Petersen MJ, Saffle JR, Morris SE, Zone JJ (2002) Treatment of toxic epidermal necrolysis with intravenous immunoglobulin in children. J Am Acad Dermatol 47(4): 548–552

[122]Bachot N, Revuz J, Roujeau JC (2003) Intravenous immunoglobulin treatment for Stevens-Johnson syndrome and toxic epidermal necrolysis: a prospective noncomparative study showing no benefit on mortality or progression. Arch Dermatol 139(1): 33–36

[123]Brown KM, Silver GM, Halerz M et al (2004) Toxic epidermal necrolysis: does immunoglobulin make a difference? J Burn Care Rehabil 25(1): 81–88

[124]Shortt R, Gomez M, Mittman N, Cartotto R (2004) Intravenous immunoglobulin does not improve outcome in toxic epidermal necrolysis. J Burn Care Rehabil 25(3): 246–255

Correspondence: Richard L. Gamelli, M.D. FACS, Stritch School of Medicine – 420 Loyola University Medical Center 2160 South First Avenue Maywood, IL 60153, USA, E-mail: [e-mail]

477