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Sclerema neonatorum

April 10, 2024

Sclerema neonatorum is a rare and severe skin condition that is characterized by diffuse hardening of the subcutaneous tissue with minimal inflammation.[1][2]

Sclerema neonatorum
SpecialtyPediatrics 

Sclerema neonatorum is categorized as a kind of panniculitis that appears as subcutaneous adipose tissue and skin hardening. The hardened skin and subcutaneous fat stick to the underlying bone and muscle so much that it makes it difficult to breathe and eat, and it usually results in death.[3]

Severe respiratory or gastrointestinal disorders, congenital malformations, dehydration, and sepsis are among the comorbid conditions that affect affected infants.[4] Sclerema neonatorum usually has a very bad prognosis and a high death rate.[5]

Signs and symptoms edit

Sclerema neonatorum causes tight, adherent, and waxy-looking skin that is affected by underlying tissues. Some babies may have mottled or purple skin. It is not possible to elevate, pinch, or depress the skin. Traditionally, Sclerema neonatorum appears symmetrically on the trunk, thighs, or buttocks. However, the skin hardening rapidly spreads to the entire body's subcutaneous fat, excluding the parts of the body that are fat-free, such as the palms, soles, and genitalia. A fixed face, resembling a mask, could be the result of the facial skin becoming harder.[4]

Causes edit

sclerema neonatorum's pathogenesis is still unknown. When comparing the subcutaneous fat composition of older people with that of neonates, it is evident that the former has a higher concentration of saturated fats. Neonatal fat has a unique biochemical characteristic that increases its propensity to solidify in a cold environment.[4] It has been proposed that subcutaneous adipose hardening in sclerema neonatorum is initiated by lowered body temperatures experienced during clinical shock.[6] This theory is refuted by the fact that fat hardening shouldn't happen until skin temperature falls below freezing.[4] Other theories suggest that sclerema neonatorum develops as a result of dysregulated fat metabolism, stems from adipocyte-peripheral connective tissue dysfunction, or is a secondary effect of systemic toxicity.[7][8][9]

Diagnosis edit

sclerema neonatorum is typically diagnosed clinically when a critically sick newborn exhibits diffuse skin hardening. The afflicted skin cannot be pitted, folded, or pinched because it is attached to the underlying tissue. For histopathologic confirmation, a skin biopsy could be helpful if the diagnosis is uncertain.[4]

Necrosis of subcutaneous fat without a significant inflammatory infiltrate and without obvious granulomatous changes, the formation of needle-shaped clefts in adipocytes, sometimes in a radial arrangement, and fibrous thickening of the tissue surrounding fat lobules are histopathologic findings that support a diagnosis of sclerema neonatorum.[10][11]

Outlook edit

sclerema neonatorum is linked to a high death rate because it worsens breathing and other essential functions in critically ill newborns.[4] Based on case series, the survival rate of affected neonates is estimated to be between 13 and 39 percent.[3] There are usually no long-term skin problems among survivors.[11]

Epidemiology edit

Usually affecting newborns, Sclerema neonatorum manifests itself during the first week of life, though some cases have been documented to occur outside of this time frame. Based on the compilation of case reports, it appears that men may experience the condition slightly more frequently than women (male to female ratio: 1.6:1).[3]

See also edit

References edit

  1. ^ William D. James, Timothy G. Berger; et al. (2006). Andrews' Diseases of the Skin: clinical Dermatology. Saunders Elsevier. p. 490. ISBN 978-0-7216-2921-6.
  2. ^ Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. pp. 1515, 1524, 1525. ISBN 978-1-4160-2999-1.
  3. ^ a b c Zeb, A; Darmstadt, G L (March 27, 2008). "Sclerema neonatorum: a review of nomenclature, clinical presentation, histological features, differential diagnoses and management". Journal of Perinatology. 28 (7). Springer Science and Business Media LLC: 453–460. doi:10.1038/jp.2008.33. ISSN 0743-8346. PMID 18368059. S2CID 205177400.
  4. ^ a b c d e f "UpToDate". UpToDate. Retrieved February 14, 2024.
  5. ^ Park, Seh Hyun; Kim, Soo-Chan (2017). "Sclerema Neonatorum in a Full-Term Infant Showing Favorable Prognosis". Annals of Dermatology. 29 (6). Korean Dermatological Association and The Korean Society for Investigative Dermatology: 790–793. doi:10.5021/ad.2017.29.6.790. ISSN 1013-9087. PMC 5705365. PMID 29200772.
  6. ^ Hughes, Wilson E.; Hammond, Morton L. (1948). "Sclerema neonatorum". The Journal of Pediatrics. 32 (6). Elsevier BV: 676–692. doi:10.1016/s0022-3476(48)80224-6. ISSN 0022-3476.
  7. ^ Kellum, Robert E. (April 1, 1968). "Sclerema Neonatorum". Archives of Dermatology. 97 (4). American Medical Association (AMA): 372. doi:10.1001/archderm.1968.01610100012002. ISSN 0003-987X.
  8. ^ Warwick, Warren J. (June 1, 1963). "Sclerema Neonatorum— A Sign, Not a Disease". JAMA: The Journal of the American Medical Association. 184 (9). American Medical Association (AMA): 680–683. doi:10.1001/jama.1963.03700220056007. ISSN 0098-7484. PMID 13999025.
  9. ^ Villacorte, G; Frank, D J (January 1967). "Sclerema neonatorum. A report of nine cases". The Ohio State Medical Journal. 63 (1): 57–59. PMID 6037143.
  10. ^ Polcari, Ingrid C.; Stein, Sarah L. (July 28, 2010). "Panniculitis in childhood". Dermatologic Therapy. 23 (4). Hindawi Limited: 356–367. doi:10.1111/j.1529-8019.2010.01336.x. ISSN 1396-0296. PMID 20666823.
  11. ^ a b Requena, Luis; Yus, Evaristo Sánchez (2001). "Panniculitis. Part I. Mostly septal panniculitis". Journal of the American Academy of Dermatology. 45 (2). Elsevier BV: 163–186. doi:10.1067/mjd.2001.114736. ISSN 0190-9622. PMID 11464178.

Further reading edit

  • Shrestha, Sandeep; Chaudhary, Nagendra; Koirala, Sujit; Gupta, Ruchi (2017). "Sclerema Neonatorum Treated Successfully with Parenteral Steroids: An Experience from a Resource Poor Country". Case Reports in Pediatrics. 2017. Hindawi Limited: 1–4. doi:10.1155/2017/4836142. ISSN 2090-6803. PMC 5444035. PMID 28584673.
  • Zeb, Amna; Rosenberg, Rebecca E.; Ahmed, ASM Nawshad Uddin; Saha, Samir K.; Chowdhury, MAK Azad; Ahmed, Saifuddin; Darmstadt, Gary L. (2009). "Risk Factors for Sclerema Neonatorum in Preterm Neonates in Bangladesh". Pediatric Infectious Disease Journal. 28 (5). Ovid Technologies (Wolters Kluwer Health): 435–438. doi:10.1097/inf.0b013e3181951af4. ISSN 0891-3668. PMID 19319014.

External links edit

  • DermNet
  • VisualDx

April 10, 2024
sclerema, neonatorum, rare, severe, skin, condition, that, characterized, diffuse, hardening, subcutaneous, tissue, with, minimal, inflammation, specialtypediatrics, categorized, kind, panniculitis, that, appears, subcutaneous, adipose, tissue, skin, hardening. Sclerema neonatorum is a rare and severe skin condition that is characterized by diffuse hardening of the subcutaneous tissue with minimal inflammation 1 2 Sclerema neonatorumSpecialtyPediatrics Sclerema neonatorum is categorized as a kind of panniculitis that appears as subcutaneous adipose tissue and skin hardening The hardened skin and subcutaneous fat stick to the underlying bone and muscle so much that it makes it difficult to breathe and eat and it usually results in death 3 Severe respiratory or gastrointestinal disorders congenital malformations dehydration and sepsis are among the comorbid conditions that affect affected infants 4 Sclerema neonatorum usually has a very bad prognosis and a high death rate 5 Contents 1 Signs and symptoms 2 Causes 3 Diagnosis 4 Outlook 5 Epidemiology 6 See also 7 References 8 Further reading 9 External linksSigns and symptoms editSclerema neonatorum causes tight adherent and waxy looking skin that is affected by underlying tissues Some babies may have mottled or purple skin It is not possible to elevate pinch or depress the skin Traditionally Sclerema neonatorum appears symmetrically on the trunk thighs or buttocks However the skin hardening rapidly spreads to the entire body s subcutaneous fat excluding the parts of the body that are fat free such as the palms soles and genitalia A fixed face resembling a mask could be the result of the facial skin becoming harder 4 Causes editsclerema neonatorum s pathogenesis is still unknown When comparing the subcutaneous fat composition of older people with that of neonates it is evident that the former has a higher concentration of saturated fats Neonatal fat has a unique biochemical characteristic that increases its propensity to solidify in a cold environment 4 It has been proposed that subcutaneous adipose hardening in sclerema neonatorum is initiated by lowered body temperatures experienced during clinical shock 6 This theory is refuted by the fact that fat hardening shouldn t happen until skin temperature falls below freezing 4 Other theories suggest that sclerema neonatorum develops as a result of dysregulated fat metabolism stems from adipocyte peripheral connective tissue dysfunction or is a secondary effect of systemic toxicity 7 8 9 Diagnosis editsclerema neonatorum is typically diagnosed clinically when a critically sick newborn exhibits diffuse skin hardening The afflicted skin cannot be pitted folded or pinched because it is attached to the underlying tissue For histopathologic confirmation a skin biopsy could be helpful if the diagnosis is uncertain 4 Necrosis of subcutaneous fat without a significant inflammatory infiltrate and without obvious granulomatous changes the formation of needle shaped clefts in adipocytes sometimes in a radial arrangement and fibrous thickening of the tissue surrounding fat lobules are histopathologic findings that support a diagnosis of sclerema neonatorum 10 11 Outlook editsclerema neonatorum is linked to a high death rate because it worsens breathing and other essential functions in critically ill newborns 4 Based on case series the survival rate of affected neonates is estimated to be between 13 and 39 percent 3 There are usually no long term skin problems among survivors 11 Epidemiology editUsually affecting newborns Sclerema neonatorum manifests itself during the first week of life though some cases have been documented to occur outside of this time frame Based on the compilation of case reports it appears that men may experience the condition slightly more frequently than women male to female ratio 1 6 1 3 See also editPanniculitis Skin lesion List of cutaneous conditionsReferences edit William D James Timothy G Berger et al 2006 Andrews Diseases of the Skin clinical Dermatology Saunders Elsevier p 490 ISBN 978 0 7216 2921 6 Rapini Ronald P Bolognia Jean L Jorizzo Joseph L 2007 Dermatology 2 Volume Set St Louis Mosby pp 1515 1524 1525 ISBN 978 1 4160 2999 1 a b c Zeb A Darmstadt G L March 27 2008 Sclerema neonatorum a review of nomenclature clinical presentation histological features differential diagnoses and management Journal of Perinatology 28 7 Springer Science and Business Media LLC 453 460 doi 10 1038 jp 2008 33 ISSN 0743 8346 PMID 18368059 S2CID 205177400 a b c d e f UpToDate UpToDate Retrieved February 14 2024 Park Seh Hyun Kim Soo Chan 2017 Sclerema Neonatorum in a Full Term Infant Showing Favorable Prognosis Annals of Dermatology 29 6 Korean Dermatological Association and The Korean Society for Investigative Dermatology 790 793 doi 10 5021 ad 2017 29 6 790 ISSN 1013 9087 PMC 5705365 PMID 29200772 Hughes Wilson E Hammond Morton L 1948 Sclerema neonatorum The Journal of Pediatrics 32 6 Elsevier BV 676 692 doi 10 1016 s0022 3476 48 80224 6 ISSN 0022 3476 Kellum Robert E April 1 1968 Sclerema Neonatorum Archives of Dermatology 97 4 American Medical Association AMA 372 doi 10 1001 archderm 1968 01610100012002 ISSN 0003 987X Warwick Warren J June 1 1963 Sclerema Neonatorum A Sign Not a Disease JAMA The Journal of the American Medical Association 184 9 American Medical Association AMA 680 683 doi 10 1001 jama 1963 03700220056007 ISSN 0098 7484 PMID 13999025 Villacorte G Frank D J January 1967 Sclerema neonatorum A report of nine cases The Ohio State Medical Journal 63 1 57 59 PMID 6037143 Polcari Ingrid C Stein Sarah L July 28 2010 Panniculitis in childhood Dermatologic Therapy 23 4 Hindawi Limited 356 367 doi 10 1111 j 1529 8019 2010 01336 x ISSN 1396 0296 PMID 20666823 a b Requena Luis Yus Evaristo Sanchez 2001 Panniculitis Part I Mostly septal panniculitis Journal of the American Academy of Dermatology 45 2 Elsevier BV 163 186 doi 10 1067 mjd 2001 114736 ISSN 0190 9622 PMID 11464178 Further reading editShrestha Sandeep Chaudhary Nagendra Koirala Sujit Gupta Ruchi 2017 Sclerema Neonatorum Treated Successfully with Parenteral Steroids An Experience from a Resource Poor Country Case Reports in Pediatrics 2017 Hindawi Limited 1 4 doi 10 1155 2017 4836142 ISSN 2090 6803 PMC 5444035 PMID 28584673 Zeb Amna Rosenberg Rebecca E Ahmed ASM Nawshad Uddin Saha Samir K Chowdhury MAK Azad Ahmed Saifuddin Darmstadt Gary L 2009 Risk Factors for Sclerema Neonatorum in Preterm Neonates in Bangladesh Pediatric Infectious Disease Journal 28 5 Ovid Technologies Wolters Kluwer Health 435 438 doi 10 1097 inf 0b013e3181951af4 ISSN 0891 3668 PMID 19319014 External links editDermNet VisualDx Retrieved from https en wikipedia org w index php title Sclerema neonatorum amp oldid 1208842227, wikipedia, wiki, book, books, library,

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