Severe erytrodermic psoriasis in child twins: from clinical-pathological diagnosis to treatment of choice through genetic analyses: two case reports

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• 作者：Elena Campione ; Laura Diluvio ; Alessandro Terrinoni ; Augusto Orlandi…
• 关键词：Erythroderma ; Psoriasis ; Infancy ; Cyclosporine
• 刊名：BMC Research Notes
• 出版年：2014
• 出版时间：December 2014
• 年：2014
• 卷：7
• 期：1
• 全文大小：1,167 KB
• 参考文献：1. Pruszkowski, A, Bodemer, C, Fraitag, S, Teillac-Hamel, D, Amoric, JC, de Prost, Y (2000) Neonatal and infantile erythrodermas: a retrospective study of 51 patients. Arch Dermatol 136: pp. 875-880 CrossRef
  2. Fraitag, S, Bodemer, C (2010) Neonatal erythroderma. Curr Opin Pediatr 22: pp. 438-444 CrossRef
  3. Al-Dhalimi, MA (2007) Neonatal and infantile erythroderma: a clinical and follow-up study of 42 cases. J Dermatol 34: pp. 302-307 CrossRef
  4. Bard, S, Torchia, D, Schachner, LA (2010) Managing pediatric patients with psoriasis. Am J Clin Dermatol 11: pp. 15-17 CrossRef
  5. Sticherling, M, Augustin, M, Boehncke, WH, Christophers, E, Domm, S, Gollnick, H, Reich, K, Mrowietz, U (2011) Therapy of psoriasis in childhood and adolescence - a German expert consensus. J Dtsch Dermatol Ges 9: pp. 815-823
  6. Trueb, RM (2009) Therapies for childhood psoriasis. Curr Probl Dermatol 38: pp. 137-159 CrossRef
  7. Silverberg, NB (2009) Pediatric psoriasis: an update. Ther Clin Risk Manag 5: pp. 849-856 CrossRef
  8. Linder, D, Sampogna, F, Torreggiani, A, Balato, N, Bianchi, L, Cassano, N, Conti, A, Gisondi, P, Musumeci, ML, Piaserico, S, Potenza, C, Romano, GV, di Luzio, PU (2012) Psodisk, a new visual method for assessing the burden of psoriasis on patients. J Eur Acad Dermatol Venereol 26: pp. 1163-1166 CrossRef
  9. Ferlosio, A, Arcuri, G, Doldo, E, Scioli, MG, De Falco, S, Spagnoli, LG, Orlandi, A (2012) Age-related increase of stem marker expression influences vascular smooth muscle cell properties. Atherosclerosis 224: pp. 51-57 CrossRef
  10. Dogra, S, Kaur, I (2010) Childhood psoriasis. Indian J Dermatol Venereol Leprol 76: pp. 357-365 CrossRef
  11. Farber, EM, Mullen, RH, Jacobs, AH, Nall, L (1986) Infantile psoriasis: a follow-up study. Pediatr Dermatol 3: pp. 237-243 CrossRef
  12. Petrof, G, Mellerio, JE, McGrath, JA (2012) Desmosomal genodermatoses. Br J Dermatol 166: pp. 36-45 CrossRef
  13. Bowden, PE (2011) Peeling skin syndrome: genetic defects in late terminal differentiation of the epidermis. J Invest Dermatol 131: pp. 561-564 CrossRef
  14. Nini, G, Bianchi, L, Iraci, S, Camplone, G, Spagnuolo, A, Adorno, D, Papola, F (1989) HLA antigens and infantile psoriasis. Acta Derm Venereol Suppl (Stockh) 146: pp. 59-62
  15. Marrakchi, S, Guigue, P, Renshaw, BR, Puel, A, Pei, XY, Fraitag, S, Zribi, J, Bal, E, Cluzeau, C, Chrabieh, M, Towne, JE, Douangpanya, J, Pons, C, Mansour, S, Serre, V, Makni, H, Mahfoudh, N, Fakhfakh, F, Bodemer, C, Feingold, J, Hadj-Rabia, S, Favre, M, Genin, E, Sahbatou, M, Munnich, A, Casanova, JL, Sims, JE, Turki, H, Bachelez, H, Smahi, A (2011) Interleukin-36-receptor antagonist deficiency and generalized pustular psoriasis. N Engl J Med 18: pp. 620-628 CrossRef
  16. Pietroleonardo, L, Di, SA, Campione, E, Chimenti, S, Orlandi, A, Bianchi, L (2013) Confocal reflectance microscopy in pityriasis rubra pilaris. J Am Acad Dermatol 68: pp. 689-691 CrossRef
  17. Lallas, A, Apalla, Z, Karteridou, A, Lefaki, I (2013) Photoletter to the editor. Dermoscopy for discriminating between pityriasis rubra pilaris and psoriasis. J Dermatol Case Rep 30: pp. 20-22
  18. Haliasos, EC, Kerner, M, Jaimes-Lopez, N, Rudnicka, L, Zalaudek,
• 刊物主题：Biomedicine general; Medicine/Public Health, general; Life Sciences, general;
• 出版者：BioMed Central
• ISSN：1756-0500

文摘

Background Pediatric erythroderma is a severe cutaneous disorder, which may pose diagnostic and therapeutic challenges. Psoriasis, ichthyoses, atopy, seborrhoeic dermatitis, pityriasis rubra pilaris, infections, metabolic diseases, drugs reaction, may cause erythroderma. The therapy should be tailored on each aetiology, if possible. The biochemical and metabolic imbalance should be corrected, and particular attention should be paid to the psychosocial behavior often related to this disfiguring disease. Case presentation Two 3?year-old Caucasian twins have been suffering from an unmanageable erythroderma since the age of 8?months. The diagnosis of psoriasis, already remarkably expressed in the father’s family in three cases of fraternal twins, could be enforced for several points. Major histocompatibility complex, class I, Cw*06 was detected in both twins; we found no transglutaminase-1, no corneodesmosin, nor any Interleukin-36 receptor antagonist gene mutations. We performed a cutaneous histology, positive immunostaining for Lympho-epithelial Kazal-type-related inhibitor, dermoscopy and reflectance confocal microscopy. The twins had previously received systemic steroids, short cycles of low-dosage ciclosporine, followed by etanercept at the dosage of 0,8?mg/kg, without reliable results. Cyclosporine was then reconsidered at a dosage of 5?mg/kg/day with close blood monitoring. After three months of treatment, consistent clearing and significant improvement of their social and psychological behaviour were achieved. After over one year of continuous therapy with cyclosporine, the twins have still maintained the result obtained. Conclusion Pediatric erythroderma may pose a great challenge as a potentially life-threatening condition causing extreme distress in children, parents and pediatricians. In young patients it is mandatory to establish correct clinical and instrumental procedures, possibly supplemented by genetic analyses such as those we required, in order to determine an effective and safe therapy in terms of cost-benefit and put patients and family in the best condition to perform common daily activities.