Pernio: Tanı ve tedavi seçenekleri

Pernio (chilblains), ilk olarak 1881 yılında Henry Piffard tarafından tanımlanan, nem ve soğuğa karşı anormal bir inflamatuvar cevaptır. Pernio dondurucu olmayan soğuğa maruz kalan duyarlı kişilerde gelişir. Genç kadınlarda oldukça yaygın görülmekle birlikte, çocuk ve yaşlılarda da görülebilir. Özellikle el ve ayak parmakları olmak üzere, distal ekstremiteleri etkileyen, inflamatuvar, eritemli veya menekşe rengi, ağrılı veya kaşıntılı kutanöz lezyonlarla karakterizedir. Şiddetli olgularda bül ve ülserayon görülebilir. Tanı genellikle klinik bulgulara dayalı olarak konur. Deri biyopsisi perniyoyu taklitçilerinden ayırmada yardımcı olabilir. Histopatolojide genellikle yoğun papiller dermal ödem, süperfisyal ve derin perivasküler lenfositik infiltrat izlenir. Pernio idiyopatik olabileceği gibi, sistemik lupus eritematozus gibi sistemik hastalıklarla da ilişkili olabilir. İdiyopatik pernio kendi kendini sınırlayıcıdır ve günler içerisinde rezolüsyona uğrayabilir. Perniyonun anahtar tedavisi soğuk maruziyetinden kaçınılmasıdır. Nifedipin, tedavide ve relapsların önlenmesinde etkili bulunmuştur. Diğer tedaviler topikal florlu kortikosteroidler, nikotinamid, sempatektomi ve ultraviyole B tedavisini içerir. Bu derleme, perniyonun klinik özellikleri, ayırıcı tanısı ve tedavisi ile ilgili genel bir bakış sağlamaktadır.

Perniosis: Diagnosıs and treatment alternatives

Perniosis (chilblains) is an abnormal inflammatory response to cold and damp that was first described by Henry Piffard in 1881. Perniosis develops among susceptible individuals who are exposed to nonfreezing cold. Perniosis most commonly occurs among young women but may occur among older individuals or among children. It is characterized by inflammatory, erythematous or violaceous, pruritic or painful cutaneous lesions involving distal extremities, espe¬cially the fingers and the toes. In severe cases, blistering and ulceration may occur. The diagnosis of perniosis is usually based on clinical grounds. A skin biopsy may assist differentiation from mimicks. Histology usually shows intense papillary dermal edema, superficial and deep perivascular lymphocytic infiltrates. Perniosis can be idiopathic or associated with a systemic disease such as systemic lupus erythematosus. Idiopathic perniosis is self-limited and can resolve within days. The key therapy for perniosis is avoiding further cold exposure. Nifedipine was found to be effective and to prevent relapse. Other therapies include topical fluorinated corticosteroids, nicotinamide, sympathectomy, and ultraviolet B phototherapy. This review provides an overview of the clinical features, differential diagnosis ve treatment of perniosis.

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  • 1.Oumeish OY. Common acrally distributed dermatoses. Clin Dermatol. 2011 Mar-Apr;29(2):130-139.
  • 2.Piffard HG. A treatise on the Materia Medica and therapeutics of the skin. New York: William Wood and Co.; 1881. p. 236.
  • 3.Jordaan HF. The diagnosis and management of perniosis (chilblains). SA Fam Pract 2007;49(6):28-29.
  • 4.Vano-Galvan S, Martorell A. Chilblains. CMAJ. 2011 Oct 24. (Epub ahead of print)
  • 5.Guadagni M, Nazzari G. Acute perniosis in elderly people: a predictive sign of systemic disease? Acta Derm Venereol. 2010 Sep;90(5):544-545.
  • 6.Prakash S, Weisman MH. Idiopathic chilblains. Am J Med. 2009 Dec;122(12):1152-1155.
  • 7.St Clair NE, Kim CC, Semrin G, et al. Celiac disease presenting with chilblains in an adolescent girl. Pediatr Dermatol. 2006;23:451-454.
  • 8.White KP, Roth MJ, Milanese A. Perniosis in association with anorexia nervosa. Pediatr Dermatol. 1994;11:1-5.
  • 9.Souwer IH, Lagro-Janssen AL. Chronic chilblains. BMJ. 2011 Jun 7;342:d2708.
  • 10.Raza N, Habib A, Razvi SK, et al. Constitutional and behavioral risk factors for chilblains: a case-control study from Pakistan. Wilderness Environ Med. 2010 Mar;21(1):17-21.
  • 11.Simon TD, Soep JB, Hollister JR. Pernio in pediatrics. Pediatrics. 2005 Sep;116(3):e472-475.
  • 12.Weston WL, Morelli JG. Childhood pernio and cryoproteins. Pediatr Dermatol. 2000;17:97–99.
  • 13.Yazawa H, Saga K, Omori F, et al. The chilblain-like eruption as a diagnostic clue to the blast crisis of chronic myelocytic leukemia. J Am Acad Dermatol. 2004;50(suppl):S42–S44.
  • 14.Tan BB, Lear JT, English JS. Metastasis from carcinoma of breast masquerading as chilblains. J R Soc Med. 1997;90:162.
  • 15.Reinertsen JL. Unusual pernio-like reaction to sulindac. Arthritis Rheum.1981;24:1215.
  • 16.Inoue G, Miura T. Microgeodic disease affecting the hands and feet of children. J Pediatr Orthop. 1991 Jan-Feb;11(1):59-63.
  • 17.Meller Y, Bar-Ziv J, Goldstein J, et al. Phalangeal microgeodic syndrome in childhood: a case report. Acta Orthop Scand. 1982 Aug;53(4):553-556.
  • 18.Burgdorf WHC, Plewig G, Wollf HH, Landthaler M. Braun-Falco’s Dermatology. 3rd ed. Berlin, Springer-Verlag, 2009;598-617.
  • 19.Chan Y, Tang WY, Lam WY, et al. A cluster of chilblains in Hong Kong. Hon Kong Med J. 2008 Jun;14(3):185-191.
  • 20.Crowson AN, Magro CM. Idiopathic perniosis and its mimics: a clinical and histological study of 38 cases. Human Pathol. 1997 Apr;28(4):478-484.
  • 21.Goette DK. Chilblains (perniosis). J Am Acad Dermatol 1990;23: 257-262.
  • 22.Yang X, Perez OA, English JC 3rd. Adult perniosis and cryoglobulinemia: a retrospective study and review of the literature. J Am Acad Dermatol 2010;62:e21-2.
  • 23.Rustin MH, Newton JA, Smith NP, et al. The treatment of chilblains with nifedipine: the results of a pilot study, a double-blind placebocontrolled randomized study and a long-term open trial. Br J Dermatol 1989;120:267-275.
  • 24.Cribier B. Chilblain. Ann Dermatol Venereol 2001;128:557-560.
  • 25.Burry JN. Adverse effects of topical fluorinated corticosteroid agents on chilblains. Med J Aust. 1987;146:451–452.
  • 26.Ganor S. Corticosteroid therapy for pernio. J Am Acad Dermatol. 1983;8:136.
  • 27.Langtry JA, Diffey BL. A double-blind study of ultraviolet phototherapy in the prophylaxis of chilblains. Acta Derm Venereol. 1989;69:320–322.
  • 28.Yang X, Perez OA, English JC 3rd. Successful treatment of perniosis with hydroxychloroquine. J Drugs Dermatol. 2010 Oct;9(10):1242-1246.
  • 29.Noaimi AA, Fadheel BM. Treatment of perniosis with oral pentoxyfylline in comparison with oral prednisolone plus topical clobetasol ointment in Iraqi patients. Saudi Med J. 2008 Dec;29(12):1762-1764.
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  • Başlangıç: 2009
  • Yayıncı: MEDİTAGEM Ltd. Şti.