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Analysing of sarcoidosis patients with scar involvement at onset

Analysing of sarcoidosis patients with scar involvement at onset. Sevtap Sipahi Demirkok*, Muammer Bilir* *Division of Lung Diseases, Department of Internal Medicine, Cerrahpasa Medical School,Istanbul University, Istanbul, Turkey. Background.

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Analysing of sarcoidosis patients with scar involvement at onset

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  1. Analysing of sarcoidosis patients with scar involvement at onset Sevtap Sipahi Demirkok*, Muammer Bilir**Division of Lung Diseases, Department of Internal Medicine, Cerrahpasa Medical School,Istanbul University, Istanbul, Turkey

  2. Background • The main controversy that revolves around scar sarcoidosis is its unknown pathogenesis and relationship to systemic disease. • We aimed( i) to evaluate the clinical, demographic and diagnostic features of sarcoidosis patients with scar involvement at onset. (ii) to clarify the relationship between scar sarcoidosis and systemic disease in patients who were followed-up more than two years

  3. Material and Methods • Twelve patients with scar involvement in a cohort of 492 symptomatic, newly diagnosed sarcoidosis cases at the initial presentations were included in this retrospective study (1966-2004). • The diagnosis of sarcoidosis was established when clinicoradiological findings were supported by histological evidence of non-caseating epithelioid cell granulomas in one or more than one organ system and exclusion of other disorders known to cause granulomatous disease. • The demographic features, presenting clinical features, course of the disease, initial diagnosis methods for each patient were analyzed

  4. Results • The study population included 8 female and 4 male patients with the mean age of 39.08±9.59 yrs. • At the time of initial diagnosis; • 1 patient with scar involvement had a normal chest x-ray • 6 patients presented with bilateral hilar adenopathy • 4 patients with stage II • 1 patient with stage III.

  5. Initial manifestations • Arthralgia/arthritis (6) • Respiratory symptoms ( 4) • Constütional symptoms (4) • Peripheral lymphadenopathy(4) • Eritema nodosum (4) • myalgia (2) • Ocular involvement(2 )

  6. . • Hepatomegaly (2) • Skin lesions (2) • PPD anergy(8) *The total number of observations is higher than the number of patients because some patients had more than one type of organ involvement

  7. Initial Diagnostic Methods • In five patients the initial diagnostic method was skin-scar biopsy.Out of 4, the diagnosis was supported by other organ biopsies as well. • In the other 7 patients,diagnosis was established by other organ biopsies other than scar.

  8. Follow-up of Patients with Scar Involvement • Eight patients were followed-up for a mean time of 116.5 ± 77.4 months. • Remission was noted in more than half of the patients within the first 2 years.(mean time± SD: 13.87± 6.97 months) • Seven patients followed-up more than 2 years were treated.

  9. . • Totally, 1 patient relapsed on follow-up. She had two further episodes of scar sarcoidosis in an 8 year follow-up period. • She had a spontaneous resolution of hilar and mediastinal lymphadenopathy 6 months following the diagnosis. • Four years after complete remission of sarcoidosis the patient complained that her thyroidectomy incision scar rose up, reddened and hardened. • Sipahi Demirkök S,Arzuhal N,Devranoglu G,Demirkesen C,Tuzun Y. Recurrent sarcoidosis on scar associated with vitiligo. 14th EADV Congress,2005,London,England,Journal of the Eurepean Academy of Dematology and Venerology (Abstr) 2005;19(suppl;2) 52(P09). • Sipahi Demirkök S,Arzuhal N,Devranoglu G,Demirkesen C,Tuzun Y. Recurrent sarcoidosis on scar associated with vitiligo.International Journal of Dermatology (article in press)

  10. Infiltration of old scars in sarcoidosis • Controversial results were reported that scar sarcoidosis was associated with subacute or chronic forms systemic sarcoidosis.

  11. Prevelance of scar sarcoidois • Cutaneous involvement during the course of systemic sarcoidosis is observed in 9-37% of the patients. • Infiltration old scars resulting from surgery, trauma, venipuncture and tattoos which we encounter frequently in the last years, is a charasteristic finding in sarcoidosis. • Samtsov AV: Cutaneous sarcoidosis,Int J Dermatol, 31: 385-391, 1992.

  12. . • In a few study,investigating with the behaviour of scar sarcoidosis reported controversial results besides different frequency of scar sarcoidosis. • In the acute phase of the disease,scar sarcoidosis may follow EN. • However,there are a lot of reports which suggest that it is more often associated with longstanding pulmonary involvement and uveitis,peripheral lymphadenopathy,bone cysts or parotid enlargement. *Scadding JG, Mitchell DN: Sarcoidosis of the skin, in Scadding JG, Mitchell DN (eds): Sarcoidosis, Chapman&Hall Medical, London, 1985, p.181-206. *James DG.Dermatologic aspects of sarcoidosis. QJM. 1959;28:109 124

  13. . • There are also some reports in which show coexistence of scar sarcoidosis with acute or chronic forms of sarcoidosis with a tendency to resolve simultaneously *Manz LA, Rodman OG: Reappearance of quiescent scars sarcoidosis, Arch Dermatol, 129: 105-108, 1993. *Scadding JG, Mitchell DN: Sarcoidosis of the skin, in Scadding JG, Mitchell DN (eds): Sarcoidosis, Chapman&Hall Medical, London, 1985, p.181-206

  14. . • Studies reporting coexistence of scar sarcoidosis and chronic form of systemic sarcoidosis also showed important information about the behaviour of scar sarcoidosis. *Chudomira K, Velichkova L, Anavi B et al: Recurrent sarcoidosis in skin scars accompanying systemic sarcoidosis, J Eur Acad Dermatol Venereol, 17: 360-361, 2003. * Veien NK, Stahl D, Brodthagen H: Cutaneous sarcoidosis in Caucasians, J Am Acad Dermatol, 16: 534-540, 1987

  15. . • Twentytwo of of 26 patients with scar sarcoidosis described by Veien et al showed chronic active disease 2 years after diagnosis. • Besides, 17 out of 26 patients associated with the disease stage II or stage III chest x-ray and lonstanding manifestations of systemic sarcoidosis

  16. . • In the same study,only six of 26 patients with scar sarcoidosis presented as a sole lesion isolated to skin without any other manifestation of systemic sarcoidosis. *Veien NK, Stahl D, Brodthagen H: Cutaneous sarcoidosis in Caucasians, J Am Acad Dermatol, 16: 534-540, 1987

  17. . • With similar results, Caro suggested that most patients with scar sarcoidosis have other systemic manifestations and changing scars in patients in remission may indicate disease exacerbation *Caro I: Scar sarcoidosis, Cutis, 32: 531-533, 1983

  18. . • In our study,most of our sarcoidosis patients with scar involvement coexisted with subacute forms of systemic disease with a tendency to regress simultaneously.

  19. Conclusion • During follow-up of patients with sarcoidosis, examination of any scar should be part of routine physical examination in order not to miss a possible site of relapse in these patients. • If infiltration of old scars is detected in a patient, the diagnosis of sarcoidois must be verified other than scar tissue as well.

  20. . • It must be always remembered that although scar sarcoidosis is associated with the acute phase or longstanding manifestations of sarcoidosis, it may be the only manifestation of a sarcoid flare-up. • Demirkök Sipahi S.Reactivation of old scars:inevitably sarcoid. Postgrad Med J Online, 23- November-2005 (http://pmj.bmjjournals.com/cgi/eletters/81/951/60)

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