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Print version ISSN 0365-0596On-line version ISSN 1806-4841
An. Bras. Dermatol. vol.79 no.1 Rio de Janeiro Jan./Feb. 2004
Sarcoidosis in previous scars*
Hiram Larangeira de Almeida JrI; Heitor Alberto JannkeII
Professor of Dermatology
IITitular Professor of Pathology
The authors report the case of a 20-year-old male patient, who presented sudden infiltration of previous scars on the forehead, which were due to an accident occured four years ago. Light microscopy showed noncaseating, nonconfluent granulomas with few lymphocytes; special stains for fungi and mycobacteria were negative, as well as examination under polarized light. Radiologic examination of the chest, ophthalmologic screening and calcemia were normal, which lead to the diagnosis of a sarcoidosis in previous scars without systemic involvement. There was a good response to treatment with intralesional steroids.
Key-words: granuloma; sarcoidosis.
Sarcoidosis is a noninfectious granulomatous disease with unknown etiology, which can have exclusively cutaneous involvement or affect several organs, most commonly the lungs, eyes, lymph nodes and bones.
The cutaneous involvement can be specific, marked by the formation of epithelioid granulomas with poor lymphocytic infiltrate, also denominated "nude" granulomas or it can be nonspecific, such as erythema nodosum accompanying bilateral hilar adenopathy, characterizing Loefgren's syndrome.1
The forms of cutaneous involvement include sarcoidosis in previous scars, in which formation of the typical granulomas occurs in previous scars and may or may not be associated with extracutaneous manifestations.
Male patient, 20 years of age, complained that two months ago he had noticed an infiltration in scars with previous sutures on the forehead, which were a result of an automobile accident four years prior to this. The lesions were asymptomatic and the patient did not present systemic complaints.
Dermatological exam showed several infiltrations in the forehead (Figure 1); some clearly followed the path of the scars and were slightly erythematous (Figure 2); not all presented modification. The ganglions of the cervical segment were not enlarged.
With the diagnostic hypotheses of foreign body granuloma and cicatricial sarcoidosis (SC), a surgical biopsy of the lesion was performed. The anatomicopathological exam showed epithelioid cell granulomas with poor lymphocytic infiltrate, Langhans'-type giant cells and some asteroid corpuscles (Figure 3). Microscopic exam with polarized light did not demonstrate birefringent fragments. Specific stains for fungi and mycobacteria were negative.
Chest x-ray, ophthalmologic exam and calcemia were all normal.
The lesions were infiltrated with triamcinolone diacetate at a concentration of 10mg/ml, which provoked their regression.
The fundamental characteristic of sarcoidosis is the tissular infiltration by epithelioid cell granulomas, occurring preferentially in the skin, eyes, bones, lymph nodes and lungs. An exception to this is erythema nodosum, which can accompany the classic bilateral hilar adenopathy, the histology of which does not show granulomas and has an acute clinical course, with a tendency towards spontaneous resolution.2,3
The cutaneous manifestations can be expressed by a variety of lesions, such as infiltrated plaques,4 disseminated papules with the classic aspect of apple jelly or infiltration of the nose in lupus pernio.5,6 more rarely there can occur ulceration,2,3,7 erythroderma,2 dactylitis,5 hypochromic lesions,5 cicatricial alopecia,2,3 ungual lesions3 and lichenoid eruption.3
In the Brazilian dermatological literature over the last two decades there have been few reports of sarcoidosis, with descriptions of the ulcerous forms,7 lupus pernio6 and in plaques,4 all of which associated with lung lesions.
The infiltration of previous scars parallel to a clinical picture of sarcoidosis is well known, it can, however, present exclusively cutaneous involvement and even precede the systemic manifestations.
In an interesting publication regarding 188 cases of Caucasian patients, recorded over a period of 12 years in Denmark,3 Veint found 50 (26.6%) patients solely with cutaneous involvement, of which six (3.2%) only presented SC. Of the remaining 138 patients with varying stages of lung disease, 20 (14.5%) presented associated lesions in the scars. Evaluating the clinical course of the latter, 84% of the patients with SC, without specifying whether it was just cutaneous or not, were in the group with a chronic course of over two years duration.
In another report, regarding 54 South African blacks, SC was found in one (1.8%) patient, again demonstrating it is infrequent among the black population.5
SC has been described in desensitization injections,8 minor traumas,9 firearm lesions,2 venipuncture,10 after herpes zoster,11,12 scarring rituals13,14 and tattoos.15 The time between the initial lesion and onset of SC varies from several months12 to 38 years.2 In some situations, such as tattoos and in the areas of desensitization injections, the longer the time interval between the two events, the lower the possibility that it is foreign body granuloma,15 This is an important differential diagnosis which is sometimes difficult in purely cutaneous SC.
The histological diagnosis is reached by exclusion, necessitating specific stains to eliminate the possibility of granulomatous disease due to fungi and mycobacteria, similarly exam with polarized light helps to discard sarcoidosis-like foreign body granuloma, although however, this cannot be totally ruled out. The poor lymphocytic infiltrate is characteristic of granulomas in sarcoidosis and the presence of asteroid bodies, although not a pathognomonic symptom, does help to establish the diagnosis. Also presenting difficulty are granulomatous rosacea, lupus vulgaris and granuloma annulare.3 in the first two, therapeutic tests with tetracycline and exam by microbiological culture, respectively, may be necessary.3
Ultrastructure and immunohistochemistry do not facilitate the diagnosis,3,9 as they add little to conventional histology. There is a report that immunohistochemical study of SC demonstrates the expression of macrophagocytic markers and T-helper lymphocytes.9
Kwein's intradermal reaction is rarely used in the diagnosis16 and is difficult to obtain in Brazil. Although it is a classic hypothesis that patients with sarcoidosis have a negative reaction to tuberculin in the acute phase of the disease, in function of the immunological alterations that accompany the picture, there have been reports of its positivity in cases of sarcoidosis,16 thereby compromising the importance of this line of investigation. It was not performed in the case described here, since the clinical-histological correlation enabled diagnosis.
The treatment seeks to contain the formation of granulomas, using intralesional or systemic corticoids, which may or may not be associated with antimalarial agents. Methotrexate at a weekly dose of 15 to 25mg is also a therapeutic alternative.17 Other reports of success in the treatment of sarcoidosis used thalidomide18 and allopurinol.1 Intralesional infiltration of corticoid was opted for because there was no evidence of extracutaneous, involvement and regression of the lesions was acheived.
The authors opted for the title "sarcoidosis in previous scars", instead of "cicatricial sarcoidosis", in order to differentiate this from a sarcoidosis that leads to the formation of scars, this being perhaps the most correct translation of the terms found in the Anglo-Saxon literature (scar sarcoidosis - Narbensarkoidose).
The authors thank Dra. Dorothée Eich of the laboratory of histopathology at the dermatological clinic, University of Cologne (Germany) for performing the exam with polarized light.
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Prof. Dr. Hiram Larangeira de Almeida Jr.
Departamento de Medicina Especializada Faculdade de Medicina da UFPEL
Av. Duque de Caxias, 250
96030-002 Pelotas RS
Tel./Fax: (53) 278-7582
in June, 20th of 2000
Approved by the Consultive Council and accepted for publication in January, 21st of 2003
* Work done at the Dermatology Dept. of the Federal University of Pelotas Medical School.