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Print version ISSN 0365-0596
An. Bras. Dermatol. vol.85 no.6 Rio de Janeiro Nov./Dec. 2010
Sarcoidosis on skin scars - a case report*
Sônia Antunes de Oliveira ManteseI; Alceu Luiz Camargo Villela BerbertII; Thaís Silveira CesárioIII; Henrique Borges da SilvaIV
IAssociate Professor II of Dermatology of the Medical School of the Federal University of Uberlândiqa (UFU) - MD from the Federal University of São Paulo (UNIFESP) - São Paulo (SP), Brazil
IIAssistant Professor IV of Dermatology of the Medical Shool of m the Federal University of Uberlândia (UFU) - MSc in General Medicine from the Federal University of Uberlândia (UFU) - Uberlândia (MG), Brazil
IIIResident of the Dermatological Service of the University Hospital of the Federal University of Uberlândia (UFU) - Uberlândia (MG), Brazil
IVAssistant professor I of the Pathologic Anatomy Service of the Medical School of the Federal University of Uberlândia (UFU) - Uberlândia (MG), Brazil
Sarcoidosis is a systemic inflammatory disorder of unknown origin, in which non-caseating granulomas (small inflammatory nodules) are found in the affected organs. Cutaneous involvement occurs in 25% of cases with a wide range of clinical presentation. The onset of scars is unsual although clinically characteristic of cutaneous sarcoidosis. Most patients with scar sarcoidosis have a systemic disease. It is reported the case of a 65 year-old woman that developed scar nodules 20 years after she had had surgical procedures without systemic manifestations. It is worth mentioning the importance of investigating sarcoidosis with inflammatory alterations in praevia scars.
Keywords: Cicatrix; Granuloma; Sarcoidosis; Skin; Skin diseases
Sarcoidosis is a granulomatous noninfectious disease of unknown etiology that can present only cutaneous involvement or affect many organs such as lungs, eyes, lymphonodus and bones.1 The first description of sarcoidosis, in 1800, was related to its cutaneous manifestations.The term sacoidosis derives from a report from Boeck, in 1899, and it is due to the clinical similarities of the lesions with benign sarcomas. At the beginning of the year 1900, sarcoidosis was described involving lungs and other internal organs.2 Sarcoidosis usually occurs in young adults, with two peaks of incidence: between 25 and 35 and 45 and 55 years of age.3 There is a prevalence of the disease among women.3,4
Cutaneous sarcoidosis is known as a great simulator of other diseases because of the lesional polymorphism5 and therefore it represents a major diagnostic challenge.3
It is recognised a wide range of clinical presentations of cutaneous sarcoidosis: nonspecific lesions, generally related with the acute phase of the disease and with good prognosis, outstanding the erythema nodosum and specific lesions, more frequently observed in chronic diseases, with worse prognosis, such as maculopapular, nodules, plaques, infiltrated scars, lupus pernio, ulcerations, warty lesions and erythroderma.6
Cicatricial onset is rare but clinically characteristic of cutaneous sarcoidosis.7
Female patient, white, aged 65, with painless nodules on scars from surgeries she had had 20 years ago. Dermatologic exams showed the presence of erythematous violaceous nodules grouped on scars resulting from blepharoplasty, on the left upper eyelid, and on the left lateral side of infra-umbical scar resulting from hysterectomy and caesarean (Figure 1).
It was carried out incisional biopsy, with histopathology showing chronic granulomatous inflammation, with numerous epithelioid macrophages and many multinucleated giant cells, strange body type and Langhans, without evidence of necrosis (Figure 2). Staining for fungus and for resistant alcohol-acid bacillus (BAAR) in the fragment were negative.
The patient did not present any systemic manifestations. Radiography of the chest, pulmonary function tests, eletrocardiogram (ECG) and laboratorial exams (hepatic and renal functions, serum calcium, C-reactive protein) did not present any alterations.
It was decided to introduce topic corticoid, of average potency, associated with oral prednisone (40 mg/day), with complete resolution of the nodular lesions after two months, followed by a gradual reduction of oral corticotherapy.
Sacoidosis is a multisystemic disease, of unknown etiology, characterized by the formation of non-caseating granulomas in the affected organs.2,7 The involvement of the skin occurs in 25% of the cases and it can develop in any phase of the disease although it is more common at the beginning8.
The relation between cutaneous and systemic sarcoidosis has been evaluated.5 About 30% of the patients with isolated cutaneous lesions will develop systemic sarcoidosis, after a period of time which varies from one month to one year .7
Cicatricial onset has been found in 29% of the patients with cutaneous sarcoidosis.2 Besides the reactivation of scars originated from previous wounds, cicatricial sarcoidosis has been found in areas of intramuscular injections and hyaluronic acid, tatoos, venepuncture and cutaneous manifestation of herpes zoster , after a period of time which varies from six months to 59 years.7,9 The previous contamination of these scars , with strange material, has been suggested as a possible subjacent cause.7 The patient in study showed nodules on scars 20 years after the performance of surgical procedures (blepharoplasty, hysterectomy and caesarean), without report of trauma or infections.
Cicatricial sarcoidosis can occur singly or it can precede, follow or appear during the reactivation of a systemic disease. The majority of the patients with cicatricial sarcoidosis present other systemic manifestations and, changes on the scars, may indicate exarcebation of the disease.7 So, it is recommended for all patients with cutaneous sarcoidosis a periodic clinical evaluation, including complete anamnesis, physical exam, serum calcium, renal and hepatic functions, radiography of the chest, pulmonary function tests , eletrocardiogram (ECG) and ophthalmological evaluation 5,7 In the case reported there was only cutaneous manifestation of the disease, with characteristic histopathological condition ,without any other clinical or laboratorial alterations . However, it is extremely important to have a strict monitoring as, according to the medical literature, a great number of patients develop, afterwards, systemic onset.
The initial clinical hypothesis of cutaneous sacoidosis is not normally posed and the diagnosis is confirmed by biopsy after the exclusion of other causes7. The histopathological exam should investigate as differential diagnoses: infectious disease, strange body granulomas, neoplasias, immunodeficiencies, medicamental eruptions, and other granulomatous processes. Due to its lesional polimorfism the diagnosis of sarcoidosis should be based on clinical criteria; radiographic, laboratorial, epidemiological, and histopathological.3 The identification of the skin lesions is important for the diagnosis of sarcoidosis and they constitute sources, easily accessible, for the anatomopathological exam7. The cicatricial sarcoidosis frequently presents slow and spontaneous resolution.2 The intralesional and topic corticosteroids of high potency can be effective in the pure cutaneous sarcoidosis.6,7 When there are disfiguring lesions non responsive to the initial topic therapy or in cases with systemic involvement oral prednisone, hydroxichloroquine and methotrexate can be used.7 Systemic corticotherapy, in this case, was briefly used associated with topic therapy with complete resolution of the lesions after two months. It is important to pose the clinical hypothesis of sarcoidosis in patients with alterations in areas of trauma and/or scars, and once the diagnosis is confirmed, periodic monitoring for the detection of eventual systemic manifestations is needed.
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Mailing address: Received on 06.08.2009. * Work carried out in the Dermatological Service of the University Hospital of the Federal University of Uberlândia (HC/UFU) - Uberlândia (MG), Brazil.
Sônia Antunes de Oliveira Mantese
Avenida Pará, 1979 - Jardim Umuarama
Phone/fax: 34 3218 2246 (Departamento de Clínica Médica UFU)
E- mail: firstname.lastname@example.org
Approved by the Advisory Board and accepted for publication on 27.11.09.
Conflict of interest: None
Financial funding: None
Received on 06.08.2009.
* Work carried out in the Dermatological Service of the University Hospital of the Federal University of Uberlândia (HC/UFU) - Uberlândia (MG), Brazil.