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On-line version ISSN 1806-4841
An. Bras. Dermatol. vol.77 no.6 Rio de Janeiro Nov./Dec. 2002
WHAT IS YOUR DIAGNOSIS?
Katyuscia SchackerI; Jorge José de Souza FilhoII; Roberto Moreira AmorimIII; Rafael Lenzi TarnowskyIV; Caroline do Carmo GalindoV; Daniella Serafin Couto VieiraVI
M.D. at the Dermatology Service at the University Hospital
IIFull Professor, Department of Medical Clinic, "Universidade Federal de Santa Catarina", Head of the Dermatology Service at the University Hospital
IIIDermatologist, Adjunct Professsor IV at the Department of Medical Clinic, "Universidade Federal de Santa Catarina"
IVResident M.D. at the Dermatology Service at the University Hospital
VVisiting M.D. at the Dermatology Service at the University Hospital
VIAssistant Lecturer I at the Department of Anatomic Pathology of the "Universidade Federal de Santa Catarina". Pathology M.D. at the AnatomicPathology Diagnosis Institute (IDAP)
HISTORY OF THE DISEASE
Patient aged 54 years, female, white, married, housewife, natural of the municipality of Lages and resident in Florianópolis, SC, presented at the dermatology clinic complaining of lesions in the face and ear that had appeared approximately one year previously. Associated to the picture, she cited submandibular enlarged lymph node, dyspnea following moderate physical effort and occasional articular pain. She reported a prior history of hepatitis A, four years ago. She denied use of medications or similar cases in her family.
Physical exam showed a good general state, eupnea, with submandibular enlarged lymph node, movable lymph nodes with fibroelastic consistency. Dermatological exam revealed presence of erythematous maculopapules with a hardened consistency, varying from approximately 0.5 to 1cm in diameter located in the mentum, left labial joint and right retroauricular area (Figures 1 and 2).
For the laboratorial investigation, blood count, partial urine, glycemia, hepatic, renal and thyroid function, calcium, phosphorus and electrocardiogram were requested, all of which were within normal limits. Chest x-ray revealed bilateral, enlarged, hilar lymph node
Punch-biopsies were performed in two lesions, culture for fungi in selective medium resulted negative. Histopathology demonstrated epidermis without any alteration and the presence in the dermis media of non-confluent and non-caseous granulomas, some with asteroid corpuscle characterizing a sarcoidal type granulomatous dermatitis with PAS staining negative (Figure 3).
The principal diagnostic hypothesis was papulonodular sarcoidosis, followed by tuberculosis, tuberculous leprosy, syphilis and blastomycosis, the laboratory and anatomicopathological exams confirmed the initial diagnosis.
As of the diagnosis, treatment was initiated with 300mg/day chloroquine diphosphate, with periodic ophthalmologic attendance. A significant improvement was obtained in the cutaneous lesions and regression of the submandibular adenomegaly after two months of treatment, as well as improvement in the dyspnea (Figure 4).
Sarcoidosis was initially described in 1877 by Jonathon Hutchinson.1 It is a systemic non-caseous granulomatous disease that affects both sexes, of all races and ages.1,2
The etiology is unknown, but it is believed that sarcoidosis results from a genetic susceptibility and involvement of specific agents.3 Such a genetic predisposition could explain the heterogeneity and gravity of the disease among different racial groups. The histocompatibility antigens HLA A1 / B8 / Cw7 / DR3 are related to acute disease and favorable prognostic, while a chronic course is marked by HLA DR14 and DR15, among many others. The immunological abnormalities are characterized by an increase in T cells, of types Th1 and CD4, as well as macrophages in the areas of greatest inflammatory reaction. Cytokines and other mediators, produced by these cells, contribute to the formation of the granuloma. Various infectious agents are implicated in the etiology of sarcoidosis: viral, such as herpes simplex virus, retrovirus and Epstein-Barr; bacterial, such as Propionibacterium acnes, Borrelia burgdorferi, Mycoplasma, Chlamydia and Nocardia; and Mycobacteria, for examples Mycobacterium tuberculosis and Mycobacterium paratuberculosis.1,3
Cutaneous involvement occurs in 25% of sarcoidosis cases. The cutaneous lesions are classified as specific, when they present sarcoid granuloma (noncaseating granuloma) in the histopathology, the most typical being lupus pernio, lesions in infiltrated plaques, maculopapuliferous eruptions and subcutaneous nodules. The most common nonspecific lesion is erythema nodosum.4
Lupus pernio is the most characteristic cutaneous lesion of sarcoidosis. Frequently it is associated to involvement of the respiratory tract. Maculopapuliferous lesions are the most common and characterized by erythematous papules of less than 1cm in diameter that involve the periorificial areas, face, neck, back and extremities. There are also some specific lesions with uncommon manifestations, such as: ulceration; hypopigmentation; lesions with verrucose, icteroid, lichenoid and erythrodermal characteristics; folliculitis; cicatricial alopecia; and many others.2,4
Nodular erythema is considered a nonspecific, acute and benign lesion. It characterizes Löfgren's syndrome when associated to bilateral hilar adenopathy, with or without pulmonary fibrosis, migratory polyarthritis, fever and iritis.1,2,4
Extracutaneous manifestations involve constitutional symptoms, such as fever, fatigue, weight loss and manifestations in specific organs. The lungs are affected in 90% of cases.4,5 The manifestations of lung disease are dyspnea, coughing, thoracic pain and albeit rarely hemoptysis.1,2,4,6 Radiographic findings show that pulmonary sarcoidosis can be grouped into: stage 0, normal x-ray; stage I, bilateral hilar adenopathy and/or tracheal adenopathy; stage II, adenopathy and pulmonary infiltration; stage III, only pulmonary infiltration; and stage IV, pulmonary fibrosis.6 The lymph nodes, eyes, liver, spleen, thyroid, bones, kidney, nervous system and heart can also be involved.1,2,3 In the reported case, tomography of the thorax did not reveal infiltration or fibrosis of the pulmonary parenchyma; and on using a respirometer it was shown that the parameters were within normal limits.
Diagnosis of sarcoidosis is based on compatible clinical picture, with demonstration of non-caseous granuloma in the histopathology and exclusion of diseases that can cause a similar histological alteration.1 The first step for the diagnosis is to perform a biopsy. Then other affected systems should be investigated using chest X-ray, ECG, renal, liver and thyroid function, blood count, calcium, phosphorus, partial urine, ophthalmologic exam and tuberculínico test.1,3 AThere may be elevation of the angiotensin-converting enzyme (ACE), which is produced by the kidney and in sarcoidosis by the epithelioid cells in the periphery of the granulomas. ACE is not specific to sarcoidosis, however it is used as an aid in the diagnostics.2
The differential diagnosis of the cutaneous sarcoidosis is made between tuberculosis, leishmaniasis, blastomycosis, tuberculoid leprosy and syphilis.4
Chloroquine (250-500mg/d) and hydroxychloroquine (200-400mg/d) have been used as first-line drugs for the treatment of chronic cutaneous sarcoidosis.1 Other alternatives are oral corticosteroids, methotrexate, thalidomide and allopurinol. In the treatment of the acute cutaneous lesions, without systemic involvement, one can use topical, intralesional or oral corticosteroids.1,2,7
The prognostic in sarcoidosis depends mainly on the extension and gravity of the systemic involvement.8 Acute lesions have a more favorable prognostic, there can be a spontaneous remission. The clinical course is fatal for approximately one to 5% of the patients with CD.3
1. Hunninchake GW, Costabel U, Ando M, et al. Statement on Sarcoidosis. Am J Resp Crit Care Med 1999; 160: 736-55.
2. English JC, Patel PJ, Greer KE. Sarcoidoses. J Am Acad Dermatol 2001; 44: 725-43.
3. Costabel U. Sarcoidoses: clinical update. Eur Respir J 2001; 18: suppl. 32. 56-68.
4. Mana JMD, Marcoval J, Graells J, et al. Cutaneous Involvemente in Sarcoidosis. Arch Dermatol 1997; 133: 882-87.
5. Newman LS, Rose CS, Maier LA. Sarcoidosis. N EnglJ Med 1997; 336: 1224-34.
6. Linch, JP. Pulmonary Sarcoidosis: Current Concepts and Controversies. Comprehensive Therapy 1997; 23(3): 197-210.
7. Jonhs CJ, Mochelle TM. The clinical management of sarcoidosis: a 50-years experience at Johns Hopkins Hospital. Medicina 1999; 78: 65-111.
8. Neville E, Walker AN, James DG. Prognostic factors predicting the autcome of sarcoidosis: an analysis of 818 patients. Q J Med 1983; 52: 525-33.
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* Work done at the Service of Dermatology of "Hospital Universitário da Universidade Federal de Santa Catarina".