2014.4-22.Sarcoidosis

                                                                                                                            article in PDF  
Our Dermatol Online.  2014; 5(4): 423-425
DOI:.  10.7241/ourd.20144.106
Date of submission:  12.06.2014 / acceptance: 04.08.2014
Conflicts of interest: None
 

SARCOIDOSIS MIMICKING SEBORRHEIC DERMATITIS: ANOTHER CASE OF SHERLOCKIAN DERMATOLOGY

César Bimbi

Member of Dermatological Comittee of State Medical Council of Rio Grande do Sul Brazil, Brazilian Society of Dermatology
 

Corresponding author:  Dr César Bimbi    e-mail: cbimbi@terra.com.br


 

Abstract
Even tiny despicable skin signs may eventually unveil systemic illnesses enabling a prompt start of medication and better prognosis. Here, we present a case of a 28-year-old female patient who came to the office complaining of a „minor irritation” on the left eyebrow that was a discrete and asymptomatic 5mm infiltrated apple jelly coloured papule that had started 6 months earlier. Biopsy of a second similar lesion discovered on left forearm showed granulomatous features of sarcoidosis. Under subsequent systemic investigation, the presence of sarcoidosis in other organs was found to be positive as stage II pulmonary sarcoidosis, and also with reticulo-endothelial involvement manifested by enlarged mediastinal lymph nodes. This case highlights the skin as a mirror to internal multisystemic disease and also the importance of investigating even small and discrete lesions with care and in depth.
 
Key words:  sarcoidosis; differential diagnosis; seborrheic dermatitis

 

Introduction
Sarcoidosis is a systemic disorder that can involve almost any organ system but especially the lungs, lymph nodes, liver, spleen and eyes. Between 20% and 35% of patients with systemic sarcoidosis have various forms of skin involvement [1]. The clinical course of sarcoidosis is progressive with frequent remissions and relapses [2] Granulomatous infiltrates in the skin may present on diverse morphology of which few are specific for the disease. They are not only common but may be the initial presentation of the systemic inflammatory process and provide a visible clue to the diagnosis being also an easily accessible source of tissue for histologic examination [3]. Cutaneous sarcoidosis si considered among the “great imitators” in dermatology [4]. Erythema nodosum is the typical non-specific skin lesion and is often associated with an acute presentation and generally portends a good prognosis. Papules, plaques, nodules, lupus pernio and scar infiltration among others are the specific presentations and mostly tend to be chronic therefore treatment is mandatory [5]. Another forms of presentations and differential diagnoses include granulomatous rosácea; acne; benign appendageal tumours; psoriasis, lichen planus, nummular eczema, discoid lupus erythematosus, granuloma annulare cutaneous T-cell lymphoma, secondary syphilis, Kaposi’s sarcoma, lupus pernio discoid lupus erythematosus, scar furunculosis, cellulitis and inflammatory panniculitis [6].
 
Case Report
A 28-year-old female patient presented with a discrete and isolated 5mm infiltrated papule on the eyebrow (Fig. 1) that had started within a period of 6 months. Cutaneous examination revealed presence of also another asymptomatic infiltrated apple jelly coloured papule (Fig. 2) on the left forearm. Punch biopsy was proceeded and showed confluent non-caseating granulomas with epithelioid cells and few lymphocytes, with negative staining for tuberculosis and fungi (Fig. 3). Clinical investigation was initiated. When first asked, she replied negatively with regard to respiratory symptoms, but following further questioning, she remembered a persistent dry cough and exertional dyspnoea that had been happening over a period of some months. Her past medical history, personal, occupational and family histories were insignificant. Peripheral lymph nodes were not enlarged. Clinical examination of cardiovascular, respiratory and neurological systems were normal. The hematological and bio-chemical liver and kidney function tests were normal but urinary calcium was slightly increased.Erythrocyte sedimentation rate was normal. Contrast enhanced computed tomography of chest revealed enlarged lymph nodes in the mediastinum and involvement of the pulmonary parenchyma consistent with stage II of pulmonary sarcoidosis [7,8] (Figs 4A and B). Spirometry and abdominal ultrasound scan were normal.
 
 
Figure 1. Discrete millimetric seborreic-like eyebrow lesion.
Figure 2. After biopsy of the left forearm lesion.
Figure 3. Histopathology: confluent non-caseating granulomas with epithelioid cells and few lymphocytes. Stainings for tuberculosis and fungi were negative.
 
Figure 4 A and B. The patient had stage II pulmonary sarcoidosis as well as reticulo-endothelial system involvement manifested by enlarged mediastinal lymph nodes.
Discussion
Sarcoidosis is a multisystemic granulomatous disorder that affects primarily the lungs and lymphatic tissues. Cutaneous involvement may be the first indication of its presence in other organs. Pulmonary sarcoidosis leads to restrictive lung function impairment with a variable prognosis ranging from a self-limiting course (60%) to progressive fibrosis (10–30 %) with currently no good predictor of outcome [9]. A wide variety of morphologic forms of cutaneous sarcoidosis are possible, although many of these are extremely rare. Although no breathing issues were raised by this patient until she was asked more specifically, 90% of patients have pulmonary involvement although half of them are usually asymptomatic. Early investigation of cardiac sarcoidosis is also critical because sudden death can be the initial presentation [10]. The skin is said to mirror internal disease. Various systemic illnesses manifest in the skin and skin offers the advantage of easy access for biopsy and the possibility of early diagnosis and better prognosis. Recent over emphasis of dermatology on cosmiatric subjects may lead to lack of interest on the investigative aspects. In every dermatologist, there should be something of the detective to put the pieces together by posing the right questions at the right time, and more than that, to have an open mind for seemingly unimportant details [11,12]. Keen observation, intense inspection of the subject, attention to details and apparent trifles, so much emphasised by Sherlock Holmes, are particularly pertinent to the dermatologist. In Sherlock Holmes, the early master detective, there was something of the dermatologist [11]. The early diagnosis was of substantial benefit to the patient in that with just 40mg of prednisone [13] for a short period of time, respiratory symptoms, skin lesion and reticulo-endothelial pathological findings disappeared. This case hightlights the importance of close inspection of even tiny, otherwise unimportant lesions, that often represents just the tip of the iceberg. Also, adds seborrheic dermatitis to the extensive list of differential diagnosis of cutaneous sarcoidosis.
 
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