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Cutaneous Sarcoidosis

Sarcoidosis, a systemic disease distinguished by noncaseating granulomatous inflammation of various tissues, can present with cutaneous manifestations. Various skin lesions - papules, pustules, plaques, macules, etc., including erythema nodosum and lupus pernio, may constitute the clinical presentation of cutaneous sarcoidosis. A working hypothesis, laboratory tests, identification of additional signs and symptoms, and biopsy of the skin are essential steps in making the diagnosis.


Presentation

Sarcoidosis, a multisystemic disease of unknown etiology, may involve virtually any organ in the body, including the lungs, lymph nodes, liver, spleen, eyes, etc. [1] [2] [3]. In approximately 25-35% of patients, it can cause skin-related symptoms, in which case the term cutaneous sarcoidosis is used [3] [4] [5]. Furthermore, reports show that cutaneous signs of the disease are the only manifestation in 3-5% of patients [4] [5]. Numerous skin lesions have been reported in the literature, but the two most common and most important are [3] [6]:

  • Erythema nodosum - Despite its nonspecific nature (may be seen in drug-induced, infection-related, or inflammation-induced hypersensitivity reactions as well), the sudden appearance of erythematous nodules in the subcutaneous tissues of the anterior tibia is a rather common cutaneous sign of sarcoidosis [6]. The nodules are very tender on palpation and are often accompanied by fever, joint pain, and malaise [6].
  • Lupus pernio - The presence of purple-to-red indurated papules, plaques, or nodules on the face (the alar rim of the nose and surrounding area) is a more specific feature of cutaneous sarcoidosis, and is more frequently seen in women [3] [6]. Telangiectasias can also develop together with lupus pernio [5].

In addition to erythema nodosum and lupus pernio, single or multiple papules or plaques of different sizes and colors (red, brown, hypopigmented or even translucent) developing on the face and the extremities, either as isolated lesions or in a confluent pattern are other notable manifestations of this disease [2] [6]. In some cases, the term is used to denote the appearance of the lesion in a preexisting scar (scar sarcoidosis) [6].

Papule
  • Various skin lesions - papules, pustules, plaques, macules, etc., including erythema nodosum and lupus pernio, may constitute the clinical presentation of cutaneous sarcoidosis.[symptoma.com]
  • It is usually diagnosed in darkly pigmented races and may be the earliest sign of sarcoidosis . [2] : 709 Papular sarcoid is a cutaneous condition characterized by papules , which are the most common morphology of cutaneous sarcoidosis . [2] : 708 Ichthyosiform[en.wikipedia.org]
  • We report the case of a patient with rheumatoid arthritis who, after 2 months of treatment with etanercept, showed disseminated asymptomatic violaceous papules.[ncbi.nlm.nih.gov]
  • The lesions were skin colored, erythematous, violaceous, lichenoid, shiny papules and plaques. Some of the papules were umbilicated. The papules ranged in size from 2mm to 5mm.[e-ijd.org]
  • The most common types of specific lesions are papules. They commonly occur on the face, but may also occur anywhere on the body. While only a few papules may be noted, multiple scattered or confluent lesions are more common.[indianjrheumatol.com]
Skin Plaque
  • Skin plaques and lupus pernio are associated with chronic sarcoidosis.[ncbi.nlm.nih.gov]
  • Laser surgery has been used in treating disfiguring skin plaques and lupus pernio. Success in individual cases has been reported with allopurinol, isotretinoin , leflunomide , pentoxifylline, infliximab and thalidomide .[dermnetnz.org]
  • . • Total no. of patients : 516 • Cutaneous involvement : 170 (32.9%) n % Parenchymal involvement, n % Erythema nodosum 106 (20.5) 11 (10.4) Skin plaques 22 (4.3) 4 (18.2) Subcutaneous nodules 22 (4.3) 4 (18.2) Maculopapular rash 19 (3.7) 2 (10.5) Scar[slideshare.net]
  • Zoom Skin plaques have a similar frequency to papules. They consist of one or multiple round or oval infiltrated patches, brownish red in colour, and may be due to a confluence of papules.[em-consulte.com]

Workup

As cutaneous sarcoidosis possesses numerous types of lesions, it might be difficult to establish a diagnosis solely on clinical criteria. Nevertheless, the initial suspicion towards this inflammatory disease can only be raised after a thorough physical examination and a meticulously obtained patient history [4] [6]. This condition often (but not always) precedes involvement of other organs and systemic symptoms, thus the detection of associated lesions is perhaps the only tool in making a presumptive diagnosis. Given the systemic nature of the disease, a thorough laboratory workup should be performed, comprising liver and kidney function tests, serum inflammatory markers, and angiotensin-converting enzyme (ACE) [1] [6]. In addition, chest radiography, pulmonary function tests, electrocardiography (ECG), and a complete ophthalmologic exam are recommended in the workup [6]. To confirm the diagnosis of cutaneous sarcoidosis, however, biopsy of the skin with subsequent histopathological examination is the gold standard [1]. A punch biopsy allows a satisfying amount of tissue to be obtained, and the presence of non-caseating granulomas is virtually a pathognomonic finding of sarcoidosis [1] [5].

Treatment

  • Adalimumab, at the dose and duration of treatment used in this study, is likely to be an effective and relatively safe suppressive treatment for cutaneous sarcoidosis.[ncbi.nlm.nih.gov]
  • Cutaneous sarcoidosis is a rare skin disease and many aspects of the disease presentation and treatment are not well understood.[ncbi.nlm.nih.gov]
  • Treatment modalities included steroid, hydroxychloroquine, cyclosporine, topical tacrolimus, and pulsed dye laser.[ncbi.nlm.nih.gov]
  • Treatment of these lesions is a challenge since they do not respond well to conventional treatments.[ncbi.nlm.nih.gov]
  • We report the third case of pulmonary and cutaneous sarcoidosis in the course of interferon alfa treatment for melanoma. Most cases of sarcoidosis have been reported during treatment of chronic hepatitis C.[ncbi.nlm.nih.gov]

Prognosis

  • Erythema nodosum is the most common nonspecific skin manifestation, and it portends a good prognosis. Specific skin lesions have a varied clinical appearance, although often they can be distinguished by their yellow translucent character.[ncbi.nlm.nih.gov]
  • The prognosis is good with discontinuation of treatment. Other than interferon therapy, sarcoidosis or granulomatosis reactions rarely have been reported in malignant melanoma.[ncbi.nlm.nih.gov]
  • Future studies that clearly define the etiology of sarcoid will lead to better therapies and a better prognosis for affected patients.[ncbi.nlm.nih.gov]
  • Types of cutaneous lesions and existence of systemic involvement are related to prognosis of cutaneous lesions.[ncbi.nlm.nih.gov]
  • Prognosis of cutaneous sarcoidosis was favourable, as over 90% of patients had a good response to either glucocorticoids, hydroxychloroquine or tetracycline antibiotics.[ncbi.nlm.nih.gov]

Etiology

  • The identified TLR expression in cutaneous sarcoidosis indicates that a bacterial antigen could be an etiologic agent of the disease.[ncbi.nlm.nih.gov]
  • Sarcoidosis and Crohn disease (CD) are granulomatous disorders of unknown etiology that are rarely seen together in one patient.[ncbi.nlm.nih.gov]
  • Sarcoidosis is a systemic granulomatous disease of unknown etiology that affects multiple organ systems, including the pulmonary, lymphatic, skeletal, and integumentary systems.[ncbi.nlm.nih.gov]
  • Special stains for infectious etiologies were negative. These findings were compatible with morphologic and clinical features seen in sarcoidosislike lesion induced by interferon.[ncbi.nlm.nih.gov]
  • Sarcoidosis is a multi-system granulomatous disease of unknown etiology. The skin is involved in 25% of cases. Studies on cutaneous sarcoidosis from our region are lacking.[ncbi.nlm.nih.gov]

Epidemiology

  • This review will highlight developments in the epidemiology, clinical presentation, diagnosis and treatment of cutaneous sarcoidosis over the past several years.[ncbi.nlm.nih.gov]
  • The epidemiology of cutaneous sarcoidosis is not well-characterized as only referral-based studies are available.[ncbi.nlm.nih.gov]
  • Hywel Williams Professor of Dermato-Epidemiology, Nottingham UK. Professor Williams is coordinating editor of the Cochrane Skin Group.[books.google.com]
Sex distribution
Age distribution

Pathophysiology

  • While the pathophysiology is unclear, both prior research and our findings suggest that not only does sarcoidosis affect blacks more often than whites, blacks are also more likely to develop cutaneous disease.[austinpublishinggroup.com]
  • Further investigation may provide valuable insight into the immune-pathophysiology behind these diseases and ultimately aid in new therapeutic advances.[omicsonline.org]
  • The exact mechanism of this relation is not known, but some evidence supports the hypothesis that this is a consequence of Th1 lymphokine prevalence. [55] [83] Tests of delayed cutaneous hypersensitivity have been used to measure progression. [84] Pathophysiology[en.wikipedia.org]

Prevention

  • Early diagnosis and systemic treatment could prevent future complications. A series of three cases is presented in which the initial diagnosis was cutaneous sarcoidosis, but it was actually the first manifestation of a systemic disease.[ncbi.nlm.nih.gov]
  • The knowledge of the adaptive immunological mechanisms operative in cutaneous sarcoidosis may subsequently be useful for identifying prevention and treatment strategies of systemic sarcoidosis.[ncbi.nlm.nih.gov]
  • This helps relieve symptoms and prevent damage to affected organs by reducing inflammation and preventing scarring. However, steroid tablets can cause unpleasant side effects such as weight gain and mood swings if taken in high doses.[nhs.uk]
  • Prevention Although sarcoidosis cannot be prevented, some preventative measures may reduce complications. For example: Influenza vaccination. Osteoporosis prophylaxis (steroid usage).[patient.info]

References

Article

  1. Yanardag H, Tetikkurt C, Bilir M, Demirci S, Iscimen A. Diagnosis of cutaneous sarcoidosis; clinical and the prognostic significance of skin lesions. Multidiscip Respir Med. 2013;8(1):26.
  2. Dybiec E, Pietrzak A, Bartosińska J, Kieszko R, Kanitakis J. Ultrasound findings in cutaneous sarcoidosis. Postepy Dermatol Alergol. 2015;32(1):51-55.
  3. Haddad N, de Oliveira J, Nasser K da R, Corbett AMF, Tebet ACF, Reis MLJ. Musculoskeletal and cutaneous sarcoidosis: exuberant case report. An Bras Dermatol. 2014;89(4):660-662.
  4. Tong C, Zhang X, Dong J, He Y. Comparison of cutaneous sarcoidosis with systemic sarcoidosis: a retrospective analysis. Int J Clin Exp Pathol. 2014;7(1):372-377.
  5. Kumar HKS, Gayathri MN, Bharathi M, Ravikumar T. Isolated Cutaneous Sarcoidosis: A New Insight into the Old Entity. J Clin Diagn Res. 2013;7(8):1725-1726.
  6. Katta R. Cutaneous sarcoidosis: a dermatologic masquerader. Am Fam Physician. 2002;65(8):1581-1584.

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Last updated: 2018-06-22 02:00