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].
Immune System
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Cervical Lymphadenopathy
There were no annular lesions, nodules, crusting, pustules, telangiectasia or cervical lymphadenopathy. Rest of the cutaneous examination was unremarkable. [aclr.com.es]
Entire Body System
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Plethora
Cutaneous symptoms have been reported with polycythemia vera, including facial plethora, aquagenic pruritus, urticaria, purpura, Sweet's syndrome and pyoderma gangrenosum. [ncbi.nlm.nih.gov]
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Military Personnel
In Japanese persons, ophthalmologic and cardiac involvement are more common than in other races. [18] It is more common in certain occupations, namely firefighters, educators, military personnel, persons who work in industries where pesticides are used [en.wikipedia.org]
Respiratoric
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Nasal Ulcer
Lesions enlarge and become confluent to form progressively disfiguring nodular plaques on nose and adjacent cheeks Can involve upper respiratory tract and cause nasal ulceration, obstruction, and perforation of nasal septum Rarely involve dorsal [slideshare.net]
Skin
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Papule
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]
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]
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. In some areas, the papules had coalesced into linear streaks [Figure 1]. [e-ijd.org]
Papules and nodular lesions, such as these, are common and can be found anywhere on the body. Papules are often multiple while nodules are often solitary. LUPUS PERNIO LUPUS PERNIO Lupus pernio is characteristic of chronic sarcoidosis. [meddean.luc.edu]
Cutaneous examination revealed multiple, discrete, erythematous papules [Figure 1] along with flat-topped, violaceous papules and plaques, morphologically classical of lichen planus [Figure 2] lesions over the face, forearm, abdomen and leg. [ncbi.nlm.nih.gov]
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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. [sarcoidosisnews.com]
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]
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
Treatment modalities included steroid, hydroxychloroquine, cyclosporine, topical tacrolimus, and pulsed dye laser. [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]
Granulomatous cutaneous sarcoidosis: diagnosis, relationship to systemic disease, prognosis and treatment. SVDLD [Internet]. 17Dec.2013 [cited 14May2019];30(4):268-81. Available from: [mattioli1885journals.com]
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]
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]
Hywel Williams Professor of Dermato-Epidemiology, Nottingham UK. Professor Williams is coordinating editor of the Cochrane Skin Group. [books.google.com]
Pathophysiology
Internal Medicine (643) Pathology and Forensic Medicine (492) Paediatrics, Neonatology (487) Physiology and Pathophysiology (406) Infectology (342) Medical Chemistry and Biochemistry (325) Microbiology (287) Cardiology, Angiology (253) Surgery, Traumatology [portal.mefanet.cz]
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]
Pathophysiology of Baroreflex Failure In baroreflex failure, there is interrupted afferent baroreflex input, as well as efferent parasympathetic output. [ncbi.nlm.nih.gov]
Prevention
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]
Prevent hand-foot-and-mouth disease It’s contagious, but you can reduce your child’s risk of catching it. Find out what helps. Scabies? Treat everyone! If your child develops scabies, everyone in your household will need treatment. [aad.org]
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]
References
- 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.
- Dybiec E, Pietrzak A, Bartosińska J, Kieszko R, Kanitakis J. Ultrasound findings in cutaneous sarcoidosis. Postepy Dermatol Alergol. 2015;32(1):51-55.
- 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.
- 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.
- 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.
- Katta R. Cutaneous sarcoidosis: a dermatologic masquerader. Am Fam Physician. 2002;65(8):1581-1584.