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CASE REPORT  
Year : 2022  |  Volume : 65  |  Issue : 3  |  Page : 709-712
Systemic sarcoidosis presenting as facial palsy, granulomatous tattoo reaction and sarcoidal scar


Department of Dermatology, The Third Affiliated Hospital of Soochow University, Changzhou, Jiangsu, China

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Date of Submission07-Apr-2021
Date of Decision03-Jun-2021
Date of Acceptance09-Jun-2021
Date of Web Publication21-Jul-2022
 

   Abstract 


Cutaneous lesions are observed in approximately 25% of patients with systemic sarcoidosis. Scar sarcoidosis is a rare but peculiar cutaneous form of sarcoidosis associated with trauma, surgery, tattoos and other types of damage. We present a 32-year-old male patient with a history of unilateral facial nerve palsy, nephrolithiasis and lung involvement. A chest CT revealed multiple bilateral hilar and mediastinal lymphadenopathy and PET-CT demonstrated an inflammatory response in multiple organs and regions. Recently, the patient had developed asymptomatic papulo-nodules scattered within the areas of tattoos and previous trauma. Histopathological examination of nodules from those different areas supported the diagnosis of sarcoidosis. The lesions almost cleared after systemic therapy with oral prednisone. It is worth remembering that skin lesions in areas of tattoos and trauma may be prominent symptoms of systemic sarcoidosis. Patients with systemic sarcoidosis should avoid tattooing.

Keywords: Sarcoidosis, scar, tattoo

How to cite this article:
Yang Y, Ning J, Zhang R. Systemic sarcoidosis presenting as facial palsy, granulomatous tattoo reaction and sarcoidal scar. Indian J Pathol Microbiol 2022;65:709-12

How to cite this URL:
Yang Y, Ning J, Zhang R. Systemic sarcoidosis presenting as facial palsy, granulomatous tattoo reaction and sarcoidal scar. Indian J Pathol Microbiol [serial online] 2022 [cited 2022 Aug 15];65:709-12. Available from: https://www.ijpmonline.org/text.asp?2022/65/3/709/351599





   Introduction Top


Tattoos are increasingly popular as a permanent or semi-permanent makeup mean, accompanied by many skin and systemic diseases. Nodular tattoo reactions have been described by many reports.[1] Sarcoidosis is a granulomatous disease that can affect multiple organ systems, cutaneous lesions are observed in approximately 25% of patients with systemic sarcoidosis. Granulomatous reactions can sometimes be the only sign of cutaneous or underlying systemic sarcoidosis.[2] Scar sarcoidosis is a rare but peculiar cutaneous form of sarcoidosis associated with trauma, surgery, tattoos and other types of damage.


   Case History Top


A heavily tattooed (right upper limb and chest) 32-year-old male patient presented to our dermatological clinic due to multiple asymptomatic nodules for 2 months. Those lesions predominantly occurred on areas of black tattoos and old scars that dated back to an accidental fall that happened more than 12 years earlier. The lesions on the scars initially occurred as purplish-red papules and subsequently turned to brown nodules without itching. His tattoos were done by a professional tattooist 8 years ago. He had no prior history of tattoo reaction or flare-ups on older tattoos.

On physical examination, the patient was slightly overweight and looked strong. There was a significantly large area of black tattoos, showing landscape paintings, mainly confined to the left upper limb and left chest. Many papules and nodules were scattered on these tattoos and specifically around areas of black ink infiltration and some of them were arranged in lines [Figure 1]d and [Figure 1]e. Simultaneously, some firm fuchsia nodular lesions with various sizes and irregular borders were observed on his left elbow and knee within areas that had been previously traumatized and had scarred [Figure 1]b and [Figure 1]c. In addition, the attending doctor also found that the patient had left side lower facial paralysis [Figure 1]a, manifested by the angle of his mouth skewed to the right. However, his visual acuity, color vision and intraocular pressure were normal.
Figure 1: (a) Ptosis of the patient's left eyelid and the angle of the mouth skewed to the right, as well as midfacial flaccidity was observed. (b, c) Some firmly fuchsia nodular lesions with various sizes and irregular borders localized to the traumatic areas on his left elbow and left knee. (d, e) Multiple papules and nodules scattered on tattoo areas

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The patient had a past history of various illnesses. One year ago, the patient received “oscillating lithotripsy of kidney stones”. Five months ago, he suffered from left peripheral facial paralysis and received several kinds of irregular treatments, including taking corticosteroids and vitamin B, etc. However, the symptoms did not resolve. Owing to an intermittent cough, he was admitted to the hospital for further examination 2 months ago. A film of his chest using a contrast-enhanced computed tomography (CE-CT) [Figure 3] revealed multiple nodules and patchy ground glass opacification, multiple bilateral hilar and mediastinal lymphadenopathy in both lung fields. Examination using positron emission tomography-computed tomography (PET-CT) [Figure 4] showed multiple nodules accompanied by increased fluorodeoxyglucose (FDG) metabolism in the lungs, mediastinum, bilateral hilum, right neck (IIa region), bilateral clavicle region, lateral axilla, retroperitoneum (about T11 level), areas near iliac vessels and in bilateral groin areas. In addition, the patient had stones in his left kidney and bilateral kidney cysts. Cerebrospinal fluid analysis and serological tests, including syphilis serology, were normal. Eye examination using a slit lamp for uveitis was normal.
Figure 2: (a, b) Granulomatous inflammation of the dermis with numerous histiocytes containing tattoo pigment. (c, d) Hyperkeratosis, acanthosis, an inflammatory infiltrate with lymphocytes, histiocytes and neutrophils in the dermis, with multinucleated giant cells and vascular proliferation. a, c: H & E, ×40 magnification and b, d: H & E, ×100 magnification

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Figure 3: Multiple bilateral hilar and mediastinal lymphadenopathy, multiple nodular and patchy shadows in both lung fields. (a, b) pulmonary window image; (c, d) mediastinal window image

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Figure 4: Increased FDG uptake in multiple nodules of bilateral lungs, bihilar, mediastinal, right cervical (area II a), bilateral clavicular region, bilateral axillary, retroperitoneal (about T11 level), bilateral iliac blood vessels and bilateral inguinal region lymph nodes and the left lobe of the liver. (a) CT image; (b) PET-CT image; (c) Fusion image; (d) MP image

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At this time, the patient was examined again in our clinic. Laboratory tests, including a routine blood examination, hepatic function and blood biochemistry, were normal. Blood lipid analysis showed normal levels. Mycobacterium tuberculosis antibody and PPD test were negative. An electrocardiogram revealed sinus arrhythmia and T wave changes. B-Ultrasonic examination detected fatty liver, bilateral renal cysts and left renal calculus.

A biopsy performed on a nodule removed from the tattoo area revealed granulomatous inflammation of the dermis with numerous histiocytes containing tattoo pigment [Figure 2]a and [Figure 2]b. However, not all granulomatous inflammation in the tissue was associated with tattoo pigment. Another biopsy of a papule excised from the old scar field [Figure 2]c and [Figure 2]d revealed hyperkeratosis, acanthosis, an inflammatory infiltrate with lymphocytes, histiocytes and neutrophils in the dermis, with multinucleated giant cells and vascular proliferation. The patient was diagnosed with systemic sarcoidosis according to the clinical manifestations, the results of histopathological examination and the imaging examinations. Consequently, therapy with oral prednisone 40 mg daily was prescribed. After one month of therapy, the nodules gradually disappeared [Figure 5].
Figure 5: (a, b, c). After one month of treatment, the papules and nodules on the tattoos and previously traumatic areas were significantly improved

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   Discussion Top


Tattoos are a kind of temporary or indelible body modification, which are usually made by inserting dyes, ink and pigments, etc., into the dermal layer of the skin. The main complications related to tattoos include allergies, infections, tumors and skin disorders. Tattoo allergy is the most common complication and often occurs on small tattooed areas, mainly against red and shades of red (pink and violet).[1] The main substance in black tattoos is carbon, which is inert and has not been reported to be an active allergen in the literature. Black tattoos mainly cause papulo-nodular reactions, which are seen as markers of sarcoidosis in many published articles, although the exact mechanism involved remains unclear.[2] Some researchers have reported that substances in tattoos can induce a chronic antigenic stimulation in genetically predisposed patients, resulting in a Th1/Th2 immunity imbalance and the production of a cascade of cytokines and chemokines, which is followed by further immune cellular recruitment and activation leading eventually to the formation of sarcoid granulomas.[3]

Our patient had black tattoos covering a large area of his body surface. The papulo-nodular lesions, which were confirmed pathologically as cutaneous sarcoidosis, appeared 8 years after his tattooing. It is worth noting that some lesions were lined up, but seemed not to be due to scratching.

Further skin examination found other skin lesions on old scars resulting from an accidental fall 12 years ago. Clinically, these lesions mimicked fresh scarring hyperplasia, however, pathologically, they were consistent with scar sarcoidosis. The occurrence of scar sarcoidosis has been reported at existing scars caused by operations, trauma, tattoos, laser surgery, herpes zoster infections and so on.[4] Scar sarcoidosis is a rare condition characterized by inflammatory infiltrations and livid discoloration, which occurs in 5.4 to 13.8% of patients with cutaneous sarcoidosis and can be signs of systemic sarcoidosis.[5] The pathogenesis of scar sarcoidosis remains unknown. The sarcoid change in old scars may be due to foreign body contamination from previous trauma. Macrophages that have phagocytosed foreign bodies may release angiotensin-converting enzymes and lymphokines, which can lead to the development of granulomas. Scar sarcoidosis may result from a hypersensitivity reaction of the skin at the time of sarcoid activity elsewhere in the body.[6] Our patient developed papulo-nodular reactions in tattoos and old scars, as well as lung involvement in a short period of time, suggesting sarcoidosis activity. The chest CT scan of the patient revealed multiple bilateral hilar and mediastinal lymphadenopathy, with multiple nodular and patchy shadows in both lung fields. Simultaneously, examination using PET-CT showed an inflammatory response in multiple organs and regions.

Heerfordt-Waldenström syndrome (HWS) is a rare subacute sarcoidosis, composed of a triad of facial nerve paralysis, anterior uveitis and enlargement of the parotid glands.[7] Our patient has significant left facial paralysis, but parotid and eye symptoms were not obvious, which may have benefited from intermittent treatment. It has been reported that facial nerve palsy has a good prognosis, while multiorgan and central nervous system (CNS) involvement is associated with a poor prognosis. In addition, the patient had suffered from nephrolithiasis. Thirty-three % of patients with sarcoidosis express renal disorders. Uric acid in pulmonary sarcoidosis patients could be correlated with the probability of developing renal stones.[8]

The diagnosis of sarcoidosis is mainly based on clinical manifestation and radiologic findings, supported by histological features in the affected organ. The treatment and prognosis of sarcoidosis primarily depends on the degree of systemic involvement.[9] Therapeutic approaches include topical, intralesional and/or systemic corticosteroids, oral cytostatic drugs, chloroquine, vitamin D and thalidomide. Systemic corticosteroids are the first-line option for treating sarcoidosis, since steroids could down-regulate immune responses.[10] When the treatment goal is achieved, the steroids should be tapered off to the lowest effective and tolerated dose. Our patient was treated with oral prednisone 40 mg daily and the skin lesions in tattoos and scars decreased and some disappeared after one month.

In conclusion, if any granulomatous tattoo reaction appears, sarcoidosis should be taken into differential diagnosis and not regarded as a mere allergic reaction. Patients with inflammatory lesions at areas of old scars should be investigated for sarcoidosis. Histopathological examination of skin biopsies is necessary to confirm sarcoidosis and usually provides the correct and final diagnosis. An accurate diagnosis is in favor of prompt treatment.

The chronological link of “tattoo first – sarcoidosis after” and vice-versa is a clinical reality that cannot be denied or concluded.[1] It is recommended that patients with systemic sarcoidosis should avoid tattoos, since foreign bodies in the skin can act as a nidus for granuloma formation. The aggregation and agglomeration of black pigments in tattoos forming foreign bodies in the skin may exert an adjuvant effect that may help sensitize individuals to tissue proteins or some chemical ingredients or to metabolites of the tattoo ink, leading to autoimmune activation.

Acknowledgements

The authors are very grateful to Professor V.J. Hearing for help with the English language editing.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

The National Natural Science Foundation of China (No. 81673078) and the Science and Technology Foundation of Jiangsu, China (Grant No. BL2014036).

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Kluger N. Cutaneous complications related to tattoos: 31 cases from Finland. Dermatology 2017;233:100-9.  Back to cited text no. 1
    
2.
Sepehri M, Hutton Carlsen K, Serup J. Papulo-nodular reactions in black tattoos as markers of sarcoidosis: Study of 92 tattoo reactions from a hospital material. Dermatology 2016;232:679-86.  Back to cited text no. 2
    
3.
Wang WM, Zhu CY, Jin HZ, Zeng YP. Systemic sarcoidosis revealed by sarcoidal granulomas on eyebrow tattoos. Eur J Dermatol 2018;28:561-2.  Back to cited text no. 3
    
4.
Grema H, Greve B, Raulin C. Scar sarcoidosis--Treatment with the Q-switched ruby laser. Lasers Surg Med 2002;30:398-400.  Back to cited text no. 4
    
5.
Kim YJ, Kim YD. A case of scar sarcoidosis of the eyelid. Korean J Ophthalmol 2006;20:238-40.  Back to cited text no. 5
    
6.
Hirani S, Kulkarni S, Flowers AB. Tattoo-associated sarcoidosis. Am J Med 2020;133:1416-7.  Back to cited text no. 6
    
7.
Fraga RC, Kakizaki P, Valente NY, Portocarrero LK, Teixeira MF, Senise PF. Do you know this syndrome? Heerfordt-Waldenstrom syndrome. An Bras Dermatol 2017;92:571-2.  Back to cited text no. 7
    
8.
Yassari F, Kiani A, Taghavi K, Abdi E, Emami H, Seifi S, et al. Renal disorders in pulmonary sarcoidosis patients. Iran J Kidney Dis 2019;13:21-6.  Back to cited text no. 8
    
9.
Ramachandraiah V, Aronow W, Chandy D. Pulmonary sarcoidosis: An update. Postgrad Med 2017;129:149-58.  Back to cited text no. 9
    
10.
Mukai T, Fujiwara D, Miyake T, Fujita S, Morizane S, Morita Y. Tattoo skin reaction as a skin manifestation of systemic sarcoidosis. Mod Rheumatol Case Rep 2021;5:167-71.  Back to cited text no. 10
    

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Correspondence Address:
Ruzhi Zhang
Department of Dermatology, The Third Affiliated Hospital of Soochow University, 185 Juqian Road, Changzhou - 213003, Jiangsu
China
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijpm.ijpm_358_21

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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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