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Year : 2018  |  Volume : 6  |  Issue : 1  |  Page : 25-28

Resolution of paraneoplastic skin lesion after thymectomy

1 Division of Thoracic Surgery, Department of Surgery, Faculty of Medicine, King Abdulaziz University, Jeddah, Saudi Arabia
2 Department of Surgery, Faculty of Medicine, King Abdulaziz University, Jeddah, Saudi Arabia
3 Specialized Surgical Unit, Department of Surgery, King Abdullah Medical City, Makkah, Saudi Arabia
4 Division of Thoracic Surgery, Department of Surgery, Faculty of Medicine, King Abdulaziz University, Jeddah; Specialized Surgical Unit, Department of Surgery, King Abdullah Medical City, Makkah, Saudi Arabia

Date of Web Publication27-Feb-2018

Correspondence Address:
Dr. Ashraf A Maghrabi
Assistance Professor of Surgery Consultant Thoracic and Foregut Minimally Invasive Surgeon, Division of Thoracic Surgery, Department of Surgery, faculty of Medicine, King Abdulaziz university, Jeddah
Saudi Arabia
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ssj.ssj_46_17

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Thymoma is the most common primary mediastinal neoplasm. It is well known to have a strong association with autoimmune diseases, but associated cutaneous disorders are considerably rare presentation of thymoma as paraneoplastic manifestation. Thymectomy is an optional treatment in some autoimmune diseases such as myasthenia gravis. We describe a 51-year-old male with a history of thymoma for 6 years accompanied by generalized severe purpuric rash, which was completely resolved after thymectomy.

Keywords: Lichen planus, paraneoplastic skin lesion, pemphigus vulgaris, thymectomy, thymoma

How to cite this article:
Habeebullah AB, Kaaki A, Aljiffry M, Hamid T, Maghrabi AA. Resolution of paraneoplastic skin lesion after thymectomy. Saudi Surg J 2018;6:25-8

How to cite this URL:
Habeebullah AB, Kaaki A, Aljiffry M, Hamid T, Maghrabi AA. Resolution of paraneoplastic skin lesion after thymectomy. Saudi Surg J [serial online] 2018 [cited 2022 Aug 16];6:25-8. Available from: https://www.saudisurgj.org/text.asp?2018/6/1/25/226230

  Introduction Top

Thymoma is the most common primary mediastinal neoplasm, and it is well known to have a strong association with autoimmune disorders including myasthenia gravis (MG), pure red cell aplasia, Syndrome of Inappropriate Antidiuretic Hormone release (SIADH), and systemic lupus erythematosus. Paraneoplastic skin disorders (PSDs) are considered rare associations of thymoma.[1] Thymectomy is an optional treatment in some autoimmune diseases such as MG.[2] In recent case studies, the resection of thymoma resulted in regression of paraneoplastic skin lesions such as bullous dermatoses and oral lichen planus.[3],[4]

We report a case of thymoma accompanied by generalized severe purpuric rash that was completely resolved after transsternal complete thymectomy.

  Case Report Top

A 51-year-old Indian male presented to our emergency department (ED) with a history of shortness of the breath for 2 weeks and was admitted with a provisional diagnosis of pneumonia. His past medical history was significant for hypertension and thymoma that was diagnosed 6 years back in India. Physical examination revealed bilateral decreased breath sounds. The patient was managed for pneumonia and discharged in good condition with planned follow-up with thoracic surgeon to assess his thymoma for operative intervention.

Three weeks later, the patient presented to the thoracic surgery service for management of his thymoma. His physical examination was unremarkable after full course of antibiotics.

Laboratory data including lactate dehydrogenase, alpha-fetoprotein, beta HCG and anticholinesterase antibodies, and carcinoembryonic antigen were all within normal range. The patient was prepared for transsternal thymectomy. Pulmonary function tests showed evidence of mild obstructive pattern. Echocardiogram showed mild pericardial thickening without obvious pericardial effusion or myocardial involvement. Chest radiograph showed a well-defined, round anterior mediastinal mass [Figure 1]a. Computed tomography (CT) chest without contrast was done as the patient was known to have allergy to contrast and showed anterior left side mass with pericardial attachment [Figure 1]b. A positron emission tomography with CT (PET/CT) scans for the whole body revealed a left anterior mediastinal mass with low-grade metabolism. There was no extramediastinal involvement [Figure 2].
Figure 1: (a) Posteroanterior chest radiograph. (b) Computed tomography of the chest without contrast displays anterior mediastinal mass with calcifications

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Figure 2: Positron emission tomography/computed tomography showed large nonfludeoxyglucose-avid anterior mediastinal tumor

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While waiting for his elective admission for thymectomy procedure, the patient presented to the ED with a history of palpitation and a heart rate of 98 b/min with O2 saturation 95% on room air. He was afebrile and admitted as a case of anterior mediastinal mass. During his hospital stay, he developed severe generalized itchy purpuric rash with eczematous patches, involving the upper and lower limbs as well as anterior chest wall. There was no oral or mucosal lesion and no history of new medication or herbs ingestion. A dermatologist evaluated the patient and oral prednisolone was started empirically. Skin biopsy from the thigh showed superficial perivascular inflammatory infiltrate, and the capillaries showed endothelial reactive changes. There was no fibrinous necrosis or nuclear dust. The epidermis was also focally involved by lymphocyte infiltration and dyskeratosis. There was no evidence of malignancy. [Figure 3]a shows a final diagnosis of eosinophilic vasculitis with superficial perivascular mixed inflammatory infiltrate. Pulmonology and anesthesia cleared the patient for surgery but it was postponed due to the severe skin lesions and prednisolone treatment, and the patient was discharged home.
Figure 3: (a) Skin biopsy of the thigh lesion shows eosinophilic vasculitis with superficial perivascular mixed inflammatory infiltrate. (b) Thymoma with focal partial invasion of the capsule

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Few weeks later, the patient underwent median sternotomy for total thymectomy along with wedge resection of the lingula that was attached to the mass. Histopathological examination revealed encapsulated thymoma with focal partial invasion of the capsule (WHO class type A). The attached lung tissue and pericardial fat were free of invasion, and all surgical margins were negative [Figure 3]b. Postoperative course was uneventual. Patient discharged home in good condition. The last follow up visit was at two years post operative were the patient continue to be symptoms free with no evidence of cutaneous lesions nor evidence of recurrence of thymoma in CT chest.

  Discussion Top

Thymus is a central lymphatic organ that is responsible for many immunological functions. Thymoma is the most common mediastinal tumors and accounts for 20%–25% of all mediastinal tumors and 50% of anterior mediastinal ones.[5] Thymoma is usually associated with autoimmune disorders such as MG, pure red cell aplasia, SIADH, and systemic lupus erythematosus.[1] Although the exact mechanisms by which thymoma induces autoreactivity is not yet fully understood, the association between them appears to be strong and not coincidental. Thymectomy was found to be beneficial to patients with certain autoimmune disorders such as MG. The most probable explanation for autoimmunity related to the thymoma is that the damage triggered by tumor growth within the thymus decreases its ability to maintain self-tolerance and opens a “window of opportunity” for autoimmune diseases to develop. Understanding these mechanisms might not only guide us in managing these patients but could also improve our understanding of autoimmunity and our ability to prevent these disorders.[5]

PSD is considered to be a rare paraneoplastic manifestation of thymoma. In 1987, Gibson and Muller in their retrospective study of 172 patients with thymoma found that fungal mucocutaneous disease was the most common thymoma-associated cutaneous disorder. Two patients in that study were noted to have pemphigus, and another two had lichen planus.[6] After serial analysis of various case reports, we found that thymectomy for PSD was controversial, and the clinical outcome varied. In Gibson and Muller's study, thymectomy did not alter the clinical course with respect to cutaneous diseases.[6] However, considering the report by Yoshida et al.[3] and Takeshita et al.,[1] thymectomy was performed in 11 patients with pemphigus and thymoma. Pemphigus had improved in 5 patients (45.5%) while 2 patients got worse and 2 patients died because of severe infections. One patient out of the eleven showed no changes in symptoms and the other lost follow up.

Another Japanese study by Takeshita et al. showed that thymectomy resulted in improvement of pemphigus in only 3 out of 18 patients (16.7%), while 12/18 patients showed deterioration of pemphigus and 3 showed no changes.[1]

On reviewing the English literature, we found only 5 reported cases, where thymectomy resulted in regression of bullous dermatoses and lichen planus. In most reported cases, total thymectomy resulted in reduced serum antibodies index value including anti-desmoglein 3 (anti-Dsg3) and anti-Dsg1 antibody, which are diagnostic tests for pemphigus [Table 1].[4],[7],[8]
Table 1: Reported cases of thymoma with paraneoplastic skin disorder that improved following thymectomy in the English literature

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We think that the variable outcome maybe related to the operative techniques and management strategies. Currently, there is no strong data to support whether PSD is improved or aggravated following thymectomy for thymoma-associated cutaneous disorders.

  Conclusion Top

Our case provided a further evidence of the relationship between thymoma and PSD and the rule of thymoma resection in managing such conditions. We therefore recommend further studies to elucidate the link between these conditions.

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


Conflicts of interest

There are no conflicts of interest.

  References Top

Takeshita K, Amano M, Shimizu T, Oyamada Y, Abiko T, Kobayashi K, et al. Thymoma with pemphigus foliaceus. Intern Med 2000;39:742-7.  Back to cited text no. 1
Wolfe GI, Kaminski HJ, Aban IB, Minisman G, Kuo HC, Marx A, et al. Randomized trial of thymectomy in myasthenia gravis. N Engl J Med 2016;375:511-22.  Back to cited text no. 2
Yoshida M, Miyoshi T, Sakiyama S, Kondo K, Tangoku A. Pemphigus with thymoma improved by thymectomy: Report of a case. Surg Today 2013;43:806-8.  Back to cited text no. 3
Bobbio A, Vescovi P, Ampollini L, Rusca M. Oral erosive lichen planus regression after thymoma resection. Ann Thorac Surg 2007;83:1197-9.  Back to cited text no. 4
Shelly S, Agmon-Levin N, Altman A, Shoenfeld Y. Thymoma and autoimmunity. Cell Mol Immunol 2011;8:199-202.  Back to cited text no. 5
Gibson LE, Muller SA. Dermatologic disorders in patients with thymoma. Acta Derm Venereol 1987;67:351-6.  Back to cited text no. 6
Barbetakis N, Samanidis G, Paliouras D, Boukovinas I, Asteriou C, Stergiou E, et al. Paraneoplastic pemphigus regression after thymoma resection. World J Surg Oncol 2008;6:83.  Back to cited text no. 7
Hartz RS, Daroca PJ. Clinical-pathologic conference: Cutaneous paraneoplastic pemphigus associated with benign encapsulated thymoma. J Thorac Cardiovasc Surg 2003;125:400-6.  Back to cited text no. 8


  [Figure 1], [Figure 2], [Figure 3]

  [Table 1]


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