Journal of Postgraduate Medicine
 Open access journal indexed with Index Medicus & ISI's SCI  
Users online: 49828  
Home | Subscribe | Feedback | Login 
About Latest Articles Back-Issues Articlesmenu-bullet Search Instructions Online Submission Subscribe Etcetera Contact
 
  NAVIGATE Here 
  Search
 
  
 RESOURCE Links
 ::  Similar in PUBMED
 ::  Search Pubmed for
 ::  Search in Google Scholar for
 ::  Article in PDF (1,179 KB)
 ::  Citation Manager
 ::  Access Statistics
 ::  Reader Comments
 ::  Email Alert *
 ::  Add to My List *
* Registration required (free) 

  IN THIS Article
 ::  References
 ::  Article Figures

 Article Access Statistics
    Viewed532    
    Printed6    
    Emailed0    
    PDF Downloaded7    
    Comments [Add]    

Recommend this journal


 


 
  Table of Contents     
CASE SNIPPET
Year : 2021  |  Volume : 67  |  Issue : 2  |  Page : 119-121

Toxic epidermal necrolysis-like presentation of paraneoplastic pemphigus due to underlying thymoma: A clinical conundrum


1 Department of Dermatology, Government Medical College and Hospital, Chandigarh, India
2 Department of Surgery, Government Medical College and Hospital, Chandigarh, India

Date of Submission28-Jun-2020
Date of Decision08-Sep-2020
Date of Acceptance21-Jan-2021
Date of Web Publication30-Apr-2021

Correspondence Address:
M Bhalla
Department of Dermatology, Government Medical College and Hospital, Chandigarh
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jpgm.JPGM_752_20

Rights and Permissions




How to cite this article:
Poonia K, Chabra K, Dalal U, Bhalla M. Toxic epidermal necrolysis-like presentation of paraneoplastic pemphigus due to underlying thymoma: A clinical conundrum. J Postgrad Med 2021;67:119-21

How to cite this URL:
Poonia K, Chabra K, Dalal U, Bhalla M. Toxic epidermal necrolysis-like presentation of paraneoplastic pemphigus due to underlying thymoma: A clinical conundrum. J Postgrad Med [serial online] 2021 [cited 2021 Aug 1];67:119-21. Available from: https://www.jpgmonline.com/text.asp?2021/67/2/119/315371




A 35-year-old female presented with multiple painful oral ulcers and cutaneous eruptions for 7 days. Oral ulcers were associated with severe pain, difficulty in swallowing, and excessive salivation. On day 3 of illness, she noted multiple fluid filled flaccid blisters with underlying painful erythema which ruptured spontaneously and peeled off in sheets leaving behind eroded areas and hemorrhagic crusting [Figure 1]a, [Figure 1]b, [Figure 1]c. Extremities also had target lesions with central necrosis resembling erythema multiforme. She reported taking an analgesic (paracetamol) for unrelated condition before onset of lesions. Initially, clinical possibility of toxic epidermal necrolysis (TEN) was considered and supportive measures such as withdrawal of analgesic, maintenance of fluid and electrolytes, and paraffin gauge dressing. Patient was started on oral methylprednisolone (16 mg). But, there was no improvement and lesions were progressive. She underwent skin biopsy which revealed suprabasal bulla with few apoptotic keratinocytes [Figure 2]a. Acantholytic cells were seen on Tzanck smear. Nikolsky sign and bulla spread sign were positive. Direct immunofluorescence showed IgG fluorescence in intercellular space of the keratinocyte cell surfaces and C3 deposition in the basement membrane zone [Figure 2]b and [Figure 2]c. Chest X-ray film showed mediastinal widening. Computed tomography of chest showed a large well defined heterogeneously enhancing mass (of 4.5 cm) in prevascular and left paravascular region, medially extending between left common carotid artery and subclavian artery causing splaying, also abutting trachea medially and abutting arch of segment [Figure 1]d, consistent with thymoma. Based on these findings, she was diagnosed as paraneoplastic pemphigus (PNP) associated with thymoma. She was initiated on oral cyclophosphamide (50 mg once daily) and dose of oral methylprednisolone increased to 48 mg. After 3 weeks of diagnosis of underlying tumor, thymectomy was performed when patient was on 36 mg of methylprednisolone. The tumor was an encapsulated mass measuring 7 × 5.8 × 4 cm. Histopathology showed sheets of interspersed population of lymphocytes and epithelial (tumor) cells without invasion into surrounding tissues, consistent with thymoma, type B2 [Figure 1]e and [Figure 1]f. Oral cyclophosphamide was stopped after 2 months and steroids tapered over 6 months with complete resolution of skin lesions with post-inflammatory hyperpigmentation [Figure 1]g and [Figure 1]h. At 18 months of follow-up, patient is doing well and with no recurrence of symptoms.
Figure 1: (a) Oral lesions are irregularly shaped erosion, ulcer with surrounding inflammation; (b and c) Multiple fluid filled flaccid blisters with underlying erythema over the abdomen, back, extremities and face with few eroded areas and hemorrhagic crusting; (d) Computed tomography chest showing large well defined heterogeneously enhancing mass (red arrow heads) in prevascular and left paravascular region. Medially extending between left common carotid artery and subclavian artery causing their splaying, also abutting the trachea medially and abutting arch of segment; (e and f) Photo micrograph showing sheets of interspersed population of lymphocytes and epithelial (tumor) cells without invasion into the surrounding tissues {H and E stain 10× (d) and 40× (e); (g and h) Lesions healed with hyperpigmentation

Click here to view
Figure 2: (a) Photo micrograph showing suprabasal bulla with few apoptotic keratinocytes (H and E stain at 10×); (b and c) Direct immunofluorescence showed IgG fluorescence in the intercellular space of the keratinocyte cell surfaces and C3 deposition on the basement membrane zone

Click here to view


Several mechanisms have been proposed by which an underlying neoplasm induces autoimmunity that include: antitumor immune response cross-reacting with the normal epithelial proteins such as desmoglein and plakins, desmocollins inducing autoimmunity by molecular mimicry, or by epitope spreading. PNP has been described in association with both malignant as well as benign tumors.[1]

The acute presentation of symptoms along with a history of drug intake prior to the onset of lesions led to an initial clinical diagnosis of TEN. However, clinical progression of symptoms despite drug withdrawal and supportive measures prompted us for further work-up. Clinically it may be difficult to differentiate TEN from PNP as mucocutaneous lesions with hemorrhagic crusting and erythema multiforme-like lesions may be common to both entities. Histopathological features are also overlapping. Histopathological changes are variable in PNP and includes intraepidermal acantholysis, keratinocyte necrosis, and vacuolar interface dermatitis. Direct immunofluorescence shows deposition of IgG and complement in epidermal intercellular spaces and granular linear complement deposition along epidermal basement membrane zone.[1],[2]

Long-term morbidity and mortality in patients with PNP depends upon multiple factors that includes underlying malignancy, immunosuppressive therapy, and infection- associated complications.[3] In contrast to patients with malignant tumor-associated PNP, cases associated with benign tumors, such as benign thymomas and localized Castleman's disease show better prognosis when tumor is removed.[4]

Presentation of thymoma is usually asymptomatic. About half of these patients may have associated other autoimmune diseases such as myasthenia gravis, pure red cell aplasia, systemic lupus erythematosus, and Goodpasture syndrome. Several cutaneous disorders described in association with thymoma includes pemphigus vulgris, bullous pemphigoid, lichen planus, vitiligo, alopecia areata, and lupus erythematosus. However, TEN-like presentation of PNP is not commonly reported in association with thymoma. After an extensive literature search, only 10 cases of Steven Johnson syndrome/TEN-like presentation of PNP were found, most of the cases were reported in association with chronic lymphocytic leukemia.[2],[3],[5],[6] Kokubu et al. reported TEN mimicking PNP in patient of follicular lymphoma.[6] To best of our knowledge, TEN-like presentation of PNP in association with benign thymoma has never been reported.

To conclude, the present case highlights the importance of ruling out alternative diagnosis in patients with suspected TEN, with PNP being a close differential diagnosis as treatment and long term outcome is entirely different for both entities.

Declaration of patient consent

The authors certify that appropriate patient consent was obtained.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
 :: References Top

1.
Solimani F, Maglie R, Pollmann R, Schmidt T, Schmidt A, Ishii N, et al. Thymoma-associated paraneoplastic autoimmune multiorgan syndrome-from pemphigus to lichenoid dermatitis. Front Immunol 2019;10:1413.  Back to cited text no. 1
    
2.
McLarney RM, Valdes-Rodriguez RH, Isaza-Gonzalez G, Miller JH, Hsu S, Motaparthi K. Paraneoplastic pemphigus mimicking toxic epidermal necrolysis: An underdiagnosed entity? JAAD Case Rep 2018;4:67-71.  Back to cited text no. 2
    
3.
Konichi-Dias RL, Ramos AF, de Almeida Santos Yamashita ME, Cárcano CBM. Paraneoplastic pemphigus associated with chronic lymphocytic leukemia: A case report. J Med Case Reports 2018;12:252.  Back to cited text no. 3
    
4.
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. 4
    
5.
Padhiyar J, Patel N, Ninama K, Bilimoria F, Mahajan R, Gajjar T, et al. Paraneoplastic pemphigus presenting as toxic epidermal necrolysis: A case report. Nepal J Dermatol Venereol Leprol 2018;16:59-62.  Back to cited text no. 5
    
6.
Kokubu H, Nishikawa J, Kato T, Mukaisho KI, Hayashi D, Tateishi C, et al. Paraneoplastic pemphigus mimicking toxic epidermal necrolysis associated with follicular lymphoma: Possible pathological role of CD8 T cells. Acta Derm Venereol 2020;100:adv00204.  Back to cited text no. 6
    


    Figures

  [Figure 1], [Figure 2]



 

Top
Print this article  Email this article
 
Online since 12th February '04
2004 - Journal of Postgraduate Medicine
Official Publication of the Staff Society of the Seth GS Medical College and KEM Hospital, Mumbai, India
Published by Wolters Kluwer - Medknow