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CLINICAL IMAGE
Year : 2015  |  Volume : 141  |  Issue : 1  |  Page : 132-133

Lichenoid skin lesions & mucosal erosions as a paraneoplastic syndrome


Department of Dermatology, Venerology & Leprology, Postgraduate Institute of Medical, Education & Research, Chandigarh 160 012, India

Date of Web Publication2-Apr-2015

Correspondence Address:
Amrinder J Kanwar
Department of Dermatology, Venerology & Leprology, Postgraduate Institute of Medical, Education & Research, Chandigarh 160 012
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-5916.154524

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How to cite this article:
Vinay K, Kanwar AJ. Lichenoid skin lesions & mucosal erosions as a paraneoplastic syndrome. Indian J Med Res 2015;141:132-3

How to cite this URL:
Vinay K, Kanwar AJ. Lichenoid skin lesions & mucosal erosions as a paraneoplastic syndrome. Indian J Med Res [serial online] 2015 [cited 2019 Jun 19];141:132-3. Available from: http://www.ijmr.org.in/text.asp?2015/141/1/132/154524

A 65 year old male, a known case of chronic lymphocytic leukemia, presented to the department of Dermatology, v0 enereology and l0 eprology, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India in January 2012 with a three months history of painful oral and genital erosions, conjunctival congestion and hoarseness of voice. Simultaneously he developed multiple flaccid vesicles and crops of itchy, violaceous, mildly scaly papules and plaques over face, trunk and upper extremities ([Figure 1]). Skin biopsy showed upper dermal lichenoid infiltrate and necrotic keratinocytes. Direct immunofluorescence (IF) of perilesional skin showed cell surface and basement membrane zone deposits of IgG ([Figure 2]a). Intercellular IgG deposits were also demonstrated by indirect IF using normal human skin section (1:40; not in [Figure 2]b) and rat bladder transitional epithelium (1:10) ([Figure 2]b). On immunoblotting, patient's serum reacted with the 210 kDa envoplakin and 190 kDa periplakin. A diagnosis of paraneoplastic pemphigus (PNP) was made and the patient was treated with oral corticosteroids and intravenous rituximab but had poor response to therapy. The patient died of uncontrolled disease three months after the initial presentation.
Figure 1. Violaceous, mildly scaly, discrete to confluent papules and plaque over the back.

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Figure. 2 (a). Direct immunofluorescence of perilesional skin section showing IgG deposits on cell surface (star) and basement membrane zone (arrow). The arrow (→) points to a obliquely cut dermal papillae. (b). Indirect immunofluorescence of rat bladder section, showing positive IgG reactivity with the transitional epithelium over 1:10 titre (both arrows) 400x.

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PNP is a rare paraneoplastic dermatosis usually seen in association with haematological malignancies [1] . The cutaneous manifestations vary form a spectrum of at least five different clinical and immunopathological variants [2] . PNP differs clinically from classical

pemphigus by presence of intractable stomatitis, inflammatory lesions in association with blisters and association with internal malignancy. In nearly one third of cases, PNP precedes the diagnosis of internal malignancy and acts as a cutaneous marker [3] . Conversely, a diagnosis of PNP should always be considered in patients with known haematological malignancies, who develop severe, intractable mucosal erosions or cutaneous blisters and lichenoid plaques.

 
   References Top

1.
Kaplan I, Hodak E, Ackerman L, Mimouni D, Anhalt GJ, Calderon S. Neoplasms associated with paraneoplastic pemphigus: a review with emphasis on non-hematologic malignancy and oral mucosal manifestations. Oral Oncol 2004; 40 : 553-62.  Back to cited text no. 1
    
2.
Nguyen VT, Ndoye A, Bassler KD, Shultz LD, Shields MC, Ruben BS, et al. Classification, clinical manifestations, and immunopathological mechanisms of the epithelial variant of paraneoplastic autoimmune multiorgan syndrome: a reappraisal of paraneoplastic pemphigus. Arch Dermatol 2001; 137 : 193-206.  Back to cited text no. 2
    
3.
Sehgal VN, Srivastava G. Paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome. Int J Dermatol 2009; 48 : 162-9.  Back to cited text no. 3
    


    Figures

  [Figure 1], [Figure 2]



 

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