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Relapse of pemphigus foliaceus initiating at the site of irradiation in an elderly male with laryngeal carcinoma: Illustrating the concept of immunocompromised cutaneous district

1 Department of Dermatology, AIIMS, Raipur, Chhattisgarh, India
2 Department of Pathology and Lab Medicine, AIIMS, Raipur, Chhattisgarh, India

Date of Submission30-Jul-2020
Date of Decision30-Jul-2020
Date of Acceptance22-Feb-2021
Date of Web Publication29-Jun-2022

Correspondence Address:
Namrata Chhabra,
Department of Dermatology, AIIMS, Raipur, Chhattisgarh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijc.IJC_853_20


Radiotherapy-induced or -aggravated pemphigus is not a common occurrence with only a few cases reported so far. The radiation disrupts the local immune regulation and alters the antigenicity of keratinocytes, but the exact etiology is not clear. We report a case of an elderly man operated for laryngeal carcinoma who presented with recurrence of pemphigus foliaceus lesions starting at the irradiation site. The appearance of vesiculobullous lesions at the site of irradiation should be evaluated thoroughly to rule out immunobullous diseases.

Keywords: Pemphigus, radiotherapy, immunity Radiation can induce or aggravate autoimmune vesiculobullous diseases. It could start at the site of irradiation as a result of immunocompromised cutaneous district.

How to cite this URL:
Chhabra N, Raut S, Ganguly S, Hussain N. Relapse of pemphigus foliaceus initiating at the site of irradiation in an elderly male with laryngeal carcinoma: Illustrating the concept of immunocompromised cutaneous district. Indian J Cancer [Epub ahead of print] [cited 2022 Dec 4]. Available from:

  Introduction Top

Autoimmune bullous diseases are a rare adverse effect of radiotherapy. Most of the cases reported are associated with breast carcinoma. We report a case of an elderly man with laryngeal carcinoma, who developed lesions of pemphigus foliaceus (PF) at the site of radiotherapy.

  Case Summary Top

A 57-year-old man, a farmer, presented to our clinic with complaints of multiple fluid-filled lesions and erosions over face neck, upper trunk, and both upper limbs for last 1 month. Three and half months back, he was diagnosed with laryngeal carcinoma, for which he underwent a total laryngectomy with tracheostomy. Postsurgery, he received volumetric modulated arc therapy with a total dose of 60 Gy at 2 Gy/fraction over a period of 6 weeks. One month after completion of radiotherapy, he started developing fluid filled lesions and erosions initially restricted only to the radiation field and later spread to involve face, scalp, upper trunk, abdomen, and both arms. He gave the history of similar lesions all over the body 3 years back for which he received treatment for around 1 year with complete remission. There were no complaints of mucosal involvement. He did not have any previous documents to suggest the diagnosis or treatment. On examination, there were multiple flaccid vesicles and bullae and crusted erosions present in the above mentioned distribution, with predominant involvement around the tracheostomy site [Figure 1] and [Figure 2]. The marginal Nikolsky sign was positive. Mucosal examination did not show any significant findings. The skin biopsy from the vesicle on histological examination showed subcorneal bullae with acantholytic cells [Figure 3]. The direct immunofluorescence from the perilesional skin showed an intercellular deposit of IgG and C3. Based on these findings, a diagnosis of PF was made. The patient was started on dexamethasone cyclophosphamide pulse therapy.
Figure 1: Multiple flaccid vesicles, bullae, and crusted erosions present over lower face, neck, trunk, and upper arms with predominant involvement around the tracheostomy site

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Figure 2: Mutiple new flaccid vesicles which appeared over sites distant to the site of irradiation

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Figure 3: Skin biopsy showing subcorneal bullae with acantholytic cells and mild perivascular lymphocytic infiltrate in the dermis (H and E, ×10)

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  Discussion Top

Radiotherapy-induced or -aggravated immunobullous disease is a rare occurrence with only a few reports published in English literature.[1],[2],[3],[4],[5],[6],[7],[8],[9],[10] Approximately half of these reported cases were associated with breast carcinoma. In most of the cases, blisters were initially confined to the irradiated area followed by a variable degree of dissemination to other sites. Inadomi has reported a case of PF aggravated in an irradiated area by radiotherapy against breast cancer.[1] Vigna-Taglianti et al. reported a case of radiotherapy-induced recurrence of pemphigus vulgaris (PV) within the irradiated fields.[2] Badri et al. observed antibodies reacting with a 110-kDa antigen, which appeared following radiotherapy and was believed to induce PV.[3] Shon et al. have reported three cases of pemphigus or pemphigoid disease that occurred after radiation therapy for breast, cervical, and metastatic malignancies, respectively.[4]

The mechanism by which radiotherapy induces pemphigus is not fully understood. It is well known that irradiated or burnt skin areas undergo a destabilization of the immune control, which can lead to either a reduction of immunity (as suggested by the facilitated local occurrence of tumors and infections) or an excess of it (as implied by the possible local onset of disorders with an exaggerated immune response).[10] In other words, these areas become typical immunocompromised cutaneous district. The pathomechanisms involved in any secondary disorder occurring on irradiated or burnt skin areas may be linked to locally decreased or altered lymph flow (with dysfunction of lymph drainage) on the one hand and to fibrotic throttling or reduction of peptidergic nerve fibers (with dysfunction of neuroimmune signaling) on the other hand, resulting in a significant dysregulation of the local immune response.[10] Rucco et al. have reported that ionizing radiation may alter the antigenicity of the keratinocyte surface by disrupting the sulfhydryl groups, thus changing the immunoreactivity of the desmogleins or unmasking certain epidermal antigens.[9] Another possible explanation is immune surveillance interference by damaged T-suppressor cells, which are preferentially sensitive to radiation.[7] Robbins et al. performed immunomapping of perilesional skin for the irradiated field in a patient with radiation-induced mucocutaneous pemphigus and suggested that radiation changed either the distribution or the expression of Dsg1 in the epidermis.[8]

Although autoimmune bullous diseases are the rare adverse effect of radiotherapy, clinicians should be able to distinguish this potentially serious complication from other cutaneous eruptions developing during and after radiotherapy. The appearance of vesiculobullous lesions and erosions over the site of irradiation should be thoroughly evaluated to exclude immunobullous disease, as this is a chronic disease requiring prolonged immunosuppressives. We report this case for its unique presentation as a feature of the immunocompromised cutaneous district.

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  References Top

Inadomi T. A Case of pemphigus foliaceus aggravated in an irradiated area by radiotherapy against breast cancer. Indian J Dermatol 2015;60:93-4.  Back to cited text no. 1
[PUBMED]  [Full text]  
Vigna-Taglianti R, Russi EG, Denaro N, Numico G, Brizio R. Radiation-induced pemphigus vulgaris of the breast. Cancer Radiother 2011;15:334-7.  Back to cited text no. 2
Badri T, Hammami H, Lachkham A, Benmously-Mlika R, Mokhtar I, Fenniche S. Radiotherapy-induced pemphigus vulgaris with autoantibodies targeting a 110 kDa epidermal antigen. Int J Dermatol 2011;50:1475-9.  Back to cited text no. 3
Shon W, Wada DA, Kalaaji AN. Radiation-induced pemphigus or pemphigoid disease in 3 patients with distinct underlying malignancies. Cutis 2016;97:219-22.  Back to cited text no. 4
Ambay A, Sratman E. Ionizing radiation-induced pemphigus foliaceus. J Am Acad Dermatol 2005;54(Suppl 5):S251-2.  Back to cited text no. 5
Cianchini G, Lembo L, Colonna L, Puddu P. Pemphigus foliaceus induced by radiotherapy and response to dapsone. J Dermatol Treat 2006;17:244-6.  Back to cited text no. 6
Low GJ, Keeling JH. Ionizing radiation-induced pemphigus. Case presentations and literature review. Arch Dermatol 1990;126:1319-23.  Back to cited text no. 7
Robbins AC, Lazarova Z, Janson MM, Fairley JA. Pemphigus vulgaris presenting in a radiation portal. J Am Acad Dermatol 2007;56(Suppl 5):S82-5.  Back to cited text no. 8
Rucco V, Pisani M. Induced pemphigus. Arch Dermatol Res 1982;274:123-40.  Back to cited text no. 9
Ruocco E, Di Maio R, Caccavale S, Siano M, Lo Schiavo A. Radiation dermatitis, burns, and recall phenomena: Meaningful instances of immunocompromised district. Clin Dermatol 2014;32:660-9.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3]


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