The paraneoplastic syndrome refers to metabolic or neuromuscular manifestations of certain malignancies, and these paraneoplastic manifestations are not attributable to direct tumor invasion or distant spread of tumor cells [4].

The paraneoplastic cutaneous syndrome is defined by two criteria: (i) the dermatosis must develop only after the development of cancer, and (ii) both dermatosis and cancer follow a parallel course in which complete removal of the cancer results in clearing of the dermatosis, while recurrence of cancer causes relapse of the dermatosis [5]. However, this definition is not without limitation, and exceptions such as pemphigus exist. Robinson et al. described rare forms of pemphigus, including paraneoplastic pemphigus in 1999 [2, 5]. Unlike paraneoplastic pemphigus, the paraneoplastic nature of bullous pemphigoid has not been accepted widely. Hence, some authors prefer to use the term pemphigoid associated with malignancies (PAM) [8].

Despite several published case reports and trials, a definite association remains controversial (Table 1). In their study involving over 1000 Japanese patients, Ogava et al. found a significantly higher incidence of malignancy in BP (5.8%) than that of controls aged over 70 years (0.6%) [13]. Similar associations are cited in Asia [14]. However, studies conducted in Sweden [15] and the USA [16] failed to prove any statistically significant association.

Table 1 Published case reports of BP associated with lung carcinoma and treatment response

Since 1960, there have been case reports of bullous pemphigoid associated with lung carcinoma, although only a few include details of treatment in the metastatic setting. Data regarding the treatment regimen used for lung cancer in elderly patients with bullous pemphigoid are further limited. A systematic review from Balestri et al. in 2015 reported a total of 33 cases of solid tumors. The most common cancers were breast and colon cancer (n = 4).

Although Bullous pemphigoid associated with malignancies has been reported to be refractory to conventional treatment, most case reports have suggested a good response to cancer treatment, including surgery and chemotherapy [8, 17, 18, 21,22,23]. Rook et al. reported the first case of PAM in 1968 [17]. The patient was a 40-year-old with advanced bronchial carcinoma with partial response to high-dose corticosteroids and who remained in remission after surgery. In 1987, Graham-Brown et al. reported another case of bronchial carcinoma in a 74-year-old man who did not respond to corticosteroid and azathioprine. However, after surgery and radiotherapy, PAM was controlled with low-dose corticosteroid [18]. Shigemori et al. reported an autopsied case of a 78-year-old man with bullous pemphigoid who had undifferentiated large cell carcinoma of the lung in 2001 [19].

Another case of a 52-year-old chronic smoker with squamous cell lung carcinoma was reported in 2014 in the Pan African Medical Journal. The patient was treated with cisplatin and Navelbine, combined with chest and cerebellar radiotherapy along with local corticosteroid therapy, resulting in regression of bullous lesions after two courses of chemotherapy [21]. Das et al. also reported a case of a 76-year-old man with squamous cell carcinoma of the lung, which showed significant improvement after treatment with cisplatin and gemcitabine [23].

Two other case reports were published in Japanese in 1986 and 1996 [2, 6] but did not include details about histopathology. Bullous pemphigoid associated with small cell lung cancer was reported in a French publication by Lakhdar et al. in 2014. The patient was a 44-year-old smoker who had marked regression of the bullous lesions with chemotherapy [20]. Safini et al. also reported a case of bullous pemphigoid associated with small cell lung cancer in a 46-year-old man. He received cisplatin and etoposide, resulting in regression of 75% of cutaneous lesions after two cycles of chemotherapy [22].

We performed a literature search on Embase, Medline, and PubMed from 1996 to 18 August 2020. Table 1 summarizes the cases reported. Our observation and literature review suggest that bullous pemphigoid associated with malignancy may be underreported. The reported association with lung cancer is rare and even more so in case of squamous cell lung cancer. Almost all the reports suggest good response to various chemotherapy regimens that include cisplatin. However, to our knowledge, this is the first case report to show that carboplatin can be used as an effective alternative in treatment of malignancy-associated bullous pemphigoid. This is even more relevant as carboplatin is more commonly used in advanced lung cancer compared with cisplatin, more so in elderly population due to better tolerability.

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