Gastric Cancer and Paraneoplastic Pemphigus

Abstract

Paraneoplastic pemphigus is a relatively rare but significant acquired autoimmune mucocutaneous disorder that is characterised by diffuse erythema, painful blistering and sores of the skin and mucus membranes. The underlying pathogenesis is believed to be triggered by altered immune system in response to underlying neoplasm. The manifestations can predate, occur at the same time or after the diagnosis of cancer. Associations with gastric cancer have only been reported twice. A 78-year-old lady presented with a month's history of extensive skin lesions that started off as bullous lesions and biopsy revealed bullous pemphigus. Endoscopy for anemia revealed gastric cancer. This case reinforced the need to consider underlying malignancy in elderly patient with new onset dermatological presentation. Keywords Gastric cancer . Pemphigus vulgaris . Paraneoplastic manifestations . Bullous dermatological lesionsA 78-year-old lady presented with a month's history of extensive skin lesions that started off as bullous lesions. She had anorexia of recent onset but without any significant weight loss. Physical examination revealed multiple ruptured lesions throughout body (Fig. 1a) (trunk, genitalia and the extremities) with exudative discharge and bleeding. She also had painful lesions around the lip. Laboratory investigations revealed normocytic anemia of 7.3 g/dl. A skin biopsy showed ulcerated skin lesion lined by a layer of discohesive basal cells and direct immunofluorescence study showed pericellular positive staining for IgG consistent with pemphigus vulgaris (Fig. 1b, c). An upper gastrointestinal endoscopy showed diffuse esophageal candidiasis and multiple small geographic ulcers in the antrum (type IIc+a) (Fig. 1d). The biopsies of the antral ulcers showed diffuse infiltrating signet cell adenocarcinoma. Serum anti-desmoglin 3 was positive. A diagnosis of paraneoplastic pemphigus with the gastric cancer was made. A staging computed tomography scan revealed no metastases or any other abnormalities. Despite repeated discussions, the patient declined surgical intervention. She was managed with immunosuppressive therapy with some improvement of the skin condition. Her condition worsened and she passed away almost 2 years after the initial presentation.Paraneoplastic pemphigus is a relatively rare but significant acquired autoimmune mucocutaneous disorder that is characterised by diffuse erythema, painful blistering and sores of the skin and mucus membranes. The underlying pathogenesis is believed to be triggered by altered immune system in response to underlying neoplasm. The manifestations can predate, occur at the same time or after the diagnosis of cancer. Associations with gastric cancer have only been reported twice [1,2]. Associations with other malignancies such breast, colon and bladder cancers have been reported [3]. Other paraneoplastic cutaneous manifestations associated with gastric malignancies include Leser-Trelat syndrome, palmo-plantar keratoderma, acanthosis nigricans and paraneoplastic dermato...

Paraneoplastic Pemphigus: Insight into the Autoimmune Pathogenesis, Clinical Features and Therapy

“…Gastric cancers have been rarely reported as a PNP trigger. Indeed, only three cases of PNP related to gastric cancers (1 gastric lymphoma and two adenocarcinomas) have been reported in the literature [ 23 , 24 ]. A single PNP case caused by melanoma, described as localized paraneoplastic pemphigus, has been reported [ 25 ].…”

Pemphigus: Current and Future Therapeutic Strategies

“…Up to 84% of all PNP cases are secondary to hematologic malignancies (33), including non-Hodgkin lymphomas (38.6%), chronic lymphocytic leukemia (18.4%), Castleman's disease (18.4%), thymoma (5.5%), Waldenstrom's macroglobulinemia (1.2%), Hodgkin lymphoma (0.6%), and monoclonal gammopathy (0.6%) (6, 33). Less frequently, epithelial carcinomas (8.6%), sarcomas (6.2%) and gastric cancers have been described in association with PNP (6, 33–35). Some cases of PNP have been reported to be triggered by anti-neoplastic drugs, including fludarabin and bendamustine (36) or radiotherapy (37).…”

A case report of esophageal cancer in a patient with pemphigus vulgaris: A coincidence or something beyond that?

“…Although the neoplasm is usually detected before the onset of PNP, pemphigus can be manifested before the detection of an occult tumor in 30% of cases 4,5 . PNP following gastric cancers (gastric lymphoma and adenocarcinoma) is rarely reported 6,7 . It is worthy to note that PNP might share clinical, histopathological and serological features with PV 8 .…”

“… 4 , 5 PNP following gastric cancers (gastric lymphoma and adenocarcinoma) is rarely reported. 6 , 7 It is worthy to note that PNP might share clinical, histopathological and serological features with PV. 8 Hence, we could not rule out the diagnosis of PNP for our case, though his clinical and laboratory features were in favor of the diagnosis of PV.…”