Histological findings included elongation of rete ridges and variably dilated follicular structures containing many eosinophils, neutrophils, and their debris ( fig. 1c). These inflammatory cells occasionally disrupted the follicles and invaded into the adjacent acanthotic epidermis. A direct immunofluorescence study exhibited the intercellular deposition of IgG, C3c and C3d, but not IgA or IgM, in the epidermis. An indirect immunofluorescence study using the patient's serum demonstrated intercellular deposition of IgG at 1 : 160. He had circulating antibodies to desmoglein 3 (137 index; normal, < 10), but not desmoglein 1, as assessed by enzyme-linked immunosorbent assay. Thus, the skin eruption was diagnosed as pemphigus vegetans. The presence of scrotal tongue as well as bullous and heavily verrucous skin lesion favoured the Neumann type. 2 The patient was treated with prednisolone, 20 mg daily, which flattened the verrucous lesions with pigmented sequelae and was gradually tapered to 5 mg daily. Four months after starting of the therapy, he had an episode of acute abdominal pain and was admitted to a hospital. Endoscopic and roentgenological examinations revealed that he had gastric cancer, Borrmann type I ( fig. 1d), which was histologically diagnosed as poorly differentiated adenocarcinoma. He received partial gastrectomy, but the skin eruption and scrotal tongue continued at a low level with prednisolone, 7.5 mg daily. Two months later, metastatic lesions were found in the liver. The patient died of multiple organopathy 4 months after the operation. Autopsy was not performed.The coexistence of pemphigus vegetans and internal neoplasms has been rarely reported in the literature. [3][4][5] The simultaneous occurrence of pemphigus vegetans and gastric cancer suggests that they were closely associated with each other in our patient. Even after surgical removal of gastric cancer, the remaining metastatic lesions in the liver seems to render pemphigus lesions persistent. Our patient had scrotal tongue, which is seen in nearly a half of patients with the Neumann type. 3 Scrotal tongue coexists with internal malignancies in not only pemphigus vegetans but also other disorders such as acquired hypertrichosis lanuginosa. 6 Therefore, it is considered that pemphigus vegetans, gastric cancer/liver metastasis, and scrotal tongue were causally related. It should be noted that pemphigus vegetans may be a dermadrome for internal malignancies.
References1 Lever WF. Pemphigus and Pemphigoid. Charles C Thomas, Springfield, IL, 1965: 41-45. 2 Premalatha S, Jakakumar S, Yesdian P, Thambiah AS. Cerebriform tongue-A clinical sign in pemphigus vegetans. Br J Dermatol 1981; 104: 587-591. 3 Bastiaens MT, Zwan NV, Verschueren GL, Stoof TJ, Niepoer C. Three cases of pemphigus vegetans: induction by enalapril association with internal malignancy. Int J Dermatol 1994; 33: 168-171. 4 Serwin AB, Bokiniec E, Chodynicka B. Pemphigus vegetans in a patient with lung cancer. Dermatol Online J 2005; 11: 13. 5 Ogawa H, Sakuma M, Morioka S et ...
