Contact pemphigus

Antonio Vozza, Vincenzo Ruocco, Sarah Brenner, Ronni Wolf

Research output: Contribution to journalArticlepeer-review


In June 1990, a 23-year-old farmer presented with a 2-month history of bullous and eroded lesions that initially appeared in the umbilical region, on palms and soles, and then involved the scalp, trunk, and the oral and genital mucosae. He had no personal or family history of blistering diseases. About 3 weeks before the outbreak of the first bullae, the patient had had an unintended cutaneous contact with 1,3-dichloropropene (E+Z isomers), a soil fumigant that he periodically used as a field pesticide. In particular, some of the chemical that he was injecting into the terrain accidentally spilled on his palms and on the lower part of the vest over his abdominal region. He did not immediately wash off the liquid from his skin, as suggested by the instruction manual in case of an accident, but only wiped the wet skin regions with a towel. Hours later, a burning sensation and redness were noticed on the palms and the umbilical region, but these symptoms resolved in a few days. After 1 week, the patient complained of itching vesicles on his palms and umbilical and plantar regions. A topical steroid cleared up the vesicles, diminished, but did not eliminate, the pruritus that persisted for about 2 weeks, when crops of vesicles and blisters appeared on these regions and then affected other sites of the skin and external mucosae. On examination, large bullae and some erosions were present on his palms (Fig. 1), soles, and trunk. Tense vesicles and erosions were present on his wrists and scalp. Eroded, painful areas involved the oral and genital mucosa. Routine physical examination and biochemical tests were unremarkable. The histologic examination of a biopsy specimen from a fresh vesicle showed an intraepidermal bulla with acantholytic cells and suprabasal splitting. No eosinophilic spongiosis or keratinocyte necrosis were detected on the lesional and perilesional skin. A mild lymphocytic perivascular infiltrate was present in the papillary dermis. Direct immunofluorescence of the perilesional skin revealed deposition of IgG and C3 in the intercellular spaces of the epidermis (Fig. 2). Indirect immunofluorescence (IIF) using monkey esophagus as substrate, was positive for intercellular antibodies at a titer of 1:160. Two patch-tests were performed with 1,3-dichloropropene in acetone diluted 1:10 and 1:5, respectively. The first patch (dilution 1:10) was removed after 24 hours: it was negative and the skin site showed no further reaction until the next day, when a mild erythema appeared. The second patch (dilution 1:5) was removed after 48 hours: this was positive, showing erythema, edema, and microvesiculation. A Tzanck test, done at the site of the patch vesicles, revealed a mixture of lymphocytes, 'tadpole,' and acantholytic cells. The patient was hospitalized with the diagnosis of pemphigus vulgaris and treated with oral prednisone, 75 mg per day, cyclophosphamide, 100 mg per day, and topical skin care. The blistering stopped within a few days, and reepithelialization of both skin and mucosae occurred gradually over the next several weeks. The serum antibody titer decreased to 1:20 after 40 days of therapy. The prednisone and cyclophosphamide dosages were gradually tapered. After 2 months, the patient was discharged, receiving prednisone, 10 mg every other day, and cyclophosphamide, 50 mg per week. He was instructed to avoid any exposure to pesticides. In the next months he remained lesion-free and the treatments were discontinued in December 1990. He resumed his work and never returned for the recommended periodic check-ups. In June 1994, the patient was referred to us for dyshidrotic eczema restricted to his palms. He told us that over the past 3 years he had avoided contact with any pesticide and remained free of skin or mucosal lesions. After his informed consent, the Gruppo Italiano Ricerca Dermatit da Contatto e Ambientali (GIRDCA) standard series of patch tests and two additional patch tests with 1,3-dichloropropene (1:10 and 1:5, respectively, acetone-diluted) were mounted and removed after 48 hours. Apart from a weakly positive reaction to nickel sulfate, all other tests were negative, including those with 1,3-dichloropropene. Intercellular antibodies in his serum were positive up to a titer of 1:20. HLA-typing revealed the following haplotype: A2, -; B35, 57; Bw6, -; Cw4, -; DR8, 14; DQ1, 7. Topical treatment with a steroid-plus-salicylic acid ointment cleared up all dyshidrotic lesions in a week. A low nickel diet was recommended.

Original languageEnglish
Pages (from-to)199-201
Number of pages3
JournalInternational Journal of Dermatology
Issue number3
StatePublished - Mar 1996


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