PYODERMA VEGETANS WITH lg A DEFICIENCY.

A 41-year-old male with known congenital IgA deficiency developed pyoderma vegetans during a subtropical holiday. He responded well to topical treatment with aluminium subacetate.

Introduction

Pyoderma vegetans (PV) is an unusual response to bacterial infection. Most reported cases have been in the setting of immunodeficiency especially HIV-AIDS. We report a case of pyoderma vegetans in a background of IgA deficiency. This was acquired sub-tropically and responded well to topical treatment.

Case Report

A 41-year-old Caucasian male developed multiple, tender, crusted lesions on an erythematous base after a 3-month holiday in southern India. He had no systemic illness other than known IgA deficiency. At the time of presentation, he was on omeprazole and prednisolone prescribed by his general practitioner for these lesions. These lesions had appeared over a 2-week period, having started as small, tender pustules. There was no associated fever, malaise, night sweats, abdominal symptoms, or cough. All family contacts including domestic pets in India remained healthy. Examination revealed a healthy male with multiple lesions [Figures 1 and 2].There was no lymphadenopathy. Chest examination was within normal limits. Differential diagnoses considered at this stage were cutaneous tuberculosis, leishmaniasis, actinomycosis, PV, and cutaneous amoebiasis. The full blood count indicated to be marginally elevated the white cell count showed a neutrophil predominance. Serology confirmed IgA deficiency. Serology for HIV was negative. Stool microscopy and culture was negative for protozoa/helminths. A chest x-ray showed no abnormalities. Histological examination of tissue showed pseudoepitheliomatous hyperplasia. Swabs were positive for Staphylococcus aureus. Fungal cultures were negative. Cultures were negative for AFB. The patient was treated with aluminium subacetate soaks three times daily. Two weeks after treatment with topical therapy, the lesions had cleared [Figure 3].

Figure 1

Abdomen of the male showing multiple crusted plaques on an erythematous base (433 × 325 mm, 180 × 180 DPI)

Figure 2

Close-up view of lesions on an inflamed base (433 × 325 mm, 180 × 180 DPI)

Figure 3

Healed lesions after aluminium acetate soaks (541 × 406 mm, 72 × 72 DPI)

Discussion

Pyoderma vegetans (PV) is a cutaneous response to bacterial infections – most often staphylococcal and streptococcal. It was originally described by Hallopeau in 1898.[1] Histology is characterized by pseudoepitheliomatous hyperplasia. Cases have been reported on a background of HIV-AIDS;[2-4] inflammatory bowel disease;[5] lupus nephritis;[6] and mycosis fungoides.[7] Treatment options for PV are curettage, antibiotics, and topical therapies including aluminium subacetate soaks and attention to any underlying systemic illness. In summary, we describe a 41-year-old male with known IgA deficiency in whom PV responded successfully to topical treatment. To our knowledge, an association of PV with IgA deficiency has not been reported in the medical literature.