PEMPHIGUS FOLIACEUS AND NON‐HODGKIN'S LYMPHOMA
Authors: COWLEY NOREEN C. , NEILL SALLIE M. , STAUGHTON RICHARD CD. 
Published: May 2007

A 56‐year‐olcl Iraqi woman presented to her general practitioner with an erythematous scaly eruption on the left ear. An initial diagnosis of otitis externa was made and a 7‐day‐course of penicillin commenced. Three days later, the patient returned complaining of abdominal pain and diarrhea. Examination of the abdomen revealed a large mass. Subsequent investigation with computerized tomography (CT) scanning showed a large paraaortic mass, fine needle biopsy of which showed a diffuse non‐Hodgkins lymphoma. The majority of the cells were small follicle center cells with clear nuclei that stained positively for B‐lymphocyte antigens (L26, MB2). Bone marrow biopsy showed focal paratrabecular deposition of non‐Hodgkins lymphoma cells of a similar nature to those in the paraaortic node. The patient was started on a monthly regimen of chlorambucil 10 mg daily with prednlsolone 40 mg daily for the first 10 days and allopurinol, 200 mg daily. Fourteen days after completing the first monthly course of treatment the patient developed a generalized eruption. This was initially attributed to her allopurinol therapy, which was discontinued. The patient was given a second 10‐day course of prednisolone and chlorambucil, at the end of which the eruption had almost completely resolved. Within 9 days the eruption recurred and the patient complained of red eyes. Examination of the skin showed scattered urticated plaques with peripheral vesiculation on the trunk and annular urticated lesions without vesiculation on the legs. A scaly erythematous eruption was also noted on the left ear (Fig. 1).

The hemoglobin was 10.9 g/dL, with a normochromic, normocytic picture with rouleaux formation on the blood smear. The WBC was 7.9, with lymphopenia of 1.0 (normal range 1.5–4.0 × 10−9). Lymphocyte subset analysis showed decreased numbers of cytotoxic/suppressor T cells (0.16 × 10−9; normal range 0.28–1.35 × 10−9). The helper T cell num‐bers and the platelet count were normal. The erythrocyte sedimentation rate (ESR) was 102. Urea and electrolytes, and liver function tests were normal. Total protein was 85 g/L (normal range 60–80 g/L). Serum electrophoresis showed an acute phase response with increased IgA of 6.0 g/L (normal range 0.8–4.0 g/L). Routine hematoxylin and eosin stain of a biopsy of an urticated vesicular lesion on the trunk and from the erythematous lesion on the ear showed small subcorneal blisters beginning to form that contained eosinophils together with eosinophilic spongiosis. Direct immunofluorescence of perilesional uninvolved skin showed intercellular deposition of igG and C3 typical of pemphigus. Indirect immunoflu‐orescence revealed circulating IgG intercellular antibodies to atitre of 1:160.

The patient was treated with prednisolone, 80 mg daily. The previous regimen of chlorambucil, 10 days each month, was continued and daily allopurinol recommenced. This was followed by resolution of the eruption, the eruption on the ear being the last area to resolve. Ten‐day courses of chlorambucil were given monthly for a further 7 months, following which, a course of palliative radiotherapy was given to the paraaortic nodes. Although repeat CT scan after 3 months of chemotherapy showed a dramatic reduction in the paraaortic mass, this has remained unchanged 1 year later.