Kayoko Matsunaga

We report two cases of anaphylaxis due to ingestion of mite-contaminated Okonomi-yaki (Japanese pancake) mix. Case 1: A 50-year-old female developed anaphylaxis after eating Okonomi-yaki prepared from Okonomi-yaki mix that had been stored at room temperature after opening. She demonstrated serum specific IgE against Dermatophagoides farinae (Der. f.), but did not demonstrate any wheat- or gluten-specific IgE antibodies. Prick tests showed positive reactions to Der. f. antigen (Torii) but not to previously unopened Okonomi-yaki mix or wheat flour that had been stored in a freezer. Case 2: A 28-year-old female developed anaphylaxis after eating Monja-yaki prepared from Okonomi-yaki mix that had been stored at room temperature after opening. She demonstrated serum specific IgE antibodies against Der. f. but no wheat- or gluten-specific IgE antibodies. Prick tests showed positive reactions to the responsible Okonomi-yaki mix and Der. f. antigen (Torii), but not to wheat flour that had been stored in a freezer. The responsible Okonomi-yaki mix contained 50 heads/g of Der. f., and a high level (64.1 μg/g) of Der f1 antigen was detected by specific ELISA. Immunoblotting revealed a specific 25 kDa band in patient sera against the responsible Okonomi-yaki mix, which was absorbed by Der. f. crude antigen.

Pemphigus vegetans is a rare variant of pemphigus vulgaris characterized by flaccid bullae that become eroded and form vegetations or papillomatous lesions, especially in intertriginous areas. The standard treatment for pemphigus vegetans has been oral administration of corticosteroids. We reported a case of pemphigus vegetans in a 67-year-old Japanese female with a high titer of anti-desmoglein 3 autoantibodies (index value of 14,433.3) treated with double filtration plasmapheresis (DFPP) and pulse cyclophosphamide therapy. The treatment schedule consisted of repeated DFPP with the use of albumin solution as the substitution fluid and subsequent administration of high-dose cyclophosphamide (12 mg/kg; total dose of cyclophosphamide pulse was 600 mg) and 2.5 mg/kg/day of methylprednisolone. The skin lesions were cured after three treatments with DFPP and cyclophosphamide, and the titer of anti-desmoglein 3 autoantibodies decreased to an index value of 26. No significant adverse effects were observed. Afterwards, she was treated with low dose systemic steroid and tacrolimus. DFPP combined with synchronized pulse cyclophosphamide may be a useful treatment regime in the management of pemphigus vegetans.

Pemphigus vegetans is a rare variant of pemphigus vulgaris characterized by flaccid bullae that become eroded and form vegetations or papillomatous lesions, especially in intertriginous areas. The standard treatment for pemphigus vegetans has been oral administration of corticosteroids. We reported a case of pemphigus vegetans in a 67-year-old Japanese female with a high titer of anti-desmoglein 3 autoantibodies (index value of 14,433.3) treated with double filtration plasmapheresis (DFPP) and pulse cyclophosphamide therapy. The treatment schedule consisted of repeated DFPP with the use of albumin solution as the substitution fluid and subsequent administration of high-dose cyclophosphamide (12 mg/kg; total dose of cyclophosphamide pulse was 600 mg) and 2.5 mg/kg/day of methylprednisolone. The skin lesions were cured after three treatments with DFPP and cyclophosphamide, and the titer of anti-desmoglein 3 autoantibodies decreased to an index value of 26. No significant adverse effects were observed. Afterwards, she was treated with low dose systemic steroid and tacrolimus. DFPP combined with synchronized pulse cyclophosphamide may be a useful treatment regime in the management of pemphigus vegetans.

An 82-year-old male visited a dermatologist for skin eruption in the left axilla 1 year and 3 months before visiting us. The lesion was treated externally as mycosis, but showed no improvement. As a result, the patient was referred to our department. A 5-cm erythematous lesion with partial erosion was present in the left axilla, and was diagnosed as extramammary Paget's disease by skin biopsy. The lesion was excised from the deep adipose tissue layer at 1-cm from the margin, followed by mesh skin grafting. There was no enlargement of the axillary lymph nodes. Left axillary lymph node swelling was noted 1 year and 2 months after surgery, and axillary lymph node metastasis was diagnosed by lymph node biopsy. Left axillary lymphadenectomy was subsequently performed. On whole body examination, no metastasis to any other organ was noted. As postoperative adjuvant therapy, irradiation at a total dose of 50Gy and 4 cycles of weekly administration of 40mg taxotere were given. No recurrence or metastasis of the tumor had occurred as of 10 months after lymphadenectomy, nor were there severe adverse effects of postoperative adjuvant therapy.[Skin Cancer (Japan) 2010 ; 25 : 33-37]

Sweet syndrome, which was reported by Sweet in 1964, is characterized by a combination of fever, neutrophilia, tender erythematous nodules or plaques, and histopathologic findings showing a dense infiltrate of mature neutrophils in the upper dermis. Systemic administration of corticosteroids is the “gold standard” for the treatment of Sweet syndrome, and the dramatic response is well known. However, considering the underlying disease and various side effects, administration of corticosteroids may be harmful in some patients. We encountered a patient with Sweet syndrome who showed dramatic and rapid response to systemic administration of potassium iodide. The patient became afebrile and free from symptoms within 48 hours after starting potassium iodide, and there have been no recurrences after cessation of the therapy. Although the mechanism of action is not clear, potassium iodide can be a first line drug, because of its effectiveness, safety, and low cost.

Three elderly male patients aged 55, 76 and 91 years presented with widespread eruptions of erythematous, flat-topped confluent red to reddish-brown papules that spared the skin folds. They had each undergone various unsuccessful treatments (oral antihistamines, topical and oral corticosteroids). We diagnosed these patients as having Ofuji's papuloerythroderma. These eruptions were not associated with malignant tumors. Their eruptions and subjective symptoms started to reduce after approximately 4-7 narrow-band UVB treatments, and the eruptions virtually disappeared after approximately 10 treatments. They had slight exacerbation but the eruptions subsided. We conclude that narrow-band UVB is more useful for papuloerythroderma than other treatments because of its convenience and limited side effects.

We report two cases of anaphylaxis due to ingestion of mite-contaminated Okonomi-yaki (Japanese pancake) mix. Case 1: A 50-year-old female developed anaphylaxis after eating Okonomi-yaki prepared from Okonomi-yaki mix that had been stored at room temperature after opening. She demonstrated serum specific IgE against Dermatophagoides farinae (Der. f.), but did not demonstrate any wheat- or gluten-specific IgE antibodies. Prick tests showed positive reactions to Der. f. antigen (Torii) but not to previously unopened Okonomi-yaki mix or wheat flour that had been stored in a freezer. Case 2: A 28-year-old female developed anaphylaxis after eating Monja-yaki prepared from Okonomi-yaki mix that had been stored at room temperature after opening. She demonstrated serum specific IgE antibodies against Der. f. but no wheat- or gluten-specific IgE antibodies. Prick tests showed positive reactions to the responsible Okonomi-yaki mix and Der. f. antigen (Torii), but not to wheat flour that had been stored in a freezer. The responsible Okonomi-yaki mix contained 50 heads/g of Der. f., and a high level (64.1 μg/g) of Der f1 antigen was detected by specific ELISA. Immunoblotting revealed a specific 25 kDa band in patient sera against the responsible Okonomi-yaki mix, which was absorbed by Der. f. crude antigen.

We reported three cases of papular tuberculid after receiving Bacille Calmette-Guerin (BCG) vaccination. Case 1 was a 5-month-old girl who developed eruptions on her extremities five weeks after the BCG vaccination. Case 2 was a 6-month-old boy who developed eruptions on his extremities one month after the BCG vaccination. Case 3 was a 5-month-old boy who developed eruptions all over his body 6 weeks after BCG vaccination. Histopathologically, dermal perivascular lymphocytic inflammatory infiltrate and non-caseous epithelioid cell granulomas were observed in all cases. We diagnosed these cases as papular tuberculid resulting from BCG vaccination. All lesions resolved in three months without treatment. To establish the diagnosis when discrete eruptions are observed in a child under one year old, it is important to ask the guardians about the time of BCG vaccination.