Fascinoma

Every day one sees something one never encountered before.
Here's a diagnostic and perhaps therapeutic challenge.

This well-nourished, alert, well-oriented 82 yo woman presents with a six month history of an erosive dermatitis of the perirectal and vaginal area. Over the past year she has had pathological fractures of both femurs and a history of herpes zoster (not cofirmed). Remarkably, she has put up with these erosions which are apparently not that painful.

O/E: Sharply marginated clean erosions around rectum and vagina. Similar lesions are found in skin folds (abdominal and under breasts). She has some oral ulcerations under her dentures and crusted lesions on the scalp. No vesicles or bullae seen or noted by other physicians.

Lab: At this time CBC normal. Antibodies for pemphigus and pemphigoid are negative.

Biopsies performed on Feb. 8, 2007 for H&E and DIF.

Who has seen a similar picture?

I am thinking about:
Pemphigus variant
Necrolytic migratory erythema
Acrodermatitis enteropathica

Will order zinc and glucagon levels if pathology is not helpful. Someone may have seen this picture before.

Updated February 16, 2007
Here's a follow-up on this patient...
Path: Shows:
Subepidermal blister with dense superficial and mid perivascular and interstitial mixed inflammatory cell infiltrate including lymphocytes, plasma cells, eosinophils , dermal edema, and papillary dermal fibrosis .
NOTE : These changes are non-diagnostic but suggestive of cicatricial pemphigoid in view of the immunofluorescence findings (see below).

See immunofluorescence results and note. (Photo by Jag Bhawan)
DIRECT IMMUNOFLUORESCENCE RESULTS : Linear "Immunostaining" was observed with IgG, IgA and intermittent with C3 at dermal epidermal junction and with C3 and IgA around blood vessels and appendages.
NOTE : These changes are non-diagnostic but consistent with cicatricial pemphigoid.

Putting this together, I suspect this is indeed an unusual variant of cicatricial pemphigoid. I have started her on prednisone 20 mg tid and tacrolimus ointment 0.1% (but insurance plans here do not always cover the ointment). A G6PD was ordered since I will probably use Dapson as well. I expect the management will be difficult.

February 22
Patient seen in f/u. To my surprise, there is early reepithelialization of perineal erosions. Oral erosions still prominent. I've lowered her prednisone to 20 mg bid -- awaiting G6PD results. Perhaps, the tacrolimus is helping. The comments havc been helpful.