Abstract: 27 year old man with four year history of a papular eruption.
HPI: This man's lesions began on the face four years ago and later developed in the groins and axillae. Initially macules, they became papular and much more numerous. At onest, they were felt to be verruca plana. Cryotherapy was tried without success.
O/E: The lesions are 2 - 3 mm in diameter brown to yellowish papules. Distribution: Face, crural folds, axillae.
Clinical Photos:
Lab: Full blood count normal, cholesterol 5.2 upper limit, triglycerides normal, LDL HDL also normal, Immumoglobulins normal, plasma electrophoresis normal, thyroid function tests normal.
Pathology: Two biopsies were done.
The first showed a mild chronic inflammatory infiltrate in the upper dermis made up of small lymphocytes and histiocytes with occasional macrophages.
Repeat biopsy from the right axilla was reported as aggregates of foam cells admixed with other histiocytes, lymphocytes and some fibroblasts: "appearance consistent with eruptive xanthomas"
Diagnosis: Do you think this man has xanthoma disseminatum?
Questions: This entity may be associated with paraproteinemia and mucous membrane involvement. Comments for diagnosis and management will be most welcome. What are your thoughts? What further would you do?
I have seen two patients with similar lesions, who also had normal serum lipids. The papules were redder in colour but with the same central depression to many of the papules. We called it xanthoma disseminatum. See illustrations and description at http://www.dermnetnz.org/dermal-infiltrative/xanthoma.html
ReplyDeleteWe didn't provide any specific treatment and the lesions later disappeared.
Excellent case thanks to share with us. I agree also with XD.
ReplyDeleteXanthoma disseminatum is a rare benign histiocytic disorder with extensive cutaneous and mucous membrane xanthomas in normolipemic patients as also said by author. By the way in some atypical cases of XD dermoscopy can give you the solution if the biopsy is not available. The dermoscopic finding is orange-yellow ('setting sun') homogenous structure. I had seen case similar like that and dermoscopy helped me to make the right diagnosis directly.
As for a treatment is extremely difficult. As far I know usually treat XD with Azothiaprine, Cyclophosphomide, Interferon-alpha, Vinblastine, Vasopressin and also some scientist was use local radiotherapy.
Some time ago we try to treated XD with Prospidin-Cytostatic (100mg a day for course 3g-I/M) with some effect. By the way in some cases have been seen to regress spontaneously.
It looks like xanthoma disseminatum, but I worry about sarcoid (despite the biopsy) and a brewing malignancy of histiocytes. I'd repeat biopsies at intervals, and have him scanned and perhaps "bone marrowed" as well.
ReplyDeleteAs for therapy, well, I think the only one is "time". R.R. Wyomissing, Pennsylvania
This is very interesting case by presented by Philip. I would consider it to be Eruptive Histiocytosis or EDV (lesions over face are like warts). One need to do repeat biopsy and may be a review by another Dermatopathologist. As for treatment, worthwhile trying acitretin for EDV or topical tazarotene for facial lesions after confirming the diagnosis. For histiocytosis, close observation, systemic evaluation and once weekly low dose methotrexate will be useful.
ReplyDeleteInteresting case but I am very worry about the clinical and histopathological diagnosis?????.It looks like disseminated viral warts.Please repeat the biopsy from 2 sites.If it would be D xanthoma,that is very strange and every day we encounter strange cases that shock our experience.We are always learing
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