Case Number : Case 928 - 13th January Posted By: Guest

Acrokeratoelastoidosis

Clinical ?? mass ?? how long??

I see diminished elastic fibers in a background of sparse cellular inflammation. My first hypothesis was Acrokeratoelastoidosis, but this is typically seen over the margins of the hands and feet. I also think this could be a late stage if Granuloma annulare.

Degenerative collagenous plaques of the hands vs acrokeratoelastoidosis presenting as knuckle padlike lesions (as a part of marginal papular acrokeratoderma disorders).

The first impression I had looking at the low power picture was nephrogenis systemic fibrosis, but taking into account the clinical information and the special stains, I think acrokeratoelastoidosis fits best. There is a case of acrokeratoelastoidosis described in literature, which presented clinically as [url="http://www.ncbi.nlm.nih.gov/pubmed/?term=3891801"]small papules located mainly over the interphalangeal joints[/url].

ITo e there is mcinous degeneration.Could be surface of synviial cyst , i.e mucus cyst .I would order deeeeep secttion.

Acrokeratoelastoidosis is a genodermatotic keratoderma and, as such, I would expect to find hyperkeratosis overlying a slightly pushed-down epidermis. To me, stratum corneum appears normal for an acral site. Furthermore, the basophilic fragmented elastic fibers are too deep located and escorted by interstitial histiocytes immersed in increased connective tissue mucin, findings that suggest subcutaneous granuloma annulare.

I agree with Maria and Robledo. There is some raggedness at the lower edge that may suggest a synovial cyst edge, and there is increased dermal mucin. Synovial cyst vs. GA.

[right][font="arial, verdana, tahoma, sans-serif"][color="#1c2837"][size=4]I agree with Dr. Robledo F. Rocha for the epidermal changes, and the lesion is deeper than [/size][/color][/font][/right][color=#1C2837][font=arial, verdana, tahoma, sans-serif][size=4]acrokeratoelastoidosis.[/size][/font][/color][right][font="arial, verdana, tahoma, sans-serif"][color="#1c2837"][size=4] If it's only one lesion, I'll considered it a resolving (fibrotic, [/size][/color][/font][/right]scarred ) [color=#1C2837][font=arial, verdana, tahoma, sans-serif][size=4][right]fibrous histiocytoma ( giant cell tumor of tendon sheath) with degenrative changes in nucli of fibroblast. Nuclear changes like this [/right][/size][/font][/color]aren't[color=#1C2837][font=arial, verdana, tahoma, sans-serif][size=4][right] seen in [/right][/size][/font][/color][color=#1C2837][font=arial, verdana, tahoma, sans-serif][size=4]acrokeratoelastoidosis[/size][/font][/color]