Sunday, March 29, 2009

Nodules in Search of a Diagnosis

Presented by
J. Erin Reid, M.D. Dermatology Resident &
Stephen P. Stone, M.D. Professor of Dermatology
Southern Illinois School of Medicine

Abstract: 70 yo man with a five year history of exophytic nodules on the lower extremities.

HPI: A 70 year old white male presented with a five year history of exophytic nodules on the lower extremities. They were increasing in number. A few had been removed by shave excision, and the areas that were treated did not regrow.
Over the past few years he had numerous nodules measuring up to 4 cm in diameter. A few of them were excoriated and crusted. There was no lymphadenopathy. He also had extensive areas of erythema and scale on his forearms, upper arms, and thighs.
He had been in the Navy over 50 years ago and served in Japan. He also went to Bangkok and Hong Kong 20 years ago. No significant past medical history

O/E: On the pre-tibial area the patient has multiple nodular lesions, as well as some erythematous and hypopigmented scars where previous lesions have been removed by shave excision. The lesions range from 1 cm to 3-4 cm in diameter.

Photos:





Pathology: Many biopsies have been performed. In May, 2006, a biopsy showed “superficial perivascular dermatitis of the mixed type, with eosinophilic spongiosis and pustules consistent with an allergic etiology”.
In February, 2007, biopsies of the right anterior and lateral leg showed “marked epidermal hyperplasia, spongiosis, and mixed intraepidermal and superficial dermal inflammatory cell infiltrate”. There was no evidence of malignancy or infection at that time, but there was evidence of chronic venous stasis change.
In January, 2009, we excised another nodule. This was read as “marked epidermal hyperplasia with acute and chronic inflammation” and was negative for fungal, bacterial and acid fast bacilli stains. There is also no evidence of malignancy or carcinoma. Cultures for fungus, anaerobes and AFP were all negative. Flow cytometry was negative.

Diagnosis: What is your differential diagnosis?
Questions: What further information would you want? What additional studies? How would you treat this man?

References will be added when available.



14 comments:

  1. Tough case. Could this be a form of verrucous psoriasis or a variant of prurigo nodularis? The lesions appeared benign and repeated biopsies are consistently non-granulomatous, non-malignant with marked epidermal hyperplasia, and non-specific perivascular changes. Culture again persistently negative.

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  2. Great Case. My list of differentials could include Halogenoderma, Deep mycosis (chromoblastomycosis?), lymphomas (CD30+?),and even Malakoplakia...but regarding the case has been presented by Dr. Stone, it must be a tougher case! I'm eager to know the answer.
    Omid
    PS: I could see only two photos. Access to one of them was denied through my filtered connection. It must contain something interesting :-)

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  3. They are certainly begging to be shaved off! Consider angiolymphoid hyperplasia with eosinophilia and I would need several negative cultures before excluding deep fungus, atypical mycobacteria etc. They also have a lymphomatous look about them despite the histology. Anyone tried a short sharp course of oral steroids to see what happens? Eosinophilic spongiosis raises the issue of pemphigus vegetans but these are too nodular and not enough eosinophils for eosinophilic granuloma. Perhaps one of the histiocytic disorders. Clinically it should be lymphoma, deep fungal infection or cutaneous mets but someone check his syphillis serology if he was in the navy!
    The pseudoepitheliomatous hyperplasia and lack of recurrence after shaving would be compatible with prurigo nodules but these appear to be sitting on the skin surface.

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  4. Just read Omid's suggestion of halogenoderma and like that as well!

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  5. As a ddx I supposed a Sporotrichosis.

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  6. To this mix, I would add pyoderma vegetans (which I am not sure I've ever seen). It may be the same thing as blastomycosis-like pyoderma. There's a good chapter on emedicine.com. It begins: "Pyoderma vegetans (PV) is a rare disorder clinically characterized by large verrucous plaques with elevated borders and multiple pustules. PV is an eruption of multiple pustular ulcerations; it may have a bacterial etiology similar to chancriform pyoderma (Brown, 1957; Su, 1979; Zilberberg, 1960; Wozniacka, In press).
    Heres' the link: http://emedicine.medscape.com/article/1055728-overview
    One wonders if this could be a reactive process -- similar to pyogenic granuloma -- in that there could be various triggers.

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  7. Very interesting case. Clinically the lesions look like: Deep Fungal Infection vs Keraoacanthoma vs Squamous Cell Carcinoma vs Metastasis.

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  8. Amit PandyaMarch 30, 2009

    I would consider HPV infection. You might be able to get HPV typing of one of the tumors. Steve Tyring at UT Houston can help with this. Amit

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  9. Rarely Iododerma/Bromoderma can produce such lesions. We only need to look into the history of medication containing iodides and Bromides (including radio contrast medium) which can be removed this is likely to heal of its own no specific treatment is required. (Comments of Bhusuan Kumar, Chandigarh, India)

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  10. I agree that Iododerma/Bromoderma should be ruled out with careful history. Biopsy should be reviewed to look for subtle acantholysis to exclude pemphigus vegetans.
    Jag Bhawan

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  11. Thanks to all for your input. Will pursue your suggestions, report back as facts are unveiled.

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  12. i thought of deep fungal infections, dermatitis artefacta as differentials.
    regards
    Dr Manish Pahwa
    Arlington, Texas , USA

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  13. This comment has been removed by a blog administrator.

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  14. great rare case,iododerma and bromoderma is sofar from the diagnosis that appear an acneform like picture.Most ddx is Chromoblastomycosis.
    firas altamimi,basra,iraq

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