Thursday, June 14, 2007

Case for Diagnosis





















Presented by Choon Siew Eng FRCP, Johor Bahru, Malaysia.
46 years old woman with 3-year history of gradually enlarging asymptomatic indurated plaques on her neck, both axillae right side of abdomen, right groin and lower back. She is otherwise well with good general health. There was no significant family history.

Physical examination revealed multiple indurated erythematous to hyperpigmented plaques on right side of her neck, right flank, both groins and axillae. The overlying skin is atrophic with brownish adherent scales.
There were multiple groups of hyperpigmented papules on abdomen, upper thighs and legs. Some papules appeared yellowish. Palms, soles, nails, scalp and mucosae are spared.
I am thinking of treating her as sarcoidosis since she is distressed by her extensive lesions
Differential Diagnoses: Nodular amyloidosis, Scleromyxoedema, Morphoea, xanthogranuloma
Repeated blood tests such as full blood count,BUSE, LFT, thyroid functions, serum and urine calcium and autoimmune screening were normal. Her ESR was also normal . CXR was normal and Mantoux test was negative.Sputum for AFB X3 negative. Biopsy from abdominal lesion showed numerous granulomata composed of epithelioid histiocytes, lymphocytes and multinucleated giant cells, Langhan’s type. Special stains for Acid fast bacilli (ZN, Wade fite) and fungal bodies (PAS) were negative. No abnormal deposits of eosinophilic amorphous material seen. No foreign body seen by polarised light.

1 comment:

  1. David ElpernJune 14, 2007

    Dear Choon,

    This is a unique case. Do you actually see sarcoid in Malaysia? If so, perhaps this is it. Her case may be "an experiment of one." I'd recommend showing some photomicrographs to some of our dermpaths and then considering therapy. Here's a recent review from the JAAD (which I think you receive) Ted Rosen (one of the authors) would comment, I am sure. Please keep us posted. There are some articles on TNF drugs for sarcoid, but I haven't reviewed them.

    J. Am. Acad. Dermatol. 2007 Jan;56(1):69-83.

    Cutaneous sarcoidosis therapy updated.

    Badgwell C, Rosen T.

    Dermatology Department, Baylor College of Medicine, and Houston Veterans Affairs
    Medical Center, Texas, USA.

    The widely accepted standard therapy for cutaneous sarcoidosis includes
    corticosteroids, antimalarials, and methotrexate. However, a better understanding
    of the basic immunopathogenic properties of sarcoidosis has elucidated a number
    of steps critical to the persistence and progression of disease that may be
    vulnerable to treatment by targeted therapy. This article reviews both standard
    and newer therapeutic options for cutaneous sarcoidosis.

    ReplyDelete

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