Tuesday, August 28, 2007

Evolving Lupus Variant?

Your thoughts are most welcome with regards to this patient. I don't have a firm diagnosis yet, but suspect this will turn out to be a form of lupus -- possibly subacute or acute LE..

HPI: A 47 year old woman was seen on June 11th, 2007 with with one month history of two 8 cm. plaques on her legs, KOH negative. Did not look like panniculitis. Initially thought to be Lyme disease, the lesions did not respond to doxycycline.

Because of progression of lesions and question of hypersensitivity disorder per biopsy the patient was treated with prednisone and the eruption subsided over a few weeks.

On 10 mg of pred a day, around 5 weeks after starting prednisone, the patient experienced a marked flare. This was during a long weekend at the beach. She said the weather was overcast and she wasn't out that much. Initial lesions recurred and there were some new papules and plaques on arms and legs. She has marked facial erythema and erythema of neck and upper chest. Other than pruritus, she feels well. No new meds.

Late August to Sept. 2007. Patient's eruption flared on legs and arms. On September 4, for the first time a definite butterfly rash on malar eminences. She feels well other than pruritus and has no arthralgias or constitutional symptoms. Sept. 5th, butterfly rash gone.

O/E: Erythematous plaques on legs. Facial erythema. Flushing of neck. Scattered papules and plaques legs and to a lesser extent arms. See photographs




Lab: 7/4/07 CBC and chemistries normal. ESR 24
8/28/07 Repeat CBC (normal) ESR 17, ANA + anticentromere 1:360
9/4/07 Repeat CBC, PLT normal, ESR still 17

Pathology: 6/30/07
Mild epidermal spongiosis with focal lymphocytic exocytosis and mild to moderate superficial and deep perivascular and interstitial lymphohistiocytic infiltrate with scattered neutrophils and rare eosinophils , extravasated erythrocytes and papillary dermal edema .

Pathologist's Note : These changes are consistent with an allergic dermal hypersensitivity reaction with mild vasculopathy, and may be seen in erythema chronicum migrans . These are not the changes of erythema nodosum . The differential diagnosis includes , in the appropriate clinical setting , a drug eruption or a pigmented purpuric eruption . P.A.S. stain is negative for fungal organisms. Clinico-pathologic correlation is suggested.

Pathology 8/27/07
Hyperkeratosis , focal parakeratosis , flattened epidermis , mild papillary dermal edema , ectatic blood vessels, a mild to moderately dense superficial and mid perivascular lymphocytic infiltrate with occasional plasma cells and extravasated erythrocytes .

Pathologist's Note : These changes are non-diagnostic . The differential diagnosis includes , in the appropriate clinical setting , a deep gyrate erythema, a collagen vascular disease , or possibly a drug eruption . The paucity of eosinophils does not favor the lymphocytic variant of urticarial vasculitis. P.A.S. stain is negative for fungal organisms. P.A.S. stain is negative for fungal organisms.

Repeat Biopsy: 9/4/07 Awaited.

I think this patient most likely has subacute L.E. Other than her skin she feels well. There is a suggestion of photosensitivity from the facts that the process began in early June and exacerbated after visits to the beach and Labor Day weekend when it was warm and sunny. Her hemogram is normal, renal function and UA normal, no fever or serositis. I've watched this evolve over three months. It's been interesting and I could be wrong. I will start her on hydroxychloroquine and get an eye consult.

Your thoughts are important to me.


  1. Sweet's and erythema nodosum are what came to my mind at the first look. She needs more work up. Erythema chronicym migrans is less "hot" in my limited experience and doesn't fit in this case. Cheers,

  2. Clinically this looks like some form of urticarial vasculitis. with positive ANA I would proceed with complements levels esp C3 and C4 and dsDNA. I agree with you SLE need to be considered seriously esp with the butterfly rash, vasculitis, positive ANA and positive anticentromere antibody.

    Anticentromere antibody is a significant lab finding. It is usually seen in CREST or systemic sclerosis. In the presence of centromere antibody without systemic sclerosis or CREST it often indicates underlying rheumatic or connective tissue disease esp SLE or an active seronegative polyarthritis or even Raynaud's.

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  4. I suspect she will turn out eventually to have limited systemic scleroderma. Medsger at Pittsburgh says the skin hardening in limited systemic scleroderma (formerly known as CREST) can be delayed for decades. In the meantime, watch for evolving GERD, slow swallowing, Raynaud's, constipation alternating with diarrhea, and particularly if she starts huffing and puffing with exercise, use echocardiograms to monitor her for pulmonary hypertension, and pulmonary function tests to check for reduction in DLCO. Also do nail capillaroscopy to see if she is developing the enlarged capillary loops typical of scleroderma.


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