Friday, January 19, 2007

Stranger than Fiction

50 yo man with unusual facial eruption

Presented by:
DJ Elpern

This 50 yo man develops an eruption on his left malar eminence annually. This occurs after his late December deer hunting trip. He is exposed to cold air. Does not take any new meds at that time. The eruption has a burning sensation.

Annular plaques with central pallor and peripheral dusky red color left malar. No other similar lesions.

Clinical Photos:
"X"marks the biopsy sites

Lab and Path:

DIAGNOSIS: Tumid lupus erythematosus
NOTE : The specimen exhibits flattened epidermis , mild to moderate papillary dermal edema, dilated superficial blood vessels and a moderate superficial and deep perivascular and periappendageal lymphocytic infiltrate with numerous extravasated erythrocytes and marked superficial and deep interstitial mucin deposition. These findings support the histologic diagnosis . The differential diagnosis includes , in the appropriate clinical setting , lymphocytic infiltrate of Jessner. P.A.S. stain is negative for fungal hyphae and basement membrane zone thickening. Clinico-pathologic correlation is suggested.

Therapy and Course:
The patient was biopsied and seen a week later for suture removal. No treatment was rendered. Here is how he looked at day six post biopsy.

Questions and Teaching Points:
Histologically this is lupus. However, the patient is asymptomatic and the lesions cleared without therapy. The cold exposure is most likely key. He may have a form of L.E. There is an entity called chilblain lupus which could fit here. Most of those patients had SLE. Has anyone seen a similar case? I will suggest serologies to him. He does not need any therapy at this time.

This and other references can be found in PubMed if "lupus chilblain cold" is searched.

Franceschini F, et. al.
Chilblain lupus erythematosus is associated with antibodies to SSA/Ro.
Adv Exp Med Biol. 1999;455:167-71.

Clinical Immunology Unit, Spedali Civili, Brescia, Italy.

Chillblain Lupus Erythematosus (CL) of Hutchinson is a subtype of Lupus Erythematosus characterized by erythematous lesions symmetrically distributed on the face, nose, fingers and toes, knees and heels. The lesions are induced by cold, damp climates. A number of patients affected by CL eventually develop features of Systemic Lupus Erythematosus (SLE). We report here 7 patients, all but one affected by SLE, with chilblain cutaneous lesions on their hands, feet and face. The onset of CL preceded the diagnosis of SLE, from 1 to 10 years in 3 cases, it was concurrent in one case and was subsequent in the other 2 cases. Six out of the seven patients referred typical Raynaud's phenomenon and one had acrocyanosis. CL lesions developed and were aggravated by the cold during autumn and winter, they improved during summer. Skin biopsy performed in 5 patients from the lesions showed, on histology, a typical pattern of alterations with granular deposits at the dermo-epidermal junction on direct immunofluorescence. Laboratory findings showed: ANA and anti-SSA/Ro were detected in all the patients, anti-SSA/Ro were isolated in 4 patients and associated with anti-Sm in one case, anti-U1 RNP in one case and with anti-Sm and anti-RNP in a third case. Complement consumption was observed in 5 patients, anti-dsDNA in the six patients with SLE, hypergammaglobulinemia in 4 and rheumatoid factor in one. The fine specificity of anti-SSA/Ro as determined by immunoblotting using a human spleen extract as a substrate, showed: anti-60kD and anti-52 kD in two sera, anti-60kD isolated in 2 sera, anti-52kD isolated in one serum (from the patient without SLE) while 2 sera did not blotted. In conclusion, our study confirms the previous report of anti-SSA/Ro antibodies in association with CL. This clinical and serologic association widens the spectrum of cutaneous disease that is associated with antibodies to SSA/Ro to include conditions such as to SCLE, hypergammaglobulinemic purpura and neonatal lupus.

1 comment:

  1. This is very interesting strange case.Yes, the picture is more in favour of chilblain lupus.In Iraq I see outbreak of chilblain ,pernoisis,every year in cold months in the last 30 years.The cold papules nodules and plaques are seen only on cold hands and feet and rarely on the face and ears.The rash simulate that of erythema nodosum or erythema multiforme but the rash is specifically cold which is vey important feature of chilblain.I did not see any change into lupus.The disease remits spontaneosly on heating but we give supportive therapy like steroids.
    khalifa sharquie


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