I have additional been diagnosed with Lupus Discoid. My Doctor is having an ANA test done. He stated that with SCLS it complicates diagnosing exactly what kind of Lupus I have. I do react to IVIG, so I take premeds and they run the IVIG at a slower rate. I am wondering if anyone else has run to to rash problems or Lupus in addition to SCLS. Regards, Rita

I was initially checked for lupus due to my horrible rash and itching prior to a capillary leak episode. The doctors had not been able to diagnose SCLS at this time so lupus was the first test on their list. The lupus test came back negative so they kept on testing me for various other conditions. I had this red rash and small to medium areas of itchy dry patches on my arms, chest, eyelids, eyebrows, back and upper thigh region as well as the butterfly red rash on my face. I still get an itchy rash prior to a capillary leak, but they have no clue why. I have had several biopsies and still no answer. Good Luck with your diagnosis.

By butterfly rash, do you mean a triangular rash on each side of the lower nose? I have been trying to figure out the cause for this, but can't get in to see a dermatologist until May 26.

I do have rash problems but I believe it is caused by the IVIG

Yes. I apologize for being unclear. My rash has appeared since my first infusion. I am now awaiting my second infusion. The length of time it takes to see a derm was an irrelevant fact, grounded in mild frustration.

Bonjour à tous, Alors qu'il semblerait qu'une forme familiale de SCLS puisse exister car divers membres de ma famille maternelle présentent les signes de cette pathologie, je souhaite apporter mon témoignage et mon expérience en ce qui concerne le LUPUS qui du fait des rougeurs apparaissant sur le contour des yeux, le haut des joues et les ailes du nez peuvent aisément se confondre avec celles apparaissant dans le cadre de SCLS. Un examen attentif fait toutefois apparaître une différence notable : les rougeurs ne s'arrêtent pas au masque (d'où le nom latin de LUPUS qui renvoie au mot LOUP c.à.d. le petit masque qui couvre le visage des personnes costumées lors du carnaval de Venise en particulier) mais se poursuivent sur tout le haut du corps sur la partie communément appelée le décolleté avec curieusement un espace blanc en triangle vertical s'étendant de l'extrémité du visage à la base du cou, accompagnés d'un gonflement, plus ou moins important du pharynx et des bronches moins visible mais générateur dans sa forme grave de symptômes identiques à ceux d'un œdème pulmonaire à issue fatale, la fuite capillaire du plasma s'étant infiltrée dans l'enveloppe entourant les bronches et les poumons. Le SCLS - dénommé "Maladie ou Syndrome de "CLARKSON" du nom du premier médecin qui a fait une communication à ce sujet dans les années 60 et qu'une dizaine de cas seulement (tous décédés) était connus à l'époque - c'est ainsi que l'une de mes cousines est décédée en 1988 d’une embolie pulmonaire attribuée au LUPUS malheureusement peu de temps avant que moi-même n'ait été diagnostiquée pour une hyperperméabilité capillaire (autre nom donné un certain au SLCS). Son médecin traitant n'étant plus en vie il ne m'a pas été possible d'obtenir d'informations plus précises à ce sujet. Amicalement, Maggy Sorry for my english, hereunder translation about this text While it would seem that a family form of SLCS could exist because various members of my maternal family introduce the signs of this pathology, I want to bring my experience and my evidence as regards the LUPUS which due to redness appearing on the outline of eyes, the top of cheeks and wings of the nose can easily become confused with those appearing as part of SLCS. The Latin name of LUPUS means WOLF in English which is the name of the small eye mask covering the face of the persons put on fancy dress during the Venice carnival. An attentive examination however shows a notable difference: redness does not stop on the mask but continue on the head, face and the top of the body to the part commonly called the neckline with, curiously, a whitespace in vertical triangle stretching of the end of the face at the root of the neck, accompanied with a swelling, more or less mattering from the pharynx and bronchus less visible but generative in its serious form of symptoms identical to those of a pulmonary edema with fatal exit, capillary escape of plasma having seeped through in the envelope encircling bronchus and lungs. SLCS - called "Illness or "CLARKSON" *Syndrome of the name of the first doctor* who made a medical communication on this subject in the sixties and when a dozen cases only (all died) was known to epoch - that's how one of my cousins (women aged 45) died in 1988 unfortunately shortly before that myself was diagnosed. So as her doctor not be alive any more it was not possible to me to get more definite information on this subject. Best regards.