Perniosis or Chillblains

[Kristysulin]

Hi all. My 14 yr old daughter was diagnosed with CD 2 years ago and has been on Remicade since her diagnosis. She has been in full remission and is doing great, however she has developed painful, burning inflammation on her toes and the underside of her fingers and fingertips. Dermatologist diagnosed her with Perniosis. I am concerned this is either a side efferct of the Remicade or a symptom of another autoimmune. We have been asked to see a rhumatologist to rule out additional autoimmune. Anyone else's child have these symptoms? It goes awat with quick dose of prednisone and as ut heals turns to a purplish blue color...I am at a loss and her GI at Hopkins says she has never seen this before.

 

[Maya142]

I looked it up on Medscape and it looks like it can be associated with autoimmune diseases. My daughter has Raynaud's Phenomenon, which is one the associated conditions. Her fingers and toes go blue/white when they get cold (and by cold, I mean even just in air-conditioning). Her rheumatologist said in her case it is secondary to her autoimmune conditions (Crohn's, arthritis).

It's worth seeing a rheumatologist. I would also about drug-induced Lupus because that can be caused by Remicade and it looks like perniosis can be associated with Lupus.

Causes
The direct cause of pernio is cold exposure; specifically, exposure to both mild nonfreezing cold and humidity seems to be required. [5, 6] Chronic pernio may be secondary to various systemic diseases as follows:
Chronic myelomonocytic leukemia [7]
Anorexia nervosa [8] : Low body mass index may predispose to pernio. [5, 4]
Dysproteinemias
Macroglobulinemia
Cryoglobulinemia, cryofibrinogenemia, cold agglutinins
Antiphospholipid antibody syndrome
Raynaud disease [9]
Celiac disease [10]
Variants include the following:
Kibes (equestrian cold panniculitis): Erythrocyanotic plaques occur on the upper lateral thighs of women who ride horses. Histology is characterized by an intense perivascular infiltrate extending into subcutaneous fat.
Chilblain lupus erythematosus [11, 12] : Violaceous "pernio" plaques appear prominent over dorsal interphalangeal joints, often with positive antinuclear antibody (ANA) or rheumatoid factor (RF). Histologic and immunofluorescent evidence of lupus is present in the skin lesions. Half of the patients have associated facial discoid lupus lesions, and 15% develop systemic lupus.
Drug-induced pernio: Sulindac-induced cases have been reported. [13]

 

[Andreanoelle]

Hi, I am a RA patient who has been on Remicade for 2.5 years. I developed perniosis about a year ago. Not really related to the cold, as I have them during the summer months as well. Had a biopsy of the lesion by a dermatologist who confirmed the diagnosis. At the same time, my ANA became positive.

I'm pretty sure mine is related to the Remicade. I haven't stopped the medication, because my RA seems to flare without it. Methotrexate alone doesn't seem to keep my RA under control.

I did try taking nifedipine and Trental, which did seem to help my perniosis symptoms.