History/Physical: This 53 yo woman presented with a 15 year history of a painful and disabling dermatitis of her buttocks and to a lesser extent face, extremities and torso. She has been seen by many dermatologists, allergists, gynecologists and primary care physicians during this time and even underwent a hysterectomy in an attempt to cure her when the mistaken diagnosis of autoimmune progesterone dermatitis was rfendered. Her partner recently left her because of the disabling nature of dermatitis.
Routine lab studies are unremarkable and ANA was negative. Some special tests are pending.
Clinical Images
March 2024
Shoulder
Histopathology: Biopsy were taken from the left and right buttock: Both specimens contain a small vessel vasculitis. The more recent lesions on the right buttock show the changes of leukocytoclastic vasculitis.
Biopsy from left buttock, March 2024. Four mm punch biopsied from each buttock showed similar changes, but left buttock had a more psoriasiform epidermis.
Thanks to Dr. David Jones, dermato- pathologist, Berkshire Medical Center, for the histopath images.
Tentative Diagnosis: Urticarial Vasculitis
She is being presented your thoughts and recommendations. Complement levels are pending. She has a very high deductable so we are limiting extensive testing that may be unnecessary.
Photo after 5 days of prednisone 20 mg b.i.d.
Reference
Stephanie L Gu, Joseph L Jorizzo. Urticarial vasculitis. Int J Womens Dermatol. 2021 Jan 29;7(3):290-297. PMID: 34222586 PMCID: PMC8243153 (Full Text)
Low dose oral steroid and Hydroxychloroquine have been useful. Local treatment does not help. Only emollient and avoidance of friction should help to reduce the erythema.
ReplyDeleteIn olden times Tab. Dapsone was used with good results.
Very important to know if her compliment levels are below normal. If so, the pt will need a more thorough workup for systemic disease involvement including C1Q autoantibody results.
ReplyDeleteIf compliment levels are in normal range, I would rely on maximizing oral dapsone and colchicine therapy as steroids bearing agents.
Rob Shapiro, Hilo, Hawaii: I wonder if this could be a case of EED. Localized typical areas showing vasculitis on biopsy. Not sure the best way to treat EED. would need to look it up in Lebwohl or if it’s in the 2023 new Fitzpatrick treatment book by Kwatra.
ReplyDeleteFran Storrs, Portland, OR: Can’t tell if bx dermal cells are neutrophils. If so, and if arm and leg lesions are in flexures, systemic contact dermatitis is suggested. Niels Hjorth’s old “baboon syndrome.” Unlikely if doesn’t abate and then recur. If does, chemical or food culprit would be ingested about 12 hrs before onset. Patch tests often pos for culprit.
ReplyDeleteDato Cheng Leng Ong, Malaysia: She has been suffering in pain for past 15 years from her clinical and histological urticaria vasculitis. She is still waiting for her blood results for serum complements to rule out Hypocomplementemic urticaria vasculitis, but clinically no signs of HUVS ( urticaria vaculitis syndrome.)
ReplyDeleteThankfully her treatments need not involve expensive medications.
Her blood can be screened to rule out G6PD deficiency before starting oral dapsone 100mg daily. Dapsone together with a small dose of oral hydroxychloroquine 200mg daily may work synergistically. Of course she needs a yearly visit to the ophthalmologist to rule out any retinal problem before or after taking hydroxychloroquine. If she responds very well, one of the two drugs can be tailed down for maintenance.
Oral analgesics that is safe may help especially initially.
Of course, topical treatment with emollients under the medical device category can be freely applied. A once daily application of a mild yet effective cream like mometasome may be added. Perfumed or medicated soaps should be avoided.