Laboratory Data

nil

To demonstrate “ the orphan patient”

To solicit any novel or promising therapeutic suggestions.

David Elpern MD had a similar patient. While his patient had smaller lesions, he had many more. All developed with what may have been acne fulminans. The case can be viewed in the archives, Case March 30, 2005

Jones KC, Fuller CD, Luh JY, Childs CC, Miller AR, Tolcher AW, Herman TS, Thomas CR Jr. Case Report and Summary of Literature: Giant Perineal Keloids Treated with Post-Excisional Radiotherapy. BMC Dermatol. 2006 Apr 19;6(1):7

BACKGROUND: Keloids are common benign tumors of the dermis, typically arising after insult to the skin. While typically only impinging on cosmesis, large or recurrent keloids may require therapeutic intervention. While no single standardized treatment course has been established, several series report excellent outcomes for keloids with post-surgery radiation therapy.

CASE PRESENTATION: We present a patient with a history of recurrent keloids arising in the absence of an ascribed trauma, and associated with a maternal familial history of keloid formation. Physical examination revealed several large perineal keloids of 6-20 cm in the largest dimension. The patient was treated with surgical extirpation and adjuvant radiation therapy. Radiotherapy was delivered to the scar bed to a total dose of 22 Gy over 11 daily fractions. Acute radiotherapy toxicity necessitated a treatment break due to RTOG Grade III acute toxicity (moderate ulceration and skin breakdown) which resolved rapidly during a 3-day treatment break. The patient demonstrated local control and has remained free of local recurrence for more than 2 years.

CONCLUSION: Radiotherapy for keloids represents a safe and effective option for post-surgical keloid therapy, especially for patients with bulky or recurrent disease.

Nico Mousdicas MD, Indianapolis, IN, USA on Apr 25, 2006

Would consider doing trial therapy on one lesion, by injecting Interferon intralesionally and perilesionally , shave use Aldara to open wound and once healed apply tailor made Compression garment to assess results and proceed from there. Good case which warrants imperical therapy

Shahbaz Janjua MD, Consultant Dermatologist, Ayza Skin and Research Centre, Lalamusa, Pakistan on Apr 25, 2006

Treatment of keloids and hypertrophic scars remains a challenging task in the modern era of scientific reseach. Lesions that are excised often recur larger than the original lesions but extirpation followed soon by radiotherpy has been proved to be effective. Surgical excision followed by intralesional steroids or interferon is another option. Orphan patient is another challenge to deal with. Support groups for such patients are doing an excellent job.

Gregory Herbich MD, Honolulu, HI, USA on Apr 26, 2006

I have experience alternating 5 FU injections with
585 nm. pulse dye laser monthly with good results.
She has larger keloids than I am used to treating so a debulking seems reasonable. Treatment takes Years Also Botox injections and Bleomycin have been tried by others.

Saraswat Abir MD, Lucknow, India on April 26, 2006

Intralesional liquid nitrogen cryotherapy can be a ray of hope in such patients and I have had good results in some pretty hopeless-looking keloids with it. (Plast Reconstr Surg. 2003 May;111(6):1841-52) I use an even simpler modification of this technique; I use a simple lumbar puncture needle instead of a specially designed one. It works as well. For the smaller lesions, you could use an 18-gauge needle attached to the cryo gun.
The only problem is the invariable depigmentation at the entry and exit points of the needle, which is sometimes irreversible. Mostly however, it responds to topical PUVA / NB-UVB / simple sun exposure. All the best with this difficult problem.

Sate Hamza MD, Winnipeg, Canada on April 27, 2006

I am wondering whether surgical excision followed by postoperative use of imiquimod 5% cream would be helpful in a case like this.

Michael Albom MD, Clinical Professor of Dermatology, Ronald O. Perelman Department of Dermatology, New York University Medical Center, New York, NY, USA on May 1, 2006

There are two issues that are predominant in this very difficult case. The first is that of the orphan patient. If possible, it would be very helpful to have one physician provide treatment to this patient. When treating chronic conditions, it is important to establish a supportive relationship in which the patient can develop trust especially when the outcomes are uncertain.

In my experience, I have had the best results with a combination of deep cryotherapy and Kenalog intralesional injections. Here is what I do: the area(s) to be treated must be anesthetically blocked outside the perimeter of the keloid using slow injections with a 30 gauge needle to minimize the discomfort of the administration of anesthetic solution. I use a combination of Lidocaine and Marcaine. After waiting about 10 minutes, I completely freeze the keloid with a spray device and a thin probe. Then I allow the frozen keloid to thaw and allow another 5 minutes or so to elapse. I then slowly inject the edematous keloid with Kenalog 40mg per cc with a 20 guage needle. It is important to inject deeply and entirely through the keloid with multiple passes of the needle. I also insure that I remain within the keloid itself and not inject beyond its deep or lateral borders. Sometimes I have found, but not consistently,that surgically debulking the keloid to just below the surface of the skin has helped followed by immediate injection of steroid. Monthly injections of Kenalog must be done to prevent recurrence of the keloid. Therefore in recent years, because surgery has not necessarily provided me with results superior to the combination of cryosurgery and Kenalog, I have just about abandoned surgery. Since the patient has several keloids, treatment will have to done monthly over many months to over a year. Clearly, one wants to avoid adrenal-pituitary axis depression with the use of high dose steroids. She may need to be monitored in conjunction with an endocrinologist. As was mentioned by another commentator (in the comment section of this case), there will probably be hypopigmentation which can be permanent. Therefore, treating one of the smaller keloids to start, would be advised and then monitor her progress. If she is truly upset with altered pigmentation, then other modalities may have to be chosen. Although there is no guarantee, the patient will more likely accept the ultimate results of any given treatment if she is educated, prior to treatment, about the advantages and disadvantages of the treatment that may be utilized.

Roger Ceilley first published this concept of combined cryotherapy and intralesional fluorinated steroid injections for reducing keloids and hypertrophic scars in: J Dermatol Surg Oncol. 1979 Jan:5 (1): 54-6.

Lastly, I have concerns in regard to your reference about radiation therapy. There is no question that radiation therapy has been effective in the treatment of keloids. However, the referenced case indicated that radionecrosis of tissue occurred even though the patient is free of keloids for two years. One wonders what potential there is for cancerous changes at the treatment site over the long term. I would venture to say that pigmentary alteration of the skin would eventually occur in your 26 year old patient. However, more importantly, I wonder what her risk would ultimately be for developing a radiation-induced skin cancer after 15, 20, or more years post treatment. Radiation treatment for keloids seem to be given in a shortened schedule as compared to the treament of skin cancers in which fractionation is extended over a 6 week course. Many articles lump the treatment of hypertrophic scars and keloids together. This is not helpful because keloids are much more resistent to any treatment as compared to hypertrophic scars. There are too many variables to mention here that could affect the long term outcomes. Most importantly, published data is lacking for long term followup in these types of patients who have undergone radiation therapy. It is important to recall that skin cancers have occurred 60 or more years after treatment in some patients at sites of previous radiation therapy.

Whatever treatments are chosen, the patient must understand that there is an element of uncertainty in what will actually occur. The fact that treatments will go on potentially for a year or two or perhaps even longer must be shared up front by the physician who hopefully "adopts" this ophaned young woman. I believe that if her physician is truly available to her over the long term, she will be better able to contend with the uncertainties of treatment and their ultimate outcomes. Lastly, a positive and patient attitude is most useful in managing the tribulations that will occur during the course of treatment. I wish you well as you manage this young woman's dilemma.

Pakhi Pereira MD, Bangalore, India on May 5, 2006

I have treated a case where the keloid had recurred within a few months of excision. The patient had a single large cosmetically disfiguring keloid on the left ear lobe after ear piercing. I treated the case with excision and oral antibiotics for a week . This was followed by Inj. triamcinolone 10mg every three weeks into the borders of the excision scar. 9 months later there was no recurrence. The patient was lost to follow-up thereafter. The cosmetic result achieved was highly satisfactory as there was apparently no difference between the two lobes. Hope this helps your 'orphan' case. Best of luck.

Hussain Mahdi MD Sanabis, Bahrain on May 12, 2006

I guess imiquimod may help after surgical removal

Ref: Stashower ME. Successful treatment of earlobe keloids with imiquimod after tangential shave excision. Dermatol Surg. 2006 Mar;32(3):380-6.