Central Centrifugal Cicatricial Alopecia: Does my Biopsy Show I have CCCA?
I Received My Scalp Biopsy Results. Does it suggest I have CCCA?
I’ve selected this question below for this week’s question of the week. It allows us to discuss the important points in evaluating scarring alopecia. Here is the question….
QUESTION
I'm a female of African descent in my early 30s. I'm on oral minoxidil and taking Nutrafol. I received the attached scalp biopsy result and I can't tell if it’s concluded that I do in fact have CCCA as it says that findings are compatible to it. To me, is not a definitive answer.
I'm really not sure what to make of this in layman's terms. Can you help?
ANSWER
Thanks for the question.
It’s important to understand that we don’t diagnosis central centrifugal cicatricial alopecia (CCCA) “just” with a biopsy. We diagnose it with two things - a biopsy PLUS the clinical examination. I only have the biopsy in front of me and I haven't examined your scalp - so I can’t say with 100 % certainly.
However, with that behind us….. it does seem like you have CCCA. I would be surprised if you did not have CCCA with the limited information you have provided to me in your question and with your submission of biopsy results.
In other words, even though I’m not 100 % sure, I’m about 95-99% sure that a patient with the information you have given would have CCCA.
But let’s delve a bit deeper and look at a few key points:
Point 1. Your biopsy shows loss of sebaceous glands
Sebaceous glands just mean the oil glands of the scalp. Loss of the sebaceous glands is a really important finding because only scarring alopecias have this finding on biopsy. If you did not have a scarring alopecia, the sebaceous glands would not be lost like they are in this biopsy. Your biopsy also shows scar tissue and the typical inflammation of lymphocytes and plasma cells we see in CCCA.
Taken together, these two points tell me you have a scarring alopecia with 100 % certainty.
Point 2. CCCA does not have have its own biopsy findings. Other scarring alopecias can look 100 % identical to CCCA.
It would be great if a doctor could biopsy CCCA and send it to the pathologist and the pathologist could just look under the microscope and say “yes, this is CCCA." Unfortunately, that’s not how it works.
The biopsy findings of CCCA can be 100 % identical to a few other scarring alopecias including lichen planopilaris (LPP) and frontal fibrosing alopecia (FFA). You might be interested to know about a 2005 study where worldclass pathologists were given biopsy slides from several patients who already had known diagnoses. Some had CCCA. Some had LPP. Some had FFA. The pathologists were not told what the patient had and could only look under the microscope to make a diagnosis. What was so interesting was that the expert pathologists could not distinguish CCCA from LPP and FFA. They all look the same under the microscope! These were not amateur or newbie pathologists. These were some of the very best pathologists in the world. Their answer was simple : CCCA looks just like LPP and FFA under the microscope. Of course, these scarring alopecias don’t look the same when the scalp is examined in the clinic - and that’s why ideally I’d need to examine your scalp myself to tell you with 100% certainty what you have.
But I’m 95-99 % sure you have CCCA. 95-99% is pretty close to 100 %.
The key point is that the best you can ever receive from a scalp biopsy of even the most classic clinical presentation of CCCA is ‘compatible’ with CCCA. If you are looking for the pathologist to commit to saying “Yes this is CCCA!” - it will never happen because a few conditions look 100 % identical. It’s up to the dermatologist to take the results from the biopsy and the results from the scalp examination and come up with the final diagnosis. The responsibility lies with the dermatologist not the pathologist.
3. You have hair loss in the crown (vertex).
Your biopsy tells me have a scarring alopecia. I looked carefully at your report. Given that your biopsy was taken from the crown, I know now that your concerns relate (at least in part) to hair loss in the crown.
What is the “most likely” type of scarring alopecia in a female of African descent in her early 30s with hair loss in the crown?
CCCA ….. by far.
It certainly does seem like CCCA is the diagnosis that relates to this biopsy and story.
Conclusion
Thanks again for the great question. It does appear that CCCA fits with your biopsy results. That’s what the pathologist is referring to by ‘compatible.’ You’ll want to review treatments for scarring alopecia and CCCA with your dermatologist. The treatments for CCCA include topical steroids, steroid injections, topical calcineurin inhibitors. Oral medications like doxycycline can be helpful for some. All patients with CCA should have blood tests. Vitamin D levels should be checked (test is called 25 hydroxy vitamin D) as well as iron levels ( test is called ferritin test) and thyroid tests (test is called TSH). If vitamin D or iron levels are found to be low they must be replenished and this typically involves taking supplements. Other blood tests might be important too depending on your full story.
You and your dermatologist can sit down and review which is best in your case. Some patients with early staged CCCA can get a bit of hair back but the main goal is to stop the hair loss from spreading and from getting worse over time.
Thank you again. I’ll include some references that may be of interest:
Use of Topical Metformin for CCCA
Reduction in Sebaceous Glands Might Not Be An Early Feature of CCCA
Trichoscopy of CCCA: The Peripilar White-Grey Halo
Increased risk of fibroids in women with CCCA
Hair Transplantation for Women with CCCA
Reference.
Mirmirani et al. Primary cicatricial alopecia: histopathologic findings do not distinguish clinical variants. Journal American Academy Dermatology 2005 Apr;52(4):637-43.