"Speaking of Vitiligo..."

I have always thought that vitiligo was beautiful, including the many patterns that form on individuals with different skin colors, shades, and hues. Vitiligo can appear on any part of the body, in many different shapes and forms. It is most frequent on the face and genitals, then the hands and feet, and then the rest of the body. One woman outlined all of her spots with a pen, which I thought was really cool and I included her picture in my blog about vitiligo and tattoos here (with her permission, of course). Since I have seen many patients over many thousands of visits in my Vitiligo Clinic, I have seen a LOT of patterns, but I still see new ones all the time. It’s one of the things that makes my job a whole lot of fun!

I personally don’t think it’s THAT important to label the exact type of vitiligo that a patient has, except for segmental vitiligo, which I’ll describe below. That’s because all of the other forms are generally treated the same way and have a similar prognosis. It’s important to note that areas of the body without hair do not respond well to most medical treatments. Hairless areas on the body in most people include the lips, fingertips, knuckles, underside of the wrists, feet, and parts of the genital areas. If your pattern of vitiligo predominantly involves these areas (such as vitiligo on the lips and fingertips, for example), then it is more difficult to treat. So, some doctors like to label the types because they know which ones have a better or worse prognosis with treatment based on their distribution in these areas. But I find that the forms don’t neatly fall into one of the clear designations, and I find it difficult to pick one. I think it’s much easier to simply call vitiligo “vitiligo”, and then tell the patient that hairless areas don’t respond very well to treatment. EXCEPT for segmental vitiligo (again, explained below). But then again, I’m a lumper, rather than a splitter, and maybe that’s why I feel the way I do.

Common types of vitiligo

Usually vitiligo manifests as multiple spots on the skin that are found on both sides of the body, most commonly in a symmetric pattern. So, if there is a spot on one side of the face, there is often a matching spot on the other side. If a spot is found near the center of the body, it will grow across the middle and involve the other side as well. Sometimes the spots are found only on mucosal parts of the body (like the lips, inner nose, or genital areas), and so the pattern is called mucosal vitiligo. If it involves the lips or other parts of the face plus the hands and/or feet, it’s called acrofacial vitiligo (“acral” means hands and feet). This is really common, since vitiligo most frequently affects the face and/or hands (over 80% of those with vitiligo). If affecting mostly the lips and fingertips, it’s called lip-tip vitiligo. If the vitiligo is only affecting a small area of the body, it may be called focal vitiligo, but I find this usually spreads and evolves into another form pretty quickly. The spots can spread a lot to involve large parts of the body all over, which is called generalized vitiligo. If it involves most of the body (some say more than 80% of it), it's called universal vitiligo – I’ve seen patients who have so much vitiligo that it was difficult to find areas of normal skin color because they were pretty much all white. Interestingly, most patients I’ve seen with universal vitiligo still have all the color in their hair, which is really interesting.

There are also different appearances that the spots themselves can have, even within the above forms defined by anatomical location on the body. Usually spots of vitiligo are pretty simple, appearing as a round and all white, surrounded by normal skin. There are 4 other appearances though, and all of these indicate that the spot is rapidly growing, or “active”, rather than stable. Think of the regular, round white spot as smoldering ashes after the forest fire has mostly burned out, while these 4 forms are a raging inferno that’s burning uncontrollably. This is important, because when I see one of these forms on the skin, I know the disease is very active and treat more aggressively to prevent the spread of vitiligo while the slower, but more effective, treatments start working.

Inflammatory vitiligo is one of these very active forms, which has a pink border of the white spot, sometimes with some scale appearing there, and it’s often itchy. The pink border is usually pretty subtle and easy to miss if you’re not looking for it. Trichrome vitiligo has three colors instead of two (“tri” means three, “chrome” means color), including the normal skin, the white center, and a lighter hypopigmented border between the two. Confetti vitiligo consists of many very small spots of vitiligo, usually 1-3mm in size, that are often clustered together in larger areas, about the size of a quarter or half dollar, or around the edges of an existing spot. Finally, the Koebner phenomenon describes when a spot of vitiligo follows an injury to the skin, such as a scratch, scrape, or burn. These spots take the shape of the injury, so they are not usually in the typically round shape, but could be a long line in the case of a scratch, for example. These appearances aren’t “types of vitiligo” per se, because they can change appearances quickly, from one to the other and then calming down and looking like the regular old white spot. Confetti vitiligo is a good example of this, changing from tens or hundreds of tiny white spots to a normal-appearing round spot when they grow together, usually in just a few months.

Segmental vitiligo – a special case

There’s one form of vitiligo, segmental vitiligo, that doesn’t follow the normal rules, and it is distinct from all of the other types listed above. It affects only one area of the body on only one side, without crossing the midline of the body on the front or back (usually). It is less common to have segmental vitiligo, which affects only about 5% of adults and 20% of children. Because this pattern is also characteristic of shingles, which typically affect the skin over just one nerve or “dermatome”, segmental vitiligo has been called dermatomal vitiligo, but this is incorrect. The pattern rarely, if ever, follows a true dermatome and often runs in an opposite direction to dermatomes, so we should not use that term.

Segmental vitiligo is unique, even beyond the fact that it doesn’t cross the midline. It spreads very quickly, faster than the other forms, but only for about 6 months (sometimes up to a year). It’s such a rapid pace that patients assume it will soon cover their entire body, but it stops abruptly and usually remains stable, without changing, forever after that. Segmental vitiligo also frequently turns the hair white in the affected spot pretty quickly, which is important because vitiligo spots with white hair usually don’t respond very well to treatment. That’s because the melanocyte stem cells, which can make more of the pigment-making cells, live in the hair follicles, but are most likely destroyed if the hair has become affected by vitiligo, turning white.

Based on this, you can imagine that segmental vitiligo doesn’t respond well to treatments unless they’re started very early, before the hair is affected. That’s the bad news about segmental vitiligo. Of course, the good news is that “what you see is what you get” after about 6 months, it doesn’t commonly spread to affect other areas after this. There are exceptions, of course, which I’ll talk about below (that’s called mixed vitiligo). The other piece of good news is that segmental vitiligo is very responsive to surgical therapy called the melanocyte-keratinocyte transplant procedure (MKTP), described in detail here. In fact, MKTP is often a cure for segmental vitiligo!

Mixed vitiligo is very rare and presents with a clear spot of segmental vitiligo that is on one side of the body and limited by the midline, but then also other spots on the body as well, which fit one of the other forms. So, this person has both segmental vitiligo and another form. We have found that the segmental part of mixed vitiligo behaves like segmental vitiligo (spreads quickly, difficult to treat medically, but responsive to MKTP), while the other spots act like the other forms, and are more responsive to conventional treatments.

Patterns of pigment return

Once we start treating the vitiligo spots, the pigment comes back, thereby reversing the disease. If there are pigmented hair follicles within the spots that we’re treating, after about 3 months of treatment we see little brown spots appearing around each hair. Because of the pattern, we call this perifollicular repigmentation, and this is the best pattern for reversing disease because each little brown spot grows over time, running into the other spots, and completely filling the former white spot. Sometimes I see this pattern even when someone isn’t intentionally treating their vitiligo, commonly in areas of the skin that are exposed to the sun. Patients often aren’t sure if this is pigment coming back, or just pigment that was left over as the white spot spread around it. I always tell them that it is definitely pigment returning, since white spots don’t leave spots of pigment behind when they spread. So, this is good news!

Even though the best pattern is perifollicular and areas without hair don’t respond well, they still do respond to treatment but in a different pattern, called marginal repigmentation. This is when the edges of the white spot creep in a little bit, shrinking the whole spot by a few millimeters. It doesn’t help a lot, but it does make the spot smaller, and can completely fill spots if they’re small to start with (less than 5mm diameter or so). Interestingly, when I was seeing patients years ago and had residents, or trainees, in my clinic with me, they would ask how I knew the bumpy edges meant the spot was getting smaller, rather than the reverse, growing larger. I didn’t know at the time, at least consciously, although I did know somehow, which I considered my “gut feeling”. It wasn’t until I read a very old textbook for vitiligo, an atlas written in the late 1800s, that I figured it out. The book actually said that if on the bumpy edge the pigmented part was rounded, then the spot was getting smaller, or repigmented. If the white part was rounded, then it was getting larger, or depigmented. I thought it was awesome that I learned something from a textbook over 135 years old! I think this is similar to what Malcolm Gladwell described in his book, Blink – when physicians knew things about their patients that they couldn’t put into words. They knew the information from having seen so many patients, but the data was in their subconscious somewhere!

Summary

Sometimes the above forms, except for segmental vitiligo, are lumped together and called “non-segmental vitiligo”, but I REALLY dislike this term. I think it’s silly to define a type of vitiligo by what it’s NOT, particularly since it is the most common form of vitiligo (80-95% of all cases). It also doesn’t have meaning for the majority of people who don’t know what segmental vitiligo is (not you, of course, because you’re reading this blog and will have learned all about segmental vitiligo). Because I don’t think that splitting vitiligo into the above forms is necessary or helpful, and because I so dislike the term non-segmental vitiligo, I usually refer to the many patterns as simply “vitiligo” and then distinguish the “segmental subtype”.

This describes the many faces of vitiligo, and now hopefully you can appreciate the patterns as I see them. And you know something about what the patterns mean, how they’ll respond to treatment, and how they’ll evolve (or not) in the future!