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Skin
sarcoid
[NOTE: you are now on a web page in Dr. Shaw's
personal website]
On hearing that you may have sarcoid, ponder
whether you have recently noticed any vision changes or eye pain (sarcoid could be in the
eye...a sort of emergency situation), any heart problems like skipping beats or unusual rate
changes or almost-fainting spells (sarcoid could be in the heart...a kind of emergency
situation), or any new-type headaches or mental changes (sarcoid might be involving the brain
and associated coverings...maybe a sort of emergency). Note the death of this pro football player, HERE. Having none of these complaints, note
the following.
Sarcoidosis is treated if it is causing
problems!...(as of April 2006) I would resist body-wide (systemic) treatment
with things like steroids if there is no problem. Quite a percentage of sarcoid cases just
"burn out" and go away in due time. So, being "followed" by your doctor seems a reasonable
tactic (expectant management)...as long as there are no problems.
One source indicates that over 70% of those
diagnosed with sarcoidosis find that the disease gets better within 2 years, whether treated
or not (but...in the face of problems...treatment wards off permanent damage...such as
eyes...& complications). Some 20-35% of cases of systemic sarcoidosis1 present as skin lesions. When pathologists make a diagnosis of
sarcoid on a skin biopsy (being a specific diagnosis), it is very important that they have
also looked for foreign-body material (they might do a "polarized light exam") and offered an
opinion as to whether the granulomatous reaction might be related to infection (they might do
bacterial and/or fungus stains or discern by types of cells not present). In cases of
granulomatous folliculitis, other causes of damaged skin follicles which can extrude contents
into skin and generate a perifollicular granulomatous reaction (S-04-13280) must be
considered.
When you have a skin problem and
a biopsy is done and your doctor tells you that you have cutaneous (skin) sarcoid, what do
you do? First and foremost, you pay careful attention to your/that doctor's advice. If that
doctor was your dermatologist and you have a regular doctor (your primary care doctor), you
need the primary care doctor in the information loop, too (and he/she may refer you to
another specialist such as an ophthalmologist or pulmonologist) to work through this. The
rapidity with which you work through further evaluation depends on whether you have any other
health complaints or not. If you are ill, you need to move quickly (but it is almost always
not an emergency). A significant percentage of patients presenting with sarcoidosis
have no complaints at all (are asymptomatic). In all of this, you must be honest about
whether you really feel normal or not.
A skin diagnosis (or any other organ, for that
matter) of "sarcoid" is a presumptive diagnosis, not an absolute diagnosis; and it
implies that you have "sarcoidosis" (systemic involvement). So, it
must be initially presumed that the disease is also affecting other organs. If the disease is
eventually documented to be present in other organs, then you would be categorized as having
"sarcoidosis"...a potentially serious disease...but see 1st sentence on this page again. If
not documented in other organs, your case diagnosis continues as skin-only sarcoid (one
study2 indicates that about 30% of skin-only cases becomes systemic sarcoidosis 6 months to 3
years later...the other 70% already being systemic) because other causes are felt to have
been excluded...sarcoid being a "diagnosis of exclusion". Here is a good website about
sarcoidosis: http://www.emedicine.com/DERM/topic381.htm and another website by a physician, which
includes a listing of doctors thought to be sarcoidosis experts http://www.sarcoidcenter.com/ and another http://www.sarcoidconnection.com/ with many helpful links, including
Dr. Judson's sarcoid program at MUSC, Charleston, S. C..
But, what for you to do long term? The deal is
that your doctor (s) need to figure out whether you have presumptive [localized]
sarcoid or [systemic] sarcoidosis. Textbooks indicate that
20% of sarcoidosis cases begin as skin only, and an "occasional" skin-only case will go away
on its own (spontaneously remit). After all of my checking, here is what I'd do if it were
me:
- this assumes that
there is only a skin lesion & biopsy compatible with sarcoid...just a presumptive
diagnosis (the conventional rule is that sarcoidosis is only absolutely diagnosed when one can
convincingly say that 2 or more organ systems are involved). Some advocate a biopsy of one of
the oral minor salivary glands before judging a case as "skin
only".
- check
temperature...sarcoidosis may only have fever as the additional finding; unintended weight loss
may be the only other finding.
- I'd get a blood CBC,
a urinalysis, and blood chemistry panel that includes the liver function tests ALT (SGPT),
alkaline phosphatase, and GGT/GGTP (GGT/GGTP are very sensitive, but medications and alcohol can elevate). CRP can be elevated. And it should
test serum calcium (if calcium is elevated, 1 alpha, 25(OH)2D3 is the main cause for
hypercalcemia in sarcoidosis and overproduced by sarcoid granulomata and/or
sarcoid-influenced pulmonary macrophages). Gamma-interferon produced by activated
lymphocytes and macrophages plays a major role in the synthesis of 1 alpha, 25(OH)2D3. PTH
release is down regulated by high serum concentration of 1 alpha, 25(OH)2D3.
Parathyroid-hormone-related protein may also contribute to the hypercalcemia of sarcoidosis.
Treatment of hypercalcemia and hypercalciuria consists of a low calcium diet, adequate
hydration, minimization of exposure to sunlight and reducing overproduction of 1 alpha,
25(OH)2D3. Prednisone, 15 to 25 mg/day, is the drug of choice to reduce the overproduction
of 1 alpha, 25(OH)2D3. If not corrected by prednisone, there is an increased incidence of
hyperparathyroidism in sarcoidosis and the problem may be parathyroid). Some would add on a
24 hour urine for calcium if serum calcium normal, in order to see that there is not
normocalciuria due calcium dilution within increased rates/volume of urine
excretion.
- If all normal, then
it still seems like skin-only sarcoid; so, I'd get a chest X-ray to be sure no evidence of
"hilar adenopathy" (lymph node involvement). If X-ray shows hilar adenopathy with/without lung
spots, there may be lung involvement.
- If chest X-ray is
normal, it is more evidence of skin-only sarcoid. I'd then get a blood test for ACE (the ACE level reflects the "load" of [sarcoid] granulomas in your
body...and is a "backstop" test against the possibility of "hidden" deposits of sarcoid in
bones or spleen...Angiotensin Converting Enzyme [ACE]). As detailed in the above link, there
are other causes of elevated ACE levels.
- As an additional
measure to guard against granulomatous infectious diseases that are just showing up in a
skin biopsy, a lung biopsy, or such as an endometrial biopsy [S-04-13928], one might also order
an hs-CRP (which is likely seriously elevated in the face of such
infections)...stable or even progressing, mild sarcoidosis can have CRP less than
8...but very likely above 5...and sarcoidosis unlikely if below 5 mg/L13; but Lofgren's syndrome (bihilar adenopathy, arthritic symptoms,
and erythema nodosum) has elevated CRP levels in the range of 47-61 mg/L13. And I'd have a TB skin test done..."positivity" would suggest
M. tuberculosis infection somewhere (while negativity would not rule out atypical
mycobacteria).
- I'd also get an eye
exam by a physician skilled in "eye grounds exam" with pupils dilated. If found to have sarcoid
of skin and eye only, you'd absolutely want treatment without delay to protect your
vision.
If all of the above are
normal, then the skin lesion is "granulomatous dermatitis, compatible with
skin-only (at least as of that point in time) sarcoid", being a
specifically diagnosed abnormality. Another skin problem often reflecting or heralding sarcoidosis
is the acute skin lesion, erythema nodosum (not being an entity specific for sarcoid).
Skin-only sarcoid just gets topical ointment or lesion injection (local...not
systemic...treatment).
I am unable to get precise information on what
percentage of patients presenting with skin only sarcoid stay stable and never have any
further trouble and only have to have topical or injection treatments of the skin spot. Said
another way, I can't give a confident answer to these two questions: "Of 'skin biopsy
compatible with sarcoid' cases presenting as cutaneous (skin) sarcoid, what percentage would
you estimate are found to be negative for evidence of systemic involvement on further
work-up? And, of those negatives, what percent stay systemically
negative?"
References:
- Katta R, "Cutaneous Sarcoidosis: A
Dermatologic Masquerader", Am. Fam. Physician, 65(8):1581-84, 15 April
2002.
- Mana J, et. al., Cutaneous Involvement in
Sarcoidosis, Arch. Dermatol. 133:882-888, July 1997.
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(posted 15 September 2004; latest update 15 April
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