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Skin sarcoid

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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). 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 very sensitive but medications and alcohol can elevate). 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:

  1. Katta R, "Cutaneous Sarcoidosis: A Dermatologic Masquerader", Am. Fam. Physician, 65(8):1581-84, 15 April 2002.
  2. 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 2007)