UNKNOWN CASE -  MYALGIA AND DERMATITIS
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  I recently saw a case in consultation which has my entire department 
puzzled:  a 38-year old caucasian man with a six-month history of 
progressive dysphagia, 80-pound weight loss (documented with pictures), 
some muscle weakness, and a psoriasiform, thick, scaly eruption 
symmetrically over the knees, elbows, hands, feet, glans (no scale). 
Previous medical history and family history were unremarkable (negative 
for psoriasis, as well).  Incisional biopsy of the scaly lesions was 
consistent with psoriasis/Reiter's, with a mention made by our 
dermatopathologist of acrokeratosis paraneoplastica.
  Studies:  ESR 120, LFT's roughly normal, CBC normal, EMG and muscle 
biopsy diagnostic of myositis, CK 3000, esophagus nearly atonic, workup for 
internal malignancy negative.  He was on the rheumatology service, and 
was checked for nearly every antibody known to man, with only his ANA 
positive (around 1:120, I believe...).  Jo, Sm, La, Ro, dsDNA, centromere 
antibodies were all negative
  Our impression was psoriasis associated with an underlying ? autoimmune 
problem.  The rheumatologists are calling the case dermatomyositis, 
despite a good skin biopsy with no changes suggestive of DM.  Has anyone 
heard of this type of explosive psoriasis, or any other associations 
which may be relevant?  Any advice or resources would be appreciated 
 
 

Chris Scholes MD
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On Tue, 12 Mar 1996, Chris Scholes wrote about a 38-year old caucasian man with 
a six-month history of progressive dysphagia, 80-pound weight loss, muscle 
weakness, and a psoriasiform, thick, scaly eruption over the knees, elbows, 
hands, feet, and glans penis. 

I suspect you are right to think that this is acrokeratosis paraneoplastica and 
polymyositis related to an as yet undiagnosed internal malignancy. The 
involvement of the knees, elbows and glans penis does sound more like garden 
variety psoriasis, however, the 80-pound involuntary weight loss makes it a 
malignancy until proven otherwise to me. It is not unusual for the skin findings 
of a paraneoplastic dermatosis to manifest prior to the actual diagnosis of the 
causative malignancy; this will probably show itself within a few months. I 
would continue to follow him closely and have a low threshold for more 
diagnostic work-up, even if you feel you have had an adequate look for the 
present--MN

Mark Naylor, M.D.
Fleishaker Assistant Professor for Dermatology Research
Center for Molecular Medicine
University of Oklahoma Health Sciences Center
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Did you check for HIV positivity?  This is a classic presentation for HIV 
associated psorisis.

Claire L. Haycox M.D., Ph.D
---------------------------

 
I guess I should have mentioned that -- HIV negative, with no history of 
risk factors...but that thought did cross our collective mind.

 

Chris Scholes MD
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Diagnosis is dermatomyositis.  I have seen the exact same situation in a
patient with metastatic melanoma.  Psoriasiform eruption does occur with
dermatomyositis, especially scaling of the scalp.  Muscle biopsy?, EMG?

John Zone MD
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In response I would favor Basex syndrome and look hard to find a malignancy.
 
Pat Condry
Rochester, NY
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How about acrodermatitis enteropathica?   A little zinc deficiency coupled
with other dietary deficiencies might explain this.  Multivitamins and
Sustacal for a therapeutic start.

Daniel Mark Siegel MD, MS(Management and Policy)
Assoc Prof of Derm
SUNY @ Stony Brook
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Muscle biopsy and EMG were both suggestive/diagnostic for dermatomyositis 
-- but skin biopsy was read by three dermatopathologists as psoriasis -- 
no changes for DM.  The patient certainly has polymyositis, but we aren't 
convinced that that is "the whole story."   

Chris Scholes MD
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