Sunil Dogra MD, Associate Professor of Dermatology,
Postgraduate Institute of Medical Education and Research, Chandigarh,
India on June 17, 2007
An interesting case indeed. I would consider clinical conditions
- CTCL, Sarcoidosis and Histiocytosis.
David Elpern MD, Consultant Dermatologist, Williamstown,
MA, USA on June 17, 2007
This is a unique case. Do you actually see sarcoid in Malaysia?
If so, perhaps this is it. Her case may be "an experiment
of one." I'd recommend showing some photomicrographs to
some of our dermpaths and then considering therapy. Here's a
recent review from the JAAD (which I think you receive) Ted
Rosen (one of the authors) would comment, I am sure. Please
keep us posted. There are some articles on TNF drugs for sarcoid,
but I haven't reviewed them.
This is a very unusual case. There have been some cases of
CTCL which presented with a sarcoidal-like pathology. Please
see the references affixed. Immunostaining may be of value.
Badgwell C, Rosen T.J. Cutaneous sarcoidosis therapy updated.
Am. Acad. Dermatol. 2007 Jan;56(1):69-83.
The widely accepted standard therapy for cutaneous sarcoidosis
corticosteroids, antimalarials, and methotrexate. However, a
of the basic immunopathogenic properties of sarcoidosis has
elucidated a number
of steps critical to the persistence and progression of disease
that may be
vulnerable to treatment by targeted therapy. This article reviews
and newer therapeutic options for cutaneous sarcoidosis.
Omid Zalgari MD, Rasht, Iran on June 17, 2007
My first diagnosis for this very interesting case would be
a variant of CTCL, most probably a granulomatous MF and perhaps
an unusual form of disseminated granulomatous slack skin. Many
thanks for sharing this case.
Ian McColl FRACP, Consultant Dermatologist,
John Flynn Medical Centre, Tugun, Queensland, Australia on
June 17, 2007
Clinically you would more favour a T cell lymphoma but sarcoidosis
can clinically mimic about anything. Was the ACE level raised?
You can get a granulomatous T cell lymphoma but is ACE raised
then as well?
Rick Sontheimer MD, Professor of Dermatology, Department
of Dermatology, University of Oklahoma Health Science Centre,
Oklahoma City, OK, USA on June 18, 2007
Agree with CTCL of granulomatous MF subtype.
Bushan Kumar MD, Former Professor of Dermatology, PGIMER,
Chandigarh, India on June 18, 2007
I would consider granulomatus MF as the only possibility. Assessment
about involvement of other organ system should be made to decide
about the therapeutic approach.
Abir Saraswat MD, Consultant Dermatologist, Lucknow,
India on June 18, 2007
Granulomatous CTCL is a strong possibility, but it is unlikely
that the histopathology would be described as "multiple
granulomata with giant cells." It should be more like band-like
infiltrate of lymphocytes (atypical or not) admixed with giant
cells and some histiocytes. Some of the photographs are strongly
reminiscent of a patient with ichthyotic sarcoidosis that I
saw some years back.
A histopath review should clear up the issue; perhaps Dr Choon
could upload some photomicrographs. A very interesting case
worth getting to the bottom of.
Shahbaz Janjua MD, Consultant Dermatologist, Ayza Skin
& Research Center, Lalamusa, Pakistan on June 18,
This is a great case. I would consider MF, sarcoidosis, amyloidosis,
lepromatous leprosy, disseminated leishmaniasis, atypical mycobacteriosis,
and deep fungal infection on clinical grounds.
Khalifa Sharquie MD, Professor of Dermatology, College
of Medicine, University of Baghdad, Baghdad, Iraq on
June 19, 2007
Well, it is a very interesting case. The overlying skin changes
are poikiloderma-like which are unusual to be seen in sarcoidosis.
And sarcoidosis is very rare in Asian people. Granulomatous
reaction could be seen in mycosis fungoides. Accordingly the
whole picture is suggestive of lymphoma. So please do Kveim
test to excude sarcoidosis and do full investigations to confirm
Sam Moshella MD, Clinical Professor of Dermatology,
Emeritus, Harvard Medical School, Boston, USA on June
I believe the patient has granulomatous MF. It would be interesting
to know whether elastic tissue stains were done to support the
diagnosis of Slack skin MF.
Choon Siew Eng FRCP, Senior Consultant Dermatologist,
Johor Bahru, Malaysia on June 21, 2007
Update on the case. Thank you for your contributions.
We are repeating the full panel of immunostains for lymphoma,
CD1a and elastic stain. CT scan from base of skull to pelvis
also did not reveal any LN or organomegaly. Will get the histology
slides up for VGRD soon. Will keep you all informed of results.
Update on HPE on June 24, 2007
Biopsy from abdominal lesion showed numerous granulomata composed
of epithelioid histiocytes, lymphocytes and multinucleated giant
cells, Langhan’s type. Special stains for Acid fast bacilli
(ZN, Wade fite) and fungal bodies (PAS) were negative. No abnormal
deposits of eosinophilic amorphous material seen. No foreign
body seen by polarised light. (slide courtesy of Dr Ghazali)
Andrew Carlson MD, Professor, Divisions of Dermatology
and Dermatopathology, Albany Medical College, Albany, NY, USA
on June 2007
Predominately intertriginous involvement that is slightly pendulous
and erythematous associated with patches of ichthyosis (acquired
ichthyosis due lymphoma or sarcoidosis?). The histology can
be best classified as diffuse interstitial granulomatous dermatitis
with numerous multinucleated giant cells. The associated lymphocytes
are small, but hyperchromatic. I don't see any regions of epidermotropism.
Overall, I am most suspicious for "granulomatous slack
skin syndrome" a variant of mycosis fungoides. The differential
diagnosis is sarcoidosis; sarcoid should be diagnosed only after
you have excluded lymphoma (look for gene rearrangement and/or
abnormal immunophenotype) and infection (mycobacterial
infection, deep fungus, leishmaniasis). What does her CXR show?
Elevated ACE or Calcium level?
Thanks for sharing the histology with me.
Update on the case by Choon Siew Eng FRCP on
Thank you for your contributions. Confirmed GSSD. Her bone
marrow and blood film normal and my haem colleague has discharged
her back to me. I am thinking of putting her on interferon first
like my other patients before resorting to CHOP. There are 42
cases reported. Mainly caucasians and male. Any one with experience
with pentostatin? I would appreciate treatment advice