Evolving CTCL in a 23 yr old man

presented by

David Elpern M.D., Williamstown, MA, USA (1)

Meera Mahalingam, MBBS, PhD, FRCPath, Boston, MA, USA (2)

March 14, 2010

(1) Dermatologist, The Skin Clinic, Williamstown, Massachusetts, USA
(2) Professor of Dermatology, Pathology and Laboratory Medicine at Boston University School of Medicine.

 
Abstract

23 yo man with progressive induration of pubis and genitalia x 1.5 years

Patient
23-year-old man
Duration
1.5 years
Distribution
Pubis, genitalia and right foot
History

This 23 yo man had pityriasis lichenoides chronica (PLC) 5 - 6 years ago.  It resolved with topical corticosteroids and he had no cutaneous complaints except mild pruritus and dermatitis three years ago which resolved after a two week course of desoximetasone cream.  He was prefectly well until ~ 1.5 years ago when he developed asymptomatic scrotal swelling.  He consulted a urologist on a number of occasions and had two scrotal ultrasounds and a surgical exploration of the scrotum.  No diagnosis was made.  He presented for a dermatologic opinion on February 8, 2010.  He is single, lives alone and is an apprentice electrician and a good historian.

Physical Examination

The patient is a healthy-appearing 23 year old.  There is impressive induration and erythema of the lower abdomen, pubis and groin.  The skin is so indurated that one can not appreciate the inguinal lymph nodes.  His scrotum is woody and infiltrated both clinically and to palpation.  The penis is normal in appearance.  There are a few hyperpigmented plaques on the right foot

Images

Laboratory Data

 

Histopathology

The specimen shows a dense pandermal infiltrate of atypical, hyperchromatic lymphocytes and intermittent epidermotropism with rare Pautrier microabscesses.  Immunohistochemical stains show the infiltrate to be composed of CD3+ cell phenotype with scattered CD20+ cell lymphocytes.  Large cell transformation is not noted.  Pathologist's Diagnosis is CTCL (Tumor Stage)

Diagnosis

CTCL - Tumor Stage in a 23-year-old man with a history of PLC.

Reason for presentation

He will be worked up at this point; but I need to decide whether to refer to a dermatologic center or a general oncologist.  We are in a rural area and he may need to be seen at an institution which has more expertise with a case such as this.  However, it may be easier for him to get his scans here and then see an oncologist with a special interest in CTCL

The patient gave informed consent to DJE to present his history and findings on VGRD which may assist in his care.
Questions

How would you proceed from this point?

References

CTCL:  eMedicine  http://emedicine.medscape.com/article/1098342-overview

PubMed has 8000 references to CTCL and 5000 to Mycosis Fungoides.  The literature is too extensive for any generalist.  These patients are best managed by dermatologic oncologists.
Keywords

cutaneous T-cell lymphoma

Comments from Faculty and Members

Robert I. Rudolph, M.D., FACP, Clinical Professor of Dermatology
University of Pennsylvania, Philadelphia, PA, USA on March 14, 2010

Great, instructive, yet scary case.

This fellow probably would benefit from aggresive PUVA and/or topical Targretin. He may need some radiation (yes, I know he has it on the scrotum, but he still may need it there).

I'd consult Peter Heald at Yale, or Alain Rook at Penn - both CTCL mavens.

Fadi Hajjaj MBBS, DDSc, MSc, Postgraduate Doctor (MD Degree), Department of Dermatology, Cardiff University, Cardiff, UK on March 15, 2010

Thanks for this interesting case presentation. This is a typical text-book case of CTCL. Suggest follow up all patients with the diagnosis of pityriasis lichnoides chronica (PLC) and Lymphomatoid papulosis (LP)

Sunil Dogra M.D. , Assistant Professor, Dermatology, PGIMER, Chandigarh, India on March 19, 2010

Transformation from PLC to MF is uncommon. However, we have seen this phenomenon in 3-4 young adolescent patients in last 5 years at our center. I would recommend to start on systemic low dose methotrexate (15mg/week) along with topical steroids or bexarotene.

Baseline investigations including USG abdomen/pelvis/scrotum, blood cell counts should be done. It is important to keep patient on long term follow up.

Please Click Here To Comment and Evaluate

Back to February 19, 2010 Case