Pseudo vitiligo in Systemic Sclerosis

presented by Henry Foong FRCP,

Ipoh, Malaysia on April 5, 2004

Consultant Dermatologist, Foong Skin Specialist Clinic, Ipoh, Malaysia
 
Name
C.L.Y.,48-year-old chinese woman
Duration
3 years
Distribution
Face, hands, legs
History

This woman has a history of multiple hypopigmented macules on her shins for 3 years. She also noticed small vascular spots on her chest which spread to face and neck. She also experienced Raynaud's phenomenon, tightening of mouth and swelling of her digits. Lately she had difficulty in swallowing.
Physical Examination

Her main findings were multiple telangiectasia on face, neck and anterior chest wall. Fingers and hands were swollen with sclerosis of the overlying skin of the fingers. Multiple hypopigmented macules on the shins of both legs. No ulcers or gangrene noted. Mouth appeared constricted and have difficulty in opening. Bilateral polyarthropathy esp proximal and distal IP joints in both hands. BP was 120/85mmHg. No proximal myopathy noted. Lungs were clear.
Images

Laboratory Data

Anti-nuclear Antibody: 1:640 (speckled pattern)

Rheumatoid Factor: 137IU/ml

RNP positive

SS-A (Ro) negative SSB-B (La) negative

Sm negative Scl-70 negative

Jo 1 negative PCNA negative

Renal function and urine FEME - normal
Histopathology

The biopsy was taken from one of the hypopigmented macules
Diagnosis
Pseudo vitiligo in Systemic Sclerosis
Comments and References

This patient has an unusual feature of vitiligo-like macules on her lower legs and a serology result which is almost suggestive of mixed connective tissue disease. CREST could be one of the differentials as she has some of its features.

This is a difficult and challenging case to treat. Nothing actually works in terms of improving or modifying the disease except for calcium channel blockers for Raynaud's. I am hesitant to use prednisolone unless it improves the outcome of the disease. The side effects may outweight the benefit.

References:

  1. Sanchez JL, Vazquez M, Sanchez NP. Arch Dermatol. 1983 Feb;119(2):129-33. "Vitiligolike macules in systemic scleroderma." (view abstract)
  2. De Villiers WJ, Jordaan HF, Bates W. Clin Exp Dermatol. 1992 Mar;17(2):127-31. "Systemic sclerosis sine scleroderma presenting with vitiligo-like depigmentation and interstitial pulmonary fibrosis." (view abstract)

Comments from Members:

Jag Bhawan MD, Professor of Dermatology and Pathology, Boston University School of Medicine, Boston, Massachusetts, USA on April 5, 2005

It would interesting to stain with Mel-5 or some other melanocytic marker to evaluate epidermal melanocytes; whether they are absent or reduced in hypopigmented areas. It will be great to compare those findings with normal appearing skin of the same patient of similar anatomic area.

Victoria P Werth, M.D., Associate Professor of Dermatology, University of Pennsylvania School of Medicine, Philadelphia, PA on April 5 2004

She is an interesting case. She may have rheumatoid arthritis overlapping with CREST. You may want to get x-rays of her hands, since if she has RA, she may benefit from a TNF inhibitor (which may work in sclerosing conditions). You may want to get PFTs with diffusion capacity and a cardiac ECHO. Pulmonary hypertension can occur at this stage in CREST and there are good treatments for this.

Julian Manzur MD, Havana, Cuba on April 6, 2004

I think postinflammatory response in the area of sclerosis may explain the hypopigmentation seen in this patient

Doug Johnson MD, Dept of Medicine, University of Hawaii School of Medicine, Honolulu, HI, USA on April 7, 2004

Nice case and well worked up. I have a similar case that is on infliximab with improvement in RA symptoms but no help with the skin. Pigment problems are probably post inflammatory. Did you look with a Wood's lamp?