Brownish depressed atrophied patches on left upper lip and depressed left lower nasolabial fold. Note biopsy marks.

Faint depressed patch on her left temple noticed recently

Laboratory Data

A repeat skin biopsy on the left upper lip and the left nasolabial folds showed similar histopathology. The epidermis and dermis appeared normal with no evidence of morphoea. Direct immunofluorescence test was negative.

Biopsy on left upper lip: H&E (low power)

Biopsy on left upper lip: H&E (high power)

Biopsy: left nasolabial fold: H&E (low power)

Biopsy left nasolabial fold: H&E (high power)

1. What is the most likely diagnosis?
2. How do we arrest the progress of the skin lesions?
3. How do we treat this patient?
   

Joel Bamford MD, Duluth, MN, USA on Oct 18, 2005

Clinically agree with lupus panniculitis. Later developent of morpheaform features and + ANA would be likely. Without biopsy demonstration of panniculitis or LE, I think more time is needed. Clinical trial or treatment with plaquenil is said to produce response within 2-3 months for Lupus panniculitis.

Amira Adel MD, Egypt on Oct 18, 2005

The most likely diagnosis is DLE. She needs systemic antimalarial and sunblock + short course of topical medium potency steroid so as not to increase the atrophy

Khaled El-hoshy MD, Troy, Michigan, USA on Oct 19, 2005

I would consider Factitious Dermatitis. Atrophie macularis et varioliformis cutis is another remote possibility. Clinical picture is suggestive of DLE but pathology is not.

Andrew Carlson MD, Professor, Divisions of Dermatopathology and Dermatology, Albany Medical College, Albany, NY, USA on Oct 19, 2005

The upper lip biopsy shows angiofibromatous change in the superficial dermis pushing down and disrupting elastotic bundles. There are certainly collagen changes. The nasolabial fold bx shows a wedged-shaped scar. I don't think lupus or morphea are in the ddx based on the histology.

Jag Bhawan MD, Professor of Dermatology and Pathology, Boston University School of Medicine, Boston, Massachusetts, USA on Oct 20, 2005

It is not morphea histologically.

Rick Sontheimer MD, Professor and Vice-Chairman, Dept. of Dermatology University of Oklahoma Health Sciences Center, Oklahama City,OK, USA on October 20, 2005

Perhaps a repeat biopsy from the margin of the newest lesion with particular attention being paid to possible inflammatory changes in the subcutaneous tissue.

Haitham Alqari MD, New York, NY, USA on October 20, 2005

Nice case. The feature is clinically suggestive of DLE more than lupus panniculitis. I will consider using hydroxychloroquine after checking her vision and G6PD. Add Tacrolimus ointment and sun block. And repeat skin biopsy in the feature if the is no improvement.

Jeffrey Callen MD, Professor of Medicine (Dermatology), Chief, Division of Dermatology, Department of Medicine, University of Louisville School of Medicine, Louisville, KY, USA on Oct 21, 2005

The clinical picture looks most like DLE to me, but there is not an interface dermatitis on the biopsy. I think that the suggestion of hydroxychloroquine seems reasonable. I will be interested in hearing about follow up

Khalifa Shaquie MD, PhD, Professor of Dermatology, College of Medicine, University of Baghdad, Baghdad, Iraq on Oct 22, 2005

This is an interesting case so called En Coup De Sabre. This is still incomplete case,that is why the diagnosis is not easy. This is not infrequently seen in Iraq. The histopathology usaully shows scar unless taken from fresh active lesion. The management is by oral steroids and oral zinc sulfate 100mg three times a day for a long time to stop the progression of the disease.

Thomas Jayakar MD, PhD , Senior Consultant Dermatologist, Apollo Hospitals and KK Childs Trust Hospital, Chennai, India on Oct 22, 2005

I would like to draw the attention af all to the following reference:
Janjua SA, McColl I, Thomas J. Lupus panniculitis involvin breast and parotid/periparotid areas; a rare presentation. J Ayub Med Coll 2004;16:86-8

Shahbaz Janjua MD, Consultant Dermatologist, Ayza Skin and Research Centre, Lalamusa, Pakistan on Oct 22, 2005

Differential diagnosis for this eruption includes morphea panniculitis, traumatic panniculitis, and localized lipoatrophy. I would also suggest a repeat biopsy from the edge of an active/fresh lesion as the histopath findings are still inconclusive. I would go for a therapeutic trial of oral antimalarials plus topical steroids in such cases.

Azad Kassim MD, Hasa, KSA, Saudi Arabia on Oct 28, 2005

Chronicity of the lesions for years and being on one side of the face(left side) i.e left upper lip extending to nasolabial fold with presence of similar lesion on left temple and unremarkable specific histopathological findings, makes diagnosis of incomplete linear morphea most likely.
I would suggest avoidance of potent topical steroids to decrease subsequent skin atrophy. A trial of topical tacrolimus or calcipotriene may be a reasonable option of treatment with regular follow up.

Editor's Note on March 21, 2006

This patient had repeat biopsies done and was sent for histological examination and direct immunofluoresce studies. Unfortunately both the histology and direct immunofluorescence were negative. She was started on hydroxychloroquine and awaiting response.