34 year old woman with unilateral receding cheek

presented by

Henry Foong FRCP

Ipoh, Malaysia

on September 20, 2005

Consultant Dermatologist, Foong Skin Specialist Clinic, Ipoh, Malaysia

Abstract The patient is a 34yo woman with unilateral receding cheek. She is suspected to have lupus panniculitis.
34-year-old woman
2 years

This 34 yo woman initially presented with a swelling on the left cheek which subsequently shrunk and receded till it became a shrunken deformity. Subsequently she noticed a similar swelling on the right cheek and was mildly tender. She had no fever, polyarthrlagia or Raynaud's symptoms.

She had been investigated earlier at a local hospital.

Physical Examination

Left cheek: marked lipoatrophy on the left cheek till contour of maxillary bone appeared prominent.

Right cheek: indurated swelling 1.5 by 1.5cm on right cheek, mildly tender and best felt on deep palpation.

Scalp: patch of scarring alopecia on the frontal scalp.


Laboratory Data

awaiting serology

Diagnosis Lupus erythematosus panniculitis
Reasons Presented

The management of lupus panniculitis in this patient poses a therapeutic challenge. One could use oral prednisolone and hydroxychloroquine to control the disease activity but refilling the lipoatrophy (after the disease activity has become settled) with a filler would be a challenge.



How would you approach this patient?


Ng PP, Tan SH, Tan T "Lupus erythematosus panniculitis: a clinicopathologic study" Int J Dermatol. 2002 Aug;41(8):488-90.

National Skin Centre, Singapore.

BACKGROUND: Lupus erythematosus panniculitis is a clinical variant of lupus erythematosus which involves the deep dermis and the subcutaneous fat. The purpose of this study was to ascertain the clinical profile of Asian patients with this condition.

METHODS: This was a retrospective study of all histologically confirmed lupus panniculitis seen at our center between 1992 and 1997. The age, sex, past history/subsequent diagnosis of systemic lupus erythematosus (SLE), presence of clinical discoid lupus erythematosus (DLE) changes on overlying skin, direct immunofluorescence, serologic, and histologic findings were analyzed.

RESULTS: There were 12 cases of lupus panniculitis, two of which were in patients already diagnosed with SLE and one in which the patient subsequently evolved into SLE. The mean age at diagnosis was 31.3 years. The face (50%), upper limbs (33%), and scalp (25%) were the most common sites of involvement. Thirty-three per cent had clinical evidence of DLE on the overlying skin, whilst 67% had histologic features of DLE on the overlying skin. A lupus band was present in 36%. Antinuclear antibody (ANA) was positive in three of 11 cases; these were in the two patients who already had SLE and in the only patient who progressed to SLE. All of the cases showed fat necrosis and, in the majority of cases, there was associated lobular and paraseptal inflammation. Thirty-three per cent showed lymphocytic vasculitis and 75% had mucin deposition. None had lymphoid nodules, subepidermal hyalinization, or calcification.

CONCLUSIONS: Lupus panniculitis affects a younger age group in Asians as compared with the Western population. Although about one-third of patients show clinical evidence of overlying DLE, two-thirds of patients show histologic evidence of DLE. It tends to have a mild disease course in the majority of cases.

Comments from Faculty and Members

Khalifa Shaquie MD, PhD, Professor of Dermatology, College of Medicine, University of Baghdad, Baghdad, Iraq on Sept 20, 2005

This is an interesting case of lupus profundus. We have similar experience with this condition comparable to Dr Foong report. This patient should be treated urgently to prevent or minimise the deformity in the right cheek. Regarding the the left deformity,the best solution is to inject with fat taken from buttock or abdominal wall. I have personal experience of injecting fat to a similar deformity and gave very nice cosmetic appearance for 3 years follow up. Although fat can resorb but could be repeated and reach a permanent status without any side effects that could be seen with other fillers.

Mohsin Ali MBBS, MRCP, Amersham, United Kingdom on Sept 20, 2005

Good work. Keep it up!

David Elpern M.D. Williamstown, MA, USA on Sept 20, 2005

I saw a similar patient a few months back.

She is a 64 yo woman who presented for a wart on a finger. Around 30 years ago, after a pregnancy, she developed facial lesions which drained pus for many years. It was called DLE and eventually healed with impressive facial lipoatrophy. She has some scarring alopecia, too. In addition, she has calcinosis cutis on arms and thighs (none have ulcerated recently). Her "lupus" has burned out and she has had not meds for > 20 years.

Thought these pictures might interest VGRD members.

Shahbaz A Janjua MD, Ayza Skin & Research Centre, Lalamusa, Pakistan on Sept 20, 2005

An excellent case presentation of lupus profundus. It reminds me of the case we (Dr.Ian McColl, Dr.Jayakar Thomas, and I) reported two years earlier. It was in fact second case report in the literature involving the periparotid and parotid regions in addition to the typical areas. I treated that patient with oral hydroxychloroquine and topical steroids. The disease progression halted after a few months of treatment but the facial disfigurement remained a challenging task to deal with. I referred the patient to a plastic surgeon to take care of the facial atrophic scars. In my opinion collagen fillers are worth trying in such cases.
You may access the case here. http://www.ayubmed.edu.pk/JAMC/PAST/16-4/Shahbaz%20CR.htm

Khaled El-hoshy M.D., Troy, Michigan, USA on Sept 20, 2005

Once diagnosis established, autologous fat transfer may be an option after disease control. Repeated sessions every 3-9 months may be needed.

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

I would normally obtain histopathologic confirmation of a presumed diagnosis of lupus panniculitis/lupus profundus, even knowing that a biopsy site in a lupus panniculitis lesion can at times be quite slow to heal. However, if the scarring alopecia of her scalp was found to be due to histopathologically-confirmed discoid LE then I think that it could be safe to assume that the swelling of her right cheek represented active lupus panniculitis while the depressed area on her left cheek represented localized lipoatrophy resulting from previous lupus panniculitis activity.

I personally have had good luck was single agent or combined antimalarial therapy for active lupus panniculitis (especially in non-smokers). However, appropriate time intervals (6 wks-3 mos) must be given to allow these drugs to work maximally. Thalidomide can also be useful in rapidly calming lupus panniculitis inflammation. Because of the depth of the lipoatrophy on her left cheek one might initially consider autologous fat transfer rather than artificial fillers.

However, caution should be taken with any type of cosmetic manipulation in lupus panniculitis for the fear of surgical trauma-induced ulceration and/or Koebnerizatin. It would be best to have all evidence of lupus panniculitis/discoid LE activity suppressed medically before cosmetic revision is considered. When weighing the cost benefit of cosmetic manipulation of lupus panniculitis,

It should be kept in mind that even therapeutic intralesional corticosteroid injections have been associated with ulcerative breakdown of lupus panniculitis lesions.

Julian Manzur M.D., Havana, Cuba on Sept 23, 2005

I have seen significant atrophy after high concentration administration of intralesional triamcinolone on plaques in patients with discoid lupus erythematosus.
Of course, this is not related with this patient.

Robert I. Rudolph, M.D., FACP, Clinical Professor of Dermatology, University of Pennsylvania, Philadelphia. PA, USA on Sept 25, 2005

My impression is that this patient has "Parry Romberg disease". While I guess LE could cause this, I would be skeptical.

Abir Saraswat MD, Lucknow, India on Sepy 26, 2005

I have treated a woman recently whose case exemplifies the problem of plastic surgical correction of these deformities.

She had recurrent nodules on both her cheeks for the last 3 to 4 years which were subsiding in several months with lipoatrophy. Dissatisfied with medical treatment, she went for excision of one of the nodules which was done by a plastic surgeon and repaired with a rotation flap. Within 2 months, a new nodule developed right on the incision line and was followed over the next few months by more nodules on other points on the margin.
She was subsequently treated with low dose oral steroids and Azathioprine. Therapy was stopped after 6 months and she has been asymptomatic for 6 months now.
In light of this, I feel that any surgical correction of lipoatrophy should only be done well after complete cessation of activity of the disease. Prof. Sontheimer's reminder of caution with any intralesional manipulation is especially apt in the spirit of primum non nocere.

Henry Foong's additional note on Sept 26, 2005 :

The serology of the patient's ANA was 1:160 (nucleolar pattern). Her dsDNA Antibody was negative. This makes the clinical diagnosis of lupus profundus / panniculitis most likely. I would agree that the disease activity must be quiscent before contemplating on any surgical procedure. Autologous fat transfer would be more favourable in view of the depth of the lipoatrophy.

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