Skin , STD & Leprosy


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Priyam Kembre, Uday Khopkar


A 27 year old unmarried female presented with painful red raised lesions over the left ear and cheek since 1 month and painful red raised lesions on the scalp with loss of hair over the lesions since 15 days. She also complained of polymenorrhea. She was diagnosed as cutaneous tuberculosis elsewhere and started on antituberculous treatment for 15 days.

Fig. 1	Fig. 2

Examination revealed well defined indurated tender erythematous plaques 3x3 cm on the left cheek (which had ulcerated following biopsy, Figure 1) and a well defined erythematous tender plaque 7x5cm over the left ear. A similar plaque measuring 3x2 cm was present over the right cheek (Figure 2). Systemic examination was normal.

The investigations revealed a normal hemoglobin and WBC count. Her urine examination was normal and the liver and renal function tests were within normal limits. Antinuclear antibody was positive (1:80) but anti-dsDNA was negative. Complement levels were normal. Histopathology of the lesion revealed a superficial and deep, perivascular, periadnexal infiltrate (Figure 3) of lymphocytes with abundant mucin deposition throughout the dermis (Figure 4).

Fig. 3	Fig. 4

What is your diagnosis?

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Answer :

Diagnosis
Tumid lupus erythematosus
Tumid lupus erythematosus is a variant of discoid LE that has no epidermal changes. This is reflected in the clinical features as smooth surface without scaling or hyperkeratosis.

Treatment

Patient was started on Prednisolone 40mg and Azathioprine 100mg. The lesions healed without scarring and with residual pigmentation

Fig. 5

Discussion
Lupus tumidus is an unusual variety of cutaneous LE(1) in which the infiltrate occurs primarily in the deeper portions of the dermis with rare epidermal changes.

Clinical presentation
Firm, sharply demarcated nodules lying beneath clinically normal skin are seen (2). Typical lesions of DLE may be found elsewhere, most frequently on the cheeks, arms, hands, breasts, buttocks, trunk and legs. Healing leads to the development of depressed areas and rarely soft slightly pink areas of anetoderma.Lupus tumidus limited to the breast called lupus mastitis (3) may herald the onset of SLE

Associations
Post traumatic or post surgical lesions are known to occur and have been reported to occur after electromyography(4). Monoclonal gammopathy has been reported with lupus tumidus(5)

Diagnosis
Histopathology is sufficient to make a diagnosis in the absence of other cutaneous systemic features of LE


Investigations

Diagnosis of lupus erythematosus may be established by ANA testing.

Acute phase reactants, such as erythrocyte sedimentation rate (Westergren) and C-reactive protein, may be elevated in active SLE,
The prognosis for childhood onset UP is very good with more than 50% cases clearing spontaneously by the teenage6. Late onset

The high frequency of subclinical nephritis necessitates regular screening with urinalyses, quantitative assessment for proteinuria, and determination of the glomerular filtration rate (GFR).

Treatment
The treatment of tumid lupus is along the lines of chronic cutaneous lupus erythematosus and it includes

Photoprotection
As the disease is aggravated by sun exposure, patients are advised photoprotection and daily use of sunscreen. (6)

Topical medication

Local steroid applications with a moderately potent corticosteroid

Intralesional corticosteroid injections are helpful especially for lesions on the mouth,lips and ears(7-9)

Oral medications

Systemic steroids

For severe disease prednisolone in the dose 0.5mg/kg is quickly effective, minimizes scarring and allows the slower acting antimalarials to act

Antimalarials

Therapy with Hydroxychloroquine 200mg twice daily tapered to once daily is effective. alternatively chloroquine 200mgtwice daily may be administered. Response to treatment is better in tumid lesions with slight scaling as compared to chronic atrophic scarring lesions

Resistant lesions of tumid lupus

• Oral thalidomide has proved remarkable in suppressing the lesions.(10) The initial dosage is 400mg/day and maintenance dose is 50-100 mg/day.polyneuropathy is the most common side effect. The drug is contraindicated in pregnancy for its known teratogenic effect.
  
  
• Clofazimine possesses antimalarial activity and suppresses the lesions of DLE at an optimum dose of 100mg/day
  
  
• Pulsed methylprednisolone 500-1000mg/day may help resistant lesions particularly of the scalp
  
  
• Cyclophosphamide (11)and azathioprine (12)may be used in cases not responding to antimalarials
  
  
  

References -

1. Winkelmann RK Panniculitis in connective tissue disease Arch Dermatology 1983 ;119;336-344
2. Arnold HL Lupus erythematosus profundus Arch Dermatology 1956;73:15-33
3. de Bandt M ,MeyerO,Grossin Met al Lupus mastitis heralding SLE with antiphospholipid syndrome J Rheumatology 1993;20:1217-1220
4. Fahrner L,DuvicM.Lupus panniculitis Arch Dermatology 1986;122:625-626
5. Fuerner EJ lupus erythematosis profundus with monoclonal gammopathy Arch Dermatol 1986;122;625-626
6. HawkJLM,Challoner AVJ ,Chaddok L.efficacy of sunscreen agents :protection factors and transmission spectra Clin.Exp.Dermatol1982;7;21-31
7.Callen JP Chronic cutaneous LE Arch Dermatol1982;118:412-416
8. James APR.Intradermal triamcinolone acetonide injection in localized lesions;Antibiot med clin ther1960;7;495
9. Rowell NR Treatment of chronic discoid lupus erythematosua with intradermal triamcinolone Br J Dermatology 1962;74;354-357
10. Knop J,Bonomann G,Happle Ret al ;Thalidomide in the treatment f 60 cases of DLE Br.J.Dermatol1983;108;461-466
11. SchultzEJ,Menter MA.treatment of discoid and subacute LE with cyclophosphamide Br J Dermatol1971;85;60-65
12. Ashinoff R, WerthVP,Franks AG Jr resistant discoid lupus erythematosus of palms and soles :successful treatment with azathioprine.J Am Acad Dermatol1988;19;961-5