CLINICAL HISTORY:

Signs and symptoms:  Initially pt noted skin irritation on her face. She saw here primary physician for this. The eruption gets itchy when pt is overheated. When asked pt states that she has had arthralgias. She complains of fatigue, but is post-partum with twins. She is not breast feeding.

Previous Treatment:  Erythromycin 333mg one PO TID for ten days. Pt completed this treatment four days before presentation to our office. Also applying Triamcinolone 0.1% cream BID-TID

Other information:  Pt is 15 weeks post-partum. She delivered twin girls via c-section 2/7/03. Her pregnancy was complicated by hypertension in the third trimester. After delivery she was started on Lisinopril 20 mg QD (started April 2003)

PHYSICAL EXAM:

Erythematous, irregularly defined plaques on malar cheeks bilaterally, as well as on her left upper lateral arm, and right knee. Petechiae of the hard palate

LABORATORY TESTS:

ANA pattern: speckled; titer: 1:160
Antihistone Antibodies: negative
FANA: positive; 1:1280; homogeneous pattern
ENA w/o R?Nase: positive (1:256)
ENA w R’Nase: positive (1:128)
Ds DNA: positive (1:160)
Total protein urine 24hr: 2630.0 mg/dl
Total protein urine spot: 263 mg/dl
Cutaneous Direct Immunofluorescent:
IgG: diffuse granular deposits basement membrane
IgM: patchy faint granular deposits basement membrane and few globular deposits present
IgA: few cytoids (globular deposits) present
C3: diffuse granular deposits basesment membrane
CBC and ESR, and Complete metabolic profile: all within normal limits

DERMATOHISTOPATHOLOGY:

nonspecific chronic dermatitis- superficial and deep with a perivascular pattern

DIFFERENTIAL DIAGNOSIS:

1.   SLE
2.   Drug induced SLE
3.   Discoid lupus
4.   Granuloma Annulare
5.   Drug eruption

SCROLL DOWN FOR ANSWER AND DISCUSSION.

CORRECT DIAGNOSIS:

systemic lupus erythematosus

DISCUSSION:

Our main differential was if this was a case of drug-induced lupus secondary to Lisinopril versus systemic lupus erythematosus. According to Litt, 9th edition, rash is seen 1.5% of patients and lupus erythematosus is listed as a reaction as well. However, the history of hypertension in the end of the pts third trimester of pregnancy without elevated liver functions, suggested that this disease process preceded the start of lisinopril. While the labs were pending we did hold the lisinopril and change to a B-blocker as an antihypertensive. In spite of this, at the next visit, two weeks later, she had more extensive skin involvement. The anti-histone antibodies were negative and this supported our suspicision that this was not drug-induced, but a true acute systemic lupus erythematosus.

Systemic lupus erythematosus affects one out of every 1,000 white persons and one out of every 250 black women from 18 to 65 years of age. The etiology still remains unclear however there is an abnormal production of antibodies by B cells. To make the diagnosis the patient must have four of eleven criteria developed by the American College of Rheumatology. Skin involvement occurs in 80% of cases. Earliest changes may be transitory or migratory arthralgias and fever, weight loss, pleuritis, adenopathy, or abdominal pain. Other abnormalities include thrombosis of various sized vessels, renal involvement (either nephritic or nephritic), myocarditis, pericarditis, Raynaud’s phenomenon, and CNS vascultis, and ITP.

Our patient had 4 of the eleven criteria to make the diagnosis of SLE. She had the typical early arthralgias. She was also of the typical sex and age. She presented with preceding renal involvement with the history of hypertension in late pregnancy without the other typical “HELP?or preeclapsia findings. Her hypertension is likely secondary to this renal involvement as is her proteinuria. She was referred to a nephrologist and decided to follow up with her primary care physician. Unfortunately, she was lost to follow up. If she remained under our care we would have considered treating options with systemic corticosteroids, antimalarials or other immunosuppressive agents. The literature supports using systemic steroids in cases with renal and central nervous system involvement, to effectively prolong survival.

TREATMENT:

Actual treatment for this patient:
Locoid cream BID to facial lesions
Cloderm cream BID to areas on arms and legs
Aggressive UVA/UVB sunblock



Other Treatment options:
Sun block, topical steroids and antimalarials are important in the treatment of cutaneous lupus. Antimalarials used are hydroxychloroquine and quinacrine or chloroquine(has more of a risk of retinopathy)

Oral steroids ( once renal disease was better defined with at least a 24hr urine and possibly a kidney biopsy).


Team approach- referral to a nephrologist and a rheumatologist or internist/ primary care physician
Oral NSAIDs- for arthralgias (avoid with pts with nephitis) and ( will increase the risk of gastric ulcers when used in combination with corticosteroids)
Steroid sparing immunosuppressive medications like methotrexate or axathioprine
Also thalidomide is one of the most effective drugs for treatment of discoid lupus.

REFERENCES:

1. Litt JZ, MD. Drug Eruption Reference Manual, 9th edition. New York, New York: The Parthenon Publishing Group Inc, 2003, pg.261.

2. Odom RB, James WD, Berger TG. Andrews?Diseases of the Skin. Philadelphia: Saunders, 2000, pgs 117-186.

3.Petri M, MD. Treatment of systemic Lupus Erythematosus: An Update. American Family Physician 1998.

Additional Comment:

© Copyright 2003-2006 by AOCD Grand Rounds

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