Disseminated Discoid Lupus Erythematosus - “the Skin-Sun Mystique”: A case report
1Professor & Head, 2 Assistant Professor, 3Post Graduate, Department of Dermatology, Venereology and Leprosy, Kamineni Institute of Medical Sciences, Narketpally.
Discoid Lupus Erythematosus (DLE) is a benign skin condition, most frequently involving the face and other photo exposed areas of the body, characterized by well-defined, red scaly patches with atrophy, scarring and pigmentary changes. The histology is characteristic. Haematological and serological changes in these patients suggest auto immune aetiology.1
A 33 year old female farmer presented with multiple, red colored, raised skin lesions associated with itching and burning on exposed parts of the body since 8 years. Patient was apparently asymptomatic 8 years back. She gave the history of coin shaped lesions which started initially as red colored lesions on both cheeks which gradually increased in number and reached present size of 6 months. Similar red colored, scaly, raised lesions appeared on face, trunk, upper and lower extremities after 1 month. These small lesions coalesced to form present sized lesions of over 6 months.
There was H/o photosensitivity, no H/o oral ulcers, no H/o fever/fatigue/weight loss, no H/o joint pains/ stiffness/swelling no h/o discoloration of fingers upon exposure to cold, no H/o shortness of breath/ chest pain/headache/confusion or memory loss and no H/o drug intake prior to onset of these symptoms.
No H/o dysphagia/tightening of skin/dry eyes/dry mouth/proximal muscle weakness, no H/o high colored urine/hematuria/ abdominal pain.
Fig 3: Clinical photograph showing multiple de-pigmented plaques on extensor aspect of both hands.
Fig 4: Clinical photograph showing small plaques coalesced to form large plaques on extensor aspect of right forearm.
Cutaneous examination showed multiple, well defined, erythematous plaques ranging in size from 2-15cm with adherent scales and hyperpigmented borders, distributed bilaterally symmetrical on face including bridge of nose, vermilion border of both upper and lower lips (Fig 1).
Plaques are distributed on right and left side of scalp, right and left side of helix of both ears, V-shaped area of neck, upper chest and dorsum of hands including fingers, upper back and anterior part of right leg (Fig 2, 3). Small plaques are coalesced to form large plaques on extensor aspect of right forearms (Fig 4). Multiple de-pigmented plaques with atrophy and scarring are seen on upper back. Carpet tack sign was positive.
Mucous membranes: Oral, Genital and Conjunctiva: normal; Hair and Nails: normal; Palms and Soles: normal. Systemic examination was unremarkable. Based on history and cutaneous examination, a provisional diagnosis of DLE was considered and subjected to necessary routine investigations done to rule out other differential diagnosis like Sub Acute Cutaneous Lupus Erythematosus, Psoriasis, Lupus Vulgaris. Routine hemogram and other biochemical investigations were within normal limits.
Erythrocyte Sedimentation Rate – 80mm in 1sthr, Electrocardiogram – normal sinus rhythm.
Chest X-ray: no abnormalities are detected. Test for Lupus Erythematosus Cell – negative. Anti Nuclear Antibodies– negative.Skin biopsy: A 4x4mm punch biopsy was taken from plaque over right hand.
Patient was started on broad spectrum sunscreens with SPF-30 applied 30 minutes before sun exposure 3-4 times daily. Topical therapy with Mometasone Furoate cream 0.1% twice daily on face, Halobetasol Propionate cream 0.05% twice daily on trunk, hands, and legs. Tab Hydroxychloroquine-200mg, orally, twice daily (after fundoscopic examination and clearance given by ophthalmology dept). Patient responded very well to the medication and is on regular follow up till date.
DLE is by far the most common manifestation of LE (Lupus Erythematosus). It commonly presents with erythematous, scaly papules and plaques occurring on sun-exposed areas, although 50% of DLE lesions are found on areas of hair-bearing scalp that are presumably protected from the sun. In the localized variety of DLE, the lesions tend to be confined to the head and neck and in the generalized variety, they occur both above and below the neck. Patients with generalized DLE have significantly greater chances of having laboratory abnormalities and of progressing to Systemic Lupus Erythematosus (SLE). Most people with DLE do not have any systemic or serologic abnormality although antinuclear antibodies may be present.2
DLE occurs at all ages and among all ethnic groups; it occurs more frequently in women than in men, but the predilection among women is not as marked as in SLE. DLE starts as an erythematous papule or plaque, usually on the head or neck, with an adherent scale. The lesion tends to spread centrifugally and as it progresses, there is follicular plugging and pigmentary changes, generally hyperpigmentation at the periphery, and hypopigmentation with atrophy, scarring, and telengiectasia at the center of the lesion.2
Involvement of the scalp commonly produces a scarring alopecia, but there has been an increase in incidence of alopecia areata among patients with LE. Scarring alopecia was present in 34% of 89 patients with DLE and was associated with a prolonged disease course. More than half of those patients had scalp disease at the onset. There are no reliable predictors of scalp involvement. Histologically, there is a perifollicular lymphocytic infiltration maximal around mid-follicle.3
In general, patients with DLE are reported to develop SLE in approximately 5-10% of cases at some point over the course of the disease.
However, the studies in Caucasian population with DLE showed that the risk of developing SLE will even be higher (up to 20%) if the patients had generalized DLE lesions, presence of high ANA titres, signs of nephropathy and rheumatologic manifestations.4
According to previous reports, presence of ANA in DLE varied from 2 - 50%. This variety was influenced by patient selection and test sensitivity. Patients with DLE who had co-existent visceral involvement have a high prevalence of ANA. The speckled pattern was uncommon in patients with SLE, but it was commonly detected in patients who had both SLE and discoid lesions (36%) and in patients with DLE (100%). Speckled pattern was the most common pattern detected.5
DLE is a chronic disease with multiple presentations. Sun protection measures must be advised to patients to lessen the risk of disease. The risk of SLE development is higher in our patient with disseminated DLE (22%) than in DLE confined to head and neck (localized form)(1.2%) and there is a high chance of residual pigmentation and scarring, therefore, its early recognition and management is essential to prevent disfigurement.
- Burns T, Breathnach S, Cox N, Griffiths C. Rook’s Textbook of Dermatology. 8th ed. Newyork, NY: John Wiley & Sons; 2013; 51.4:P.2478
- Panjwani S. Early Diagnosis and Treatment of Discoid Lupus Erythematosus. J AmBoard Fam Med. 2009;22:206-213.
- Insawang et al. Discoid lupus erythematosus: Description of 130 cases and review of their natural history and clinical course. Clin Immunol Immunopathol Res. 2010;2:1-8. 4.
- Tebbe B et al. Clinical course and prognosis of cutaneous lupus erythematosus. Clin. Dermatol. 2004;22:121-124.
- Callen JP. Cutaneous lupus erythematosus: a personal approach to management. Australas J Dermatol. 2006;47:13-27.