Discoid lupus erythematosus in acro-orificial vitiligo

INTRODUCTION

Discoid lupus erythematosus (DLE) is an autoimmune disease characterized by atrophic and discoid plaques over sun-exposed areas of skin and is the most common form of chronic cutaneous lupus erythematosus. Vitiligo is an auto immune disease resulting from progressive loss of melanocytes presenting as depigmented or achromic milky white sharply demarcated macules often associated with leukotrichia.¹ Vitiligo being an autoimmune disease, is known to be associated with other autoimmune conditions such as thyroid disease, diabetes mellitus, and alopecia areata.² However coexistence of DLE and vitiligo has been reported uncommonly. We report a case of a 60-year-old lady who developed DLE lesions over pre-existing vitiligo lesions.

CASE REPORT

A 60-year-old lady presented with white lesions on legs, which started two years ago and then progressed gradually to involve both the palms, soles, nipple, areola, lips and peri-oral areas over a period of one year. Six month ago, patient developed raised scaly lesions over the vertex of scalp and on the whitish patch around the mouth. Patient subsequently developed scaling on the lips and erosions in the oral cavity. Lesions on the scalp and perioral region were associated with burning sensation on exposure to sunlight. There was no history of multiple joint pain, fever, thyroid dysfunction or diabetes mellitus. Patient is hypertensive since one year and on regular treatment. There was no history of vitiligo, collagen vascular disease, diabetes mellitus and thyroid disorder in the family.

On examination, multiple depigmented patches were present over the lips, peri oral areas (Figure 1), right nipple, left mammary region involving nipple areola complex, right loin, antero-lateral aspect of both the legs and plantar aspect of both feet. There were few depigmented macules on the dorsum of both feet. Depigmented macules were also present over the extensor aspect of both forearm and proximal nail folds of great toes bilaterally. A single erythematous plaque with adherent scales was present over the vertex of scalp. Erythematous plaques with adherent scales were also present over, both upper and lower lips and the area above the upper lip superimposed on the depigmented patch. A single depigmented discoid plaque measuring 0.5×0.5 cm with adherent scales and surrounding rim of hyperpigmentation was present over the left side of dorsum of nose. Scaly plaques with crusts and fissure were seen on the lips. Oral cavity showed a single shallow ulcer on the hard palate. Great toe tails were dystrophic. Other nails were normal.

Punch biopsy was done from the lesion over the scalp which showed thinned out epidermis with follicular plugging, hyperkeratosis, mild spongiosis, basal cell degeneration and dermo-epidermal junction showed band of chronic inflammatory cells comprising of lymphocytes and plasma cells extending around the follicles suggestive of discoid lupus erythematosus (Figure 4).

Dermoscopy (polarised mode, 10X magnification) revealed branching vessels, focal keratin plugs, peifollicular whitish halo and structure-less white and brown areas (Figure 3).

ANA and anti-dsDNA were negative. Complete hemogram, liver function test, renal function test, thyroid profile and random blood sugar levels were within normal limits. Haemoglobin 11.9 g%, Total count 6300 cells/mm³, Differential count N₄₀ L₅₀ E_1, ESR 15 mm/hour, RBC count 4.16 million/mm³, Platelet count 2.69 lakhs/mm^3, PCV 35.3%, MCV 85.9 fL, MCH 28.6 pg, MCHC 33.7%, serum creatinine 0.9 mg/dL, serum urea 17 mg/dL, Total bilirubin 0.5 mg/dL, Direct bilirubin 0.1 mg/dL, Total protein 6.8 g/dL, Albumin 3.7 g/dL, Globulin 3.1 g/dL, SGOT 57 U/L, SGPT 44 U/L, Alkaline phosphatase 218 U/L, TSH 2 iIU/ml. Based on clinical and histopathological features a diagnosis of Chronic Cutaneous Lupus erythematosus over pre-existing vitiligo (acro-orificial/lip-tip vitiligo) was made.

Patient was prescribed topical mid-potent corticosteroid for lesions around lips and nose, to be applied twice daily. Topical super-potent corticosteroids with salicylic acid twice daily application was prescribed for the lesion on scalp. She was also given broad spectrum sunscreen lotion to be applied in all the photo-exposed areas. Ten days later, lesions on the nose and scalp flattened with decrease in scaling and erythema (Figure 2). Lesion around the mouth also showed resolution with decrease in erythema and scaling and the underlying vitiligo lesion around the mouth stood exposed.

DISCUSSION

Chronic cutaneous lupus erythematosus (CCLE) is an inflammatory and photosensitive dermatosis that likely stems from a poorly understood immune dysregulation with an autoimmune reaction in genetically predisposed individuals. CCLE occurs more frequently in women and is often characterized by erythematous, scaling plaques but may exhibit clinical variability, such as hypertrophic or lichenoid features. Around 5% of patients with DLE may progress to SLE. Vitiligo is also an autoimmune disorder with underlying genetic susceptibility and around 30% of patients with generalised vitiligo have associated autoimmune diseases.² However, the pathogenesis of concurrence of vitiligo and CCLE has not been fully understood.³

In our patient, DLE lesions developed over the vitiligo lesions two years after the onset of vitiligo. Coexistence of two or more autoimmune diseases such as lupus erythematosus and vitiligo has been uncommonly reported in literature. The co-existence of DLE and vitiligo must be differentiated from post-inflammatory depigmentation left behind after resolution of DLE lesions. Forestier et al described two patients, one with discoid lupus who developed vitiligo-like lesions and one with vitiligo whose clinical course was complicated by the appearance of lesions of discoid lupus.⁴ Both these patients presented with elevated antinuclear antibodies, while all other autoantibodies were negative. This was in contrast to our case wherein ANA and anti ds-DNA were negative. Other authors have subsequently reported further, isolated cases of patients with lupus who developed vitiligo over the course of their disease.⁵ In two cases reported by Forestier et al., DLE occurred within the vitiliginous skin on both exposed and non-exposed surfaces.³ A similar occurrence was reported by Jeffrey and Callen, Temine and Tramier, Chowdhury and Banerjee, Forestier et al and Johnson et al.^2,4,6-8 Table 1 shows a few case reports of patients who had coexisting vitiligo and DLE and their treatment. Most of these patients resided in regions of potential sun exposure, such as India and Southern Europe.^3-6,9-11

It has been observed that the autoimmune disorders are significantly elevated in vitiligo probands: Vitiligo itself, autoimmune thyroid disease, pernicious anemia, Addison's disease, systemic lupus erythematosus, and probably inflammatory bowel disease. Ying Jin et al identified several chromosomal regions that appear to contribute to this epidemiologic association, including one on chromosome 17p13.⁵

Based on a review of the literature, we believe that the coexistence of these two autoimmune diseases is not as common as might be expected for these autoimmune processes. Recent data show an autoimmune genetic basis that might further our understanding of the coexistence of the two conditions.

Treatment of vitiligo in the presence of DLE is mainly local and systemic corticosteroid. However, it is known that lip-tip vitiligo would not respond much to topical and systemic corticosteroids. The presence of one disorder may affect the therapy of the other, as photochemotherapy which would be a good alternative for lip-tip vitiligo cannot be given in the presence of DLE so one has to cautiously treat the two.

Ultraviolet light exposure and chronic inflammation play a role in development of squamous-cell carcinoma (SCC) over a pre-existing lesion of CCLE. While SCC formation in long-standing vitiligo is rare, it has been associated with PUVA photochemotherapy and with intense sun exposure.² Squamous-cell carcinomas, sometimes multiple, have been described as arising in lesions of CCLE. Hence there is a need for regular follow up of patients with vitiligo with co-existent DLE, as the exposure to ultra violet radiation may potentiate the development of SCC over the DLE lesions.

Funding: No funding sources

Conflict of interest: None declared

Ethical approval: Not required

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