Coexistence of lip-tip vitiligo and disseminated discoid lupus erythematosus with hypothyroidism: Need for careful therapeutic approach

Abstract

There have been rare published cases of discoid lupus erythematosus (DLE) with other autoimmune cutaneous and systemic disorders. We describe a 36 years old female patient with DLE lesions on the face and hands with coexistence of lip-tip vitiligo and hypothyroidism. We discuss how the treatment has to be modified and innovative in the presence of these three coexisting autoimmune disorders and how the dermatologist should do this to get a successful outcome.

INTRODUCTION

Coexistence of systemic lupus erythematosus and vitiligo has been infrequently reported.[1] However, cases of vitiligo coexisting with discoid lupus erythematosus (DLE) have been much rarer.[2] This is an important consideration as the presence of one disorder may affect the therapy of the second and require detail clinical as well as laboratory examination to rule out other autoimmune disorders. In the present case, association of DLE with vitiligo and thyroid disease altered the therapeutic approach in the affected patient.

CASE REPORT

A 36-year-old female presented with complaints of a reddish, raised lesion on left cheek since 8 years. She also gave history of appearance of similar lesions on nose, malar area, and near the ears since 2 years. There were also depigmented asymptomatic lesions on tips of fingers, dorsa of bilateral hands, perioral area and lips since 2 months and appearance of similar lesions on tips of toes since 1 month.

On examination, a depigmented well defined plaque was present over left side of the malar area extending up to nasal bridge, external auditory meatus and also the mandibular area. The central area of the plaque showed erythema and scaling and the plaque was surrounded by a hyperpigmented border. It measured around 7 × 10 cm in size in its largest diameters. Atrophy was present over the central area of the plaque while induration was present over the peripheral area [Figure 1]. Similar plaques were present over right eyelid, left ear, bilateral extensor surface of forearm, which were variable in size ranging from 1 × 2 cm to 3 × 6 cm in size in their largest diameters. Depigmented macules were present over bilateral proximal nail folds extending up to half of the lateral nail fold of the fingers and similar types of depigmented macules were observed over bilateral toe nails extending from distal nail fold to lateral nail fold, upper perioral area and lips [Figure 2]. Although all the lesions were depigmented, a clinical diagnosis of disseminated lupus erythematosus with lip-tip vitiligo was entertained.

Figure 1.

Depigmented well defined plaque with scaling on left side of the malar area extending up to external auditory meatus, nasal bridge and the mandibular area

Figure 2.

Depigmented maculeson upper and lower lip and bilateral proximal nail folds extending up to half of the lateral nail fold of the fingers

Histopathological examination of the lesion on the face showed follicular plugging, basal cell degeneration, perivascular and periappendageal lymphocytic infiltrate, hence was consistent with the diagnosis of discoid lupus erythematosus [Figure 3]. Histopathological examination from depigmented macule over finger showed decreased number of melanocytes in basal layer and was suggestive of vitiligo [Figure 4].

Figure 3.

Epidermis shows presence of follicular plugging and basal cell degeneration while perivascular and periappendageal lymphocytic infiltrate is seen in the in the dermis (H and E, ×45)

Figure 4.

Decreased number of melanocytes in basal layer of epidermis (H and E, ×45)

Lupus band test from lesional exposed site was positive and non-lesional, non-exposed skin was negative. Laboratory test revealed positive ANA, and anti SSA (Ro) Rheumatic factor, dS-DNA and other connective tissue disease markers were negative. We sent stool for occult blood, thyroid function test and serum cortisol examination to rule out possibility of other associated autoimmune disorders. Stool for occult blood was negative; serum cortisol level was normal but and thyroid function tests showed increased of TSH and decreased of T4.

The patient was advised photoprotection and started on oral hydroxychloroquine 200mg once a day for 1 week then 200 mg twice a day dosage for DLE and topical steroids for the vitiligo lesions keeping in mind that although the lip tip vitiligo lesions would have responded well to photochemotherapy, this therapy would have aggravated the lupus erythematosus lesions. Oral thyroxine hormone replacement therapy was started for low thyroid hormone. The patient was followed up after 6 weeks and 12 weeks when there was a partial resolution of facial lesions. Further patient was advised to apply 0.1% topical tacrolimus on facial lesions but follow up was not possible as the patient probably left the city.

DISCUSSION

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.[2] Autoimmune thyroid disease association with systemic lupus erythematosus is commonly reported (prevalence = 3.9-24%) and hypothyroidism is much more common as compare to hyperthyroidism (5.7%/1.7%).[3] Data regarding autoimmune thyroid disease association with DLE are not available in the literature. In two case reported by Forestier et al., DLE occurred within the vitiliginous skin on both exposed and non-exposed surfaces.[4] A similar occurrence was reported by Jeffrey Callen, Temine and Tramier, Chowdhury and Banerjee, Forestier et al. and Johnson et al.[1,4–7] DLE coexisting with vitiligo has also been reported with alopecia universalis, dermatophyte nail infection, malignant melanoma and urticaria.[8–10]

Table 1 shows a few case reports in the Indian setup and the west where patients had coexisting vitiligo and DLE and their treatment. The majority of patients with concurrent DLE and vitiligo resided in regions with potential chronic sun exposure, such as India and Southern Europe.[1,4,9]

Table 1.

Case reports of discoid lupus erythematosus with vitiligo and other diseases

Ying Jin et al. identified several chromosomal regions that appear to contribute to this epidemiologic association, including one on chromosome 17p13.[11,12] Treatment of DLE includes photoprotection, topical or intralesional glucocorticoids, or antimalarial medication. Alternative therapies include retinoids or immunosuppressive agents, such as thalidomide, methotrexate, mycophenolate mofetil, or azathioprine.[6,10] Treatment of vitiligo in the presence of DLE is mainly local and systemic corticosteroid.[8] 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 can not be given in the presence of DLE so one has to cautiously treat the two. We conclude that hypothyroidism may worsen the DLE if not treated and there are higher chances for showing resistance to treatment.[1]

It is important to treat concomitant medical or endocrinal abnormalities as well as other cutaneous conditions. Instead of topical steroids for vitiliginous lesions we can also give topical tacrolimus as an alternative. We discuss how the treatment has to be modified and innovative in the presence of these three coexisting autoimmune disorders and how the dermatologist should do this to get a successful outcome.