Verrucous Disseminated Discoid Lupus Erythematosus with Plantar and Oral Lesions in the Absence of Systemic Lupus Erthyematosus.

Sir,

Discoid lupus erythematosus (DLE) is a chronic cutaneous lesion of lupus erthymatosus (SLE). Here, we are reporting a rare presentation of disseminated DLE (DDLE).

A 65-year-old female presented with a history of pruritic skin lesions of 1.5 years duration and oral lesions of 8 months duration. On examination, there were multiple, discrete, and confluent hyperpigmented verrucous papules and plaques with generalized distribution sparing the scalp and abdomen [Figure 1a–c]. The plantar region showed confluent hyperkeratotic verrucous plaques. Crusted plaques were seen over the lower lips crossing the vermillion border with multiple erosions over the tongue, buccal mucosa, and palate [Figure 1d]. Systemic examination was unremarkable except for inguinal lymphadenopathy. We considered differentials of paraneoplastic pemphigus, verrucous LE, and hypertrophic lichen planus.

Figure 1

(a) Verrucous hyperkeratotic papules on upper limbs, (b) verrucous hyperkeratotic papules and plaques on lower limbs, (c) verrucous plaques on the plantar region, (d) crusted plaques on the lower lips with erosion in the buccal mucosa

The patient's hemogram, urine tests, liver, and renal function tests were normal. Viral markers were negative. ANA profile was negative. Chest X-ray, ultrasound of the abdomen, and malignancy workup did not reveal any pathology. Fine-needle aspiration cytology (FNAC) from the inguinal lymph node showed dermatopathic changes. System wise investigations did not show any evidence of SLE. Skin biopsy from multiple areas and oral lesions showed hyperkeratosis, keratotic plugging, basal cell degeneration, interphase dermatitis, and perivascular mononuclear infiltrate, all diagnostic of DLE [Figure 2a and b]. Direct immunofluorescence (DIF) of the skin biopsy specimen showed linear to granular staining of IgG, IgM, and C3 along the basement membrane zone diagnostic of DLE [Figure 3]. We made a final diagnosis of verrucous DDLE in the absence of SLE and the patient was started on prednisolone 30mg.

Figure 2

(a) Skin biopsy showing hyperkeratosis, keratotic plugging, basal cell degeneration, interphase dermatitis, and perivascular infiltrate, H and E, ×100. (b) Oral biopsy showing ulceration, basal cell degeneration, and interphase dermatitis, H and E, ×100

Figure 3

Direct immunofluorescence showing linear to granular staining of IgGC3 along the basement membrane zone suggestive of DLE, immunofluorescent stain, ×400

Our patient presented with generalized hyperkeratotic, verrucous papules and plaques with histopathology, and DIF diagnostic of DLE. The plantar and oral lesions also showed similar pathology. Systemic investigations did not show any evidence of SLE. In lichen planus, histopathology shows epidermal hyperplasia with focal hypergranulosis in addition to interphase dermatitis, and DIF shows shaggy deposits of fibrinogen in the basement membrane zone, which was not seen in our case. Therefore, we made a diagnosis of verrucous DDLE without SLE. Verrucous DDLE is a rare presentation of DLE.[12] The presence of plantar verrucous DLE lesions and oral DLE lesions in the absence of SLE is another unique feature of this case. DDLE has a higher chance of ending as SLE, and hence, although our patient did not qualify for SLE, frequent and long-term follow-up is mandatory. Prurigo nodularis such as like lesions, bullous, annular, telangiectoid, linear along Blaskho's lines, and atrophie blanche-like lesions are other rare presentations of DLE reported in literature.[3] Potent topical steroids, intralesional steroids, hydroxychloroquine, and systemic immunosuppressive therapy are the treatment modalities for DLE. Our patient showed prompt response to prednisolone. There have been reports of verrucous DDLE responding to oral isotretinion and thalidomide.[45] We could not come across any report in the literature of verrucous DDLE with oral and plantar lesions in the absence of SLE to the best of our knowledge.

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