Tumid lupus erythematosus: an intriguing dermatopathological connotation treated successfully with topical tacrolimus and hydroxyxhloroquine combination.

Tumid lupus erythematosus (LE) is a rare variant of lupus erythematosus, which often follows a favorable course. A case of a young woman is illustrated, who presented with an asymptomatic erythematous, solitary plaque over her face. Histopathological and direct immunofluorescence examination established a diagnosis of tumid lupus erythematosus. She responded slowly and near-completely to hydroxychloroquine sulfate; however, a flare up occurred a month later. Addition of topical tacrolimus 0.1% resulted in complete regression without leaving any residual changes. No recurrence was seen subsequently.

What was known?

Tumid lupus erythematosus is a distinct clinic-pathologic variant of cutaneous lupus erythematosus.

Topical corticosteroids and oral hydroxychloroquine have been the mainstay of treatment.

Introduction

Tumid LE, ever since its initial description, has been an intriguing entity with diagnostic pitfalls. As truly recognized by Alexiades-Armenakas et al.,[1] there have been lacunae in the definition of this entity. However, it has also been suggested that no inflexible histological criteria should be employed for the diagnosis.[2] Tumid LE has recently been included as intermittent type of chronic cutaneous LE. It is imperative to take cognizance of its clinical and histopathological features to differentiate it from various other dermatoses. The present article illustrates a case of tumid LE, attempts to discuss its differential diagnosis, and demonstrates the efficacy of tacrolimus for this variant, which has not been described in the literature so far.

Case Report

A 27-year-old married woman, mother of two, accessed our dermatology outpatient department with an asymptomatic pink-red plaque over the right cheek for the last 5 months. She denied photosensitivity, oral ulcerations, fever, and joint pains. Rest of the systemic examination was insignificant. Physical examination revealed single erythematous, indurated, 3 cm-sized plaque over the right cheek [Figure 1]. Regional lymph nodes were not enlarged. Rest of the muco-cutaneous and systemic examination was normal. A clinical differential diagnosis of tumid lupus erythematosus (tumid LE), Jessner's lymphocytic infiltrate, and pseudolymphoma was made. Microscopic examination of the sections from the lesion revealed slightly thinned epidermis, subepidermal edema, superficial and deep, largely perivascular and some periappendageal lymphocytic infiltrate [Figure 2]. Dermo-epidermal junction was normal. Increased amounts of mucin were identified on staining with Alcian blue. The biopsy specimen from the lesion was subjected to direct immunofluorescence, which showed moderate IgG, and weak IgA, IgM, C3, and fibrin deposits along the basement membrane zone in a linear fashion [Figure 3]. Facility for CD4 and CD8 staining was not available in our institution. Further instrumental investigations including anti-nuclear antibody (ANA) titers (by indirect immunofluorescence on Hep-2 cell line), complete blood counts, liver and renal functions, and blood sugar levels were normal. LE cell test was not done. Urine routine microscopy and 24-hour urine protein were normal too. In addition, imaging studies including X-ray chest and an ultrasound of abdomen were unremarkable. Accordingly, a diagnosis of tumid lupus erythematosus was entertained. After obtaining an ophthalmology clearance, the patient was administered hydroxychloroquine sulphate in a dose of 200 mg daily and was asked to take adequate photoprotection including broad-spectrum sunscreen. The lesion had regressed after a period of 4 months. However, a month later, she had a flare up in the lesion while on hydroxychloroquine and despite adequate photoprotection. Considering the potential side effects of potent topical corticosteroids on face and the very fact that topical tacrolimus has been shown to be effective in non-hyperkeratotic LE lesions, it was decided to add topical tacrolimus 0.1% ointment to HCQS. The lesion resolved completely over a period of 3 weeks [Figure 4]. Subsequently, HCQS was stopped, and she is now on oral antioxidants and photoprotection. The patient is on follow-up for 4 months post-treatment and has not shown recurrence.

Figure 1

Tumid LE illustrating single erythematous, indurated, 3 cm-sized plaque over the right cheek. The surface depicts a punch biopsy mark

Figure 2

Tumid LE depicting slightly thinned epidermis, subepidermal edema, superficial and deep, largely perivascular and some periappendageal lymphocytic infiltrate. Dermo-epidermal junction was normal

Figure 3

Tumid LE demonstrating positive direct immunofluorescence; moderate IgG, weak IgA, IgM C3, and fibrin deposits along the basement membrane zone in a linear fashion

Figure 4

Post-treatment near complete regression without atrophy and/or scarring, except post-biopsy scar

Discussion

Tumid LE, a chronic form of cutaneous LE, was first described in the year 1909.[1] Ever since, there have been occasional reports of the condition in the world literature. The course and prognosis of LET are generally more favorable than in other subtypes of CLE.[3] Recently, modification and extension of the Gilliam classification was suggested, with the intermittent CLE (ICLE) subtype, including tumid LE as a separate entity of the disease (Duesseldorf Classification 2004.[4]

The lack of large scale studies due to rarity and its different clinical and histopathological behavior from other forms of chronic cutaneous LE lesions and often occurring independently of the other forms of LE either, cutaneous or systemic, tumid LE can pose a diagnostic challenge. Clinically, it lacks the surface changes namely; scaling, atrophy, scarring, follicular plugging, and telengiectasias unlike discoid LE.[1] 70% of the patients are photosensitive, and ANA may be detected in only 10% of the patients.[5] Histopathologically, tumid LE lacks the epidermal changes namely; hyperkeratosis and follicular plugging characteristic of discoid LE; and dermo-epidermal interface changes, which are hallmark of most of the cutaneous forms of LE. Jessner's lymphocytic infiltrate of the skin (JLIS), polymorphous light eruption (PMLE), pseudolymphoma, lymphoma cutis, systemic LE with mucinosis, and granuloma faciale are its differential diagnosis with former being the most difficult to differentiate. However, in the present case, slight epidermal atrophy and more importantly DIF positivity led to the diagnosis of tumid LE.

The differential diagnoses have been succinctly outlined in [Table 1] for a glance.

Table 1

Tumid LE: Differential diagnoses

Anti-malarials and local corticosteroids have been the cornerstones of treatment. Recently, photodynamic therapy has been shown to be effective and fast treatment option for acute flares of LT; however, it does not prevent recurrences.[8] Topical tacrolimus has recently shown efficacy in non-hyperkeratotic variants of cutaneous LE.[9] However, to our knowledge, there is no evidence of the use of tacrolimus in tumid LE in the literature.[10]

What is new?

The present article demonstrates the use of tacrolimus in tumid LE.

The differential diagnoses of tumid LE have been succinctly brought up.