A Rare Case of Lichen Planus/Discoid Lupus Erythematosus Overlap Syndrome and Its Successful Treatment

ABSTRACT

An uncommon condition referred to as the overlap syndrome of lichen planus (LP) and lupus erythematosus (LE) exhibits immunopathological, histological, and clinical characteristics of both LP and LE. Though LP and LE are common individually, their overlap is rare. This condition typically presents with large, scaly, centrally atrophic lesions on the distal limbs, face, and trunk, often bluish‐red to hypopigmented in color. We describe a unique case of overlap syndrome between LP and discoid LE in a 30‐year‐old female patient presenting with lesions on her scalp, limbs, the dorsum of her hands, and oral mucosa. The lesions were neither painful nor warm to the touch. The diagnosis of overlap syndrome involving discoid LE and LP was confirmed through laboratory investigations and dermatological evaluation. Initial treatment with clobetasol propionate showed no significant improvement. Subsequently, the patient was prescribed 200 mg of azathioprine daily, along with the corticosteroid Deflazacort. Notable improvement in the lesions was observed after 4–5 weeks of follow‐up.

Summary.

• The 30‐year‐old patient in this case study had lichen planus/lupus erythematosus overlap syndrome, highlighting the complexity of autoimmune illnesses and the need for individualized treatment.

• Her unusual presentation and resistance to conventional treatments highlight the significance of thorough assessments and customized strategies for handling such complex clinical situations.

Abbreviations

ANA test

antinuclear antibody test

Anti‐dsDNA antibodies

anti‐double‐stranded deoxyribonucleic acid

DIF

direct immunofluorescence

LE

lupus erythematosus

LP

lichen planus

1. Introduction

Lichen planus (LP)/lupus erythematosus (LE) overlap syndrome is an uncommon condition that combines the clinical, histological, and immunopathological characteristics of LE and LP [1]. Even though LP and LE are prevalent diseases, an overlap is regarded as an unusual phenomenon. Lupus erythematosus–lichen planus (LE–LP) overlap syndrome was initially identified in 1977 by Romero et al. as a histopathological condition exhibiting characteristics of both diseases, though initially viewed as variants of each. It was officially recognized in 1978 by Jamison et al., who reported it in association with cryoglobulinemia and hypocomplementemia [15, 16].

In the literature, there have been about 50 reports of LE/LP overlap syndrome [2]. Although the exact etiology of overlap syndrome is uncertain, evidence suggests an autoimmune mechanism [3]. The distal arms, legs, face, and trunk are frequently affected by the lesions. These lesions are described as large, scaly patches that are centrally atrophic, bluish‐red to hypopigmented in color, and painful [4]. To get an appropriate diagnosis, it is critical to distinguish between LP and LE. While the two conditions often have different clinical symptoms, they may share immunopathological and histopathological findings, such as the presence of colloid bodies and alterations in the basement membrane [5]. There is no established course of treatment for LP/LE overlap syndrome. Glucocorticoid resistance, however, exists in certain cases [4]. Histologically, the diagnosis of LE–LP overlap syndrome requires the presence of histologic and immunologic features of LE and LP in a single biopsy specimen. We present a case in which the same situation was observed but later overcome by treatment with azathioprine.

2. Case Presentation

2.1. Initial Case History/Examination

A 30‐year‐old Pakistani woman presented to the Civil Hospital Hyderabad complaining of non‐painful skin and mucosal lesions. There was no burning sensation or pain in the lesions. Dermatological examination revealed violaceous, thickened, scaly lesions on her scalp, arms, lips, chest, and mucous membrane of the oral cavity. She did not have obesity, multiple joint pain, lethargy, loss of appetite, or loss of weight, and did not suffer from subfever, arthralgia, or myalgia. The patient had no family history of autoimmune diseases.

Physical examination showed buccal mucosa had abnormal physical characteristics, including Wickham's striae, circinate lesions with erythematous borders, and some with central atrophy and post‐inflammatory hyperpigmentation. Additionally, she had received topical clobetasol propionate treatment. As the patient complained about photosensitivity since the lesions appeared, the dermatologist advised her to stay out of the sun. Pregabalin 50 mg was given to the patient since she also had soreness in both feet. A few days later, the patient complained that topical therapy did not affect the lesions; therefore, they persisted.

2.2. Differential Diagnosis and Investigation

Physical examination revealed that the buccal mucosa had abnormal physical characteristics, including Wickham's striae, circinate lesions with erythematous borders, and some with central atrophy and post‐inflammatory hyperpigmentation. She also received topical clobetasol propionate treatment. As the patient complained of photosensitivity since the lesions appeared, the dermatologist advised her to stay out of the sun. Pregabalin 50 mg was administered to the patient because she also experienced soreness in both feet. A few days later, the patient complained that topical therapy did not affect the lesions; therefore, the lesions persisted. Laboratory examination included the hematological and biochemical profiles in Table 1 and the liver function test (Table 2), and the urine test results were normal. However, the antinuclear antibody (ANA) test was positive, with a clinically significant titer of 1:64.

TABLE 1.

Hematological and biochemical profile of the patient.

Test	Result	Reference ranges
Serum sodium	137.2 mmol/L	136–145
Serum potassium	3.6 mmol/L	3.5–5.1
Serum chloride	107 mmol/L	98–106
Serum bicarbonate	22 mEq/L	23–29
RBC	2.76 × 1012/L	3.8–5.8
Hemoglobin	10.6 g/dL	11.0–16.0
Hematocrit	30.5%	35–45
MCV	110.4 fl	76–96
MCH	38.3 pg	27–32
MCHC	34.7 G/dL	30–35
RDW‐CV	16.9%	11.6–14.6
TLC	8.9 × 109/L	4.0–11.0
Neutrophils	65%	50–70
Lymphocytes	28%	25–45
Eosinophils	02%	1–6
Monocytes	05%	10–12
Basophils	00%	0–1
Platelets	210 × 109/L	150–400

Abbreviations: MCH, mean corpuscular hemoglobin; MCHC, mean corpuscular hemoglobin concentration; MCV, mean corpuscular volume; RBC, red blood cell; RDW‐CV, red cell distribution width‐coefficient of variation; RDW‐SD, red cell distribution width‐standard deviation.

TABLE 2.

Liver function test of the patient.

Test	Results	Reference ranges
Bilirubin	0.8	Adult: 0.3–1.2
SGPT (ALT)	22	U/L… Male: < 50; Female: < 35
Alkaline phosphatase	141	U/L… Male: 30–120; Female: 35–105
Gamma GT	22	U/L… Male: < 55; Female: < 38
Creatine	0.3 mg/dL	Male: 0.7–1.2; Female: 0.5–0.9
RBS (random blood sugar)	103 mg/dL	110–170
HCV (chromatography)	Non‐reactive
HBsAg (chromatography)	Non‐reactive

Abbreviations: ALT, alanine transaminase; Gamma GT, gamma glutamyl transferase; HBsAg, hepatitis B surface antigen; HCV, hepatitis C virus; RBS, random blood sugar; SGPT, serum glutamic pyruvic transaminase.

Following a cutaneous biopsy, the biopsy report revealed subepidermal bullae, necrotic keratinocytes, and a detached necrotic epidermis. Hypergranulosis, hyperkeratosis, and parakeratosis were also observed. Basal vacuolar damage is also observed in this region. The dermis had mild inflammatory infiltration, including neutrophils, histiocytes, lymphocytes, and a small number of eosinophils. There was also evidence of perivascular and peri‐adnexal inflammatory infiltrations. The granular layer of the epidermis had variable thickness. There were no indications of granuloma or cancer. Immunofluorescence analysis of the affected skin revealed a dermal–epidermal junction with IgG, IgM, and fibrinogen clumps deposited there, but no C3. Immunofluorescence analysis of a biopsy sample of light‐exposed uninvolved skin revealed normal results. The patient was diagnosed with Discoid LE.

LP/LE overlap syndrome was diagnosed based on the clinical and histological characteristics of LP and discoid LE.

2.3. Treatment

Due to the insufficient clinical response to the topical use of clobetasol propionate, a systemic treatment was started. The patient began a regimen of azathioprine at a dosage of 50 mg once daily, administered at bedtime. Azathioprine was chosen for its immunosuppressive properties, and the dosage was determined carefully based on the patient's weight. Considering that the suggested initial dose is approximately 1 mg/kg and the patient weighed 50 kg, the decision was made to start treatment at the minimal effective dose of 50 mg. This careful strategy was aimed at reducing the likelihood of side effects while monitoring therapeutic effectiveness. The plan involved progressively increasing the dosage if there was no improvement with the initial treatment.

Simultaneously, Deflazacort, an oral corticosteroid, was prescribed to be taken twice daily, preferably in the morning and evening or in the morning and at bedtime, based on the patient's preference and tolerance.

Following the start of this regimen, the patient began to demonstrate significant clinical improvement within 2–3 weeks. As illustrated in Figures 1, 2, 3, 4, the lesions showed reduced redness, flattening, and a decrease in inflammatory characteristics, reflecting a favorable therapeutic response. The treatment was generally well tolerated, and consistent monitoring was scheduled to evaluate efficacy and address any potential side effects.

FIGURE 1.

Large scaly patch with scarring alopecia on the scalp, which has recovered after treatment.

FIGURE 2.

A significant improvement in lesions on the trunk.

FIGURE 3.

Lesions in the mucosal lining of the oral cavity.

FIGURE 4.

Recovering lesions on the upper extremity.

3. Discussion

We present a unique case of LP/discoid LE overlap syndrome involving lesions on the scalp, limbs, dorsum of the hand, and mucosa of the oral cavity in a 30‐year‐old female patient. There was no pain or burning sensation in the lesions. Laboratory findings and dermatological examinations confirmed LP/discoid LE overlap syndrome. The initial treatment included clobetasol propionate, but no improvement was observed in the lesions. Azathioprine was prescribed at a dose of 200 mg once daily, and a prescription for the corticosteroid Deflazacort was issued. This treatment significantly improved the lesions within 4–5 weeks of the follow‐up.

LP–LE overlap syndrome is a combination of two types of dermatitis. Currently, the illness is regarded as a distinct classification [8]. The combination of histological and immunological characteristics in this condition makes it difficult to distinguish between the two diseases. It is important to clearly define the terms “co‐existenc” and “overlap” to avoid confusion [11, 12].

To date, approximately 50 such cases have been reported. The overlap syndrome is thought to have various causes, such as genetics, autoimmunity, viruses, or medications [15]. The definition of this syndrome is still a topic of controversy. Some studies have suggested that the true overlap between LE and LP is characterized by the coexistence of both in the same lesion. Nagao et al. described a patient with a single lesion that exhibited the characteristics of both LE and LP. This was confirmed through both DIF and histopathological studies [13]. Table 3 provides a detailed description of LP–LE Overlap Syndrome in the Literature for the past two decades.

TABLE 3.

Reported cases of lichen planus–lupus erythematosus (LP–LE) overlap syndrome in the literature for the past two decades.

Author (year)	Age	Sex	Presentation	Diagnosis	Treatment
Lospinoso et al. (2018)	NA	NA	Cutaneous lesions, palmoplantar involvement, and nonpainful lesions	LP–LE overlap syndrome	Acitretin
Schmitz et al. (2018)		M	Exacerbation of pruritic erythematous scaly plaques	LP–LE overlap syndrome	Hydroxychloroquine with oral prednisone
Yi Liu et al. (2021)	48	F	Alopecia, skin lesions accompanied by itching on her face, trunk, and limbs, and photosensitivity	BLP/SLE overlap syndrome	Glucocorticoid combined with acitretin and immunosuppressive
Smirnov et al. (2019)	42	F	Mildly pruritic skin changes	Squamous cell carcinoma of the bilateral arms	Azathioprine
Nagao et al. (2006)	53	F	Violaceous erythema around the nostrils and upper lips and atrophic scaly erythema on the cheeks and neck	LE/LP overlap syndrome	Topical tacrolimus
Asma et al. (2019)			Pruritic and painful violaceous erythroderma	LP and dermatomyositis overlap syndrome	Methotrexate
Sandhu et al. (2020)	10	M	Persistent non‐pruritic multiple hyperpigmented scaly papules and plaques over the body	Psoriasis‐LP overlap	Acitretin 25 mg and narrowband ultraviolet B (NBUVB) phototherapy
Priyanka et al. (2015)	40	F	Multiple erythematous lesions on both upper and lower extremities and dorsa of the hand. Oral cavity: erosions with scalloped borders on the bilateral buccal mucosa with diffuse cheilitis	LE/LP overlap	Tenofovir, Lamivudine, Efavirenz
Kushner et al. (2019)	77	F	Discoid, scaly, pigmented, telangiectatic, and atrophic patches on the upper cheeks; alopecia; perifollicular erythema	Combination of LPPigm and FFA
Kiyani et al. (2018)	42	F	Rough white patches on the buccal mucosa. Low‐grade fever, malaise, and lethargy. Swelling around her knees, tingling of her fingers, and itching of the facial skin. Intraoral examination revealed bilateral, white, lace‐like (lichenoid) striations on the buccal mucosa	SLE and reticular OLP	Hydroxychloroquine and prednisone
Tukenmez et al. (2011)	26	M	Erythematous lesions on his back and itching papules on upper and lower extremities. Erythematous on his back, butterfly rash involving the nose and malar region, erythema on his ears and neck, and violaceous lichenoid papules on his upper and lower extremities	LE/LP overlap	Tacrolimus, systemic retinoids and cyclosporine

Abbreviations: BLE, bullous lupus erythematosus; FFA, frontal fibrosing alopecia; LE, lupus erythematosus; LP, lichen planus; LPPig, lichen planus pigmentosus; SLE, systemic lupus erythematosus.

Most instances occur in individuals aged 25–45 years, with a slight predominance of females. Skin lesions primarily affect the outer parts of the arms, legs, face, and torso, and often involve the palms and soles. On the extremities, painful, bluish‐red, scaly plaques and patches with central atrophy, hypopigmentation, and telangiectasia are commonly observed. Additionally, verrucous and papulonodular lesions are present on the hands and arms. Other reported symptoms include nail dystrophy, mucous membrane involvement, and scarring alopecia. Typical LP and discoid LE (DLE) lesions are rare. The likelihood of developing systemic LE is 5%–10% [9].

The case described here has a distinctive feature, which is the amalgamation of the clinical, histological, and immunological traits of LP and LE. The patient was diagnosed based on the presence of signs of both LE and LP. Irregularly shaped pink‐red papules and plaques with clear boundaries on smooth skin were among the signs of LP, along with lesions on the mucous membrane of the oral cavity. Histological examination revealed band‐like lymphohistiocytic infiltration in the upper dermis. The symptoms commonly seen in LE include pink scarring, hair loss with flaking, and thinning of the skin. The histological indications of LE include thinning, vacuolar degeneration of skin cells, areas of slight fluid accumulation between skin cells, and localized accumulation of lymphohistiocytic cells around blood vessels and hair follicles in the dermis. The distinction between LE–LP overlap syndrome and hypertrophic LP lies in the peri‐eccrine lymphocytic infiltrate, a unique feature that also sets it apart from keratoacanthomas with a lichenoid tissue reaction. LE–LP overlap syndrome can be distinguished from DLE by the existence of IgM in cystoid bodies and shaggy fibrinogen staining on DIF, a characteristic observed in LP or LP overlap syndrome [12]. In LP, most patients exhibit a “micro‐woun” that is continually being healed through epidermal migration and increased basal cell division. Conversely, in classic LE, the erosive process tends to be more prominent, and the healing process is less evident, despite surprisingly high labeling indices. This observation may clarify why moderate acanthosis and hyperkeratosis are more prevalent in LP compared to LE, where epidermal atrophy is more frequently observed [6].

Immunological testing is crucial for diagnosing LP–LE overlap syndrome, as stated by Jicha et al. in their paper. Confirming the “classic” form of the overlap syndrome involves obtaining a positive ANA testing result, detecting antibodies to the nuclear antigen, identifying anti‐dsDNA antibodies, and finding antiphospholipid antibodies [10]. The ANA testing turned out to be positive in our circumstances.

In a case reported by Demirci et al., topical corticosteroids were the only form of therapy that was attempted. Significant improvement was observed within a span of just 2 weeks [7]. Based on individual clinical observations, the effectiveness and safety of systemic and topical therapy for the overlap syndrome were assessed, as full‐scale randomized controlled trials are not feasible according to the literature data [14]. One of the most notable points in the case we reported was the patient's lack of response to topical clobetasol propionate, which is known to be successful in treating both LP and discoid LE. Her reaction to azathioprine, on the other hand, was excellent, which highlights the necessity of more vigorous systemic therapy in situations when topical treatments are ineffective. The effectiveness of azathioprine raises the possibility that immunosuppressive treatment may be required for the successful management of overlapping autoimmune diseases. To the best of our knowledge, this is the first report of a combination of LE–LP overlap in a moderately immunocompromised patient effectively and securely treated with azathioprine.

4. Patient's Perspective

The patient felt relieved to finally obtain a diagnosis following a long time of uncertainty. She was especially grateful for the clear understanding of her condition and the transition to azathioprine after topical treatments proved ineffective. This positive response not only alleviated her physical symptoms but also greatly enhanced her psychological well‐being and self‐confidence.

5. Take‐Home Message

This case emphasizes the significance of recognizing overlap syndromes even when systemic symptoms are not present. It stresses the necessity for thorough clinical and histopathological assessments, particularly in cases that do not respond to standard treatments. Additionally, it points out the possible effectiveness of systemic immunosuppressants such as azathioprine when traditional therapies are ineffective.

6. Conclusion

The case of a 30‐year‐old woman with LP/LE overlap syndrome serves as a reminder of the complexities inherent in autoimmune diseases. The unusual presentation without systemic symptoms challenges conventional wisdom regarding these conditions and underscores the importance of comprehensive diagnostic evaluations. Moreover, her resistance to topical corticosteroids, coupled with a favorable response to azathioprine, highlights the necessity for customized treatment strategies to manage overlap syndromes.

As our understanding of these complex interactions deepens, it is crucial to develop guidelines that can accommodate the diverse presentations observed in clinical practice. In summary, this case not only enriches our understanding of dermatological overlap syndromes but also encourages ongoing exploration into effective treatments tailored to individual patient needs. We can improve outcomes for patients facing similar challenges in their autoimmune journeys by fostering greater awareness and research on these conditions.

This case report has a few limitations. Pre‐operative clinical images could not be obtained, and histopathological as well as immunofluorescence photomicrographs were not available since the biopsy report was only presented to the patient in written format. Furthermore, although the diagnosis was made based on distinctive clinical and histological characteristics, the lack of visual documentation might hinder interpretability for certain readers. Despite these limitations, the case provides important clinical insights, especially regarding diagnostic difficulties and treatment response.

Author Contributions

Shehdev Meghwar: conceptualization, data curation, project administration, supervision, validation, visualization, writing – original draft, writing – review and editing. Muskan Batra: conceptualization, project administration, supervision, validation, visualization, writing – original draft, writing – review and editing. Noor Zehra Shabbir: validation, writing – original draft, writing – review and editing. Preety Devi: conceptualization, project administration, validation, visualization, writing – original draft, writing – review and editing. Ajeet Singh: validation, visualization, writing – original draft, writing – review and editing. Asma Ikram: validation, visualization, writing – original draft, writing – review and editing. Amogh Verma: validation, visualization, writing – original draft, writing – review and editing.

Ethics Statement

The authors have nothing to report.

Consent

A written informed consent was obtained from the patient based on the journal's policies.

Conflicts of Interest

The authors declare no conflicts of interest.

Meghwar S., Batra M., Shabbir N. Z., et al., “A Rare Case of Lichen Planus/Discoid Lupus Erythematosus Overlap Syndrome and Its Successful Treatment,” Clinical Case Reports 13, no. 8 (2025): e70704, 10.1002/ccr3.70704.

Data Availability Statement

The authors have nothing to report.