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Lupus erythematosus-lichen planus overlap

syndrome in an HIV-infected individual

Article in International Journal of STD & AIDS · November 2015

DOI: 10.1177/0956462415618109

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 Int J STD AIDS OnlineFirst, published on November 17, 2015 as doi:10.1177/0956462415618109
Case report
Lupus erythematosus-lichen
planus overlap syndrome
in an HIV-infected individual
Priyanka Patil, Chitra Nayak, Swagata Tambe and Dipti Das
Abstract
Lupus erythematosus-lichen planus overlap syndrome is an uncommon disorder with clinical, histological and/or immu-
nopathological features of both diseases. We report a case of lupus erythematosus-lichen planus overlap syndrome in a
patient with HIV infection. To the best of our knowledge, lupus erythematosus-lichen planus overlap syndrome with HIV
infection has never been reported in literature.
Keywords
Lichen planus, lupus erythematosus, human immunodeficiency virus
Date received: 3 July 2015; accepted: 28 October 2015
Introduction
Lupus erythematosus-lichen planus (LE-LP) overlap
syndrome is an uncommon disorder. There is an overlap
of clinical, histological and/or immunofluorescence fea-
tures of both diseases. Diagnosis depends on correlation
of clinical, histological and immunological features.
Coexistence of HIV with autoimmune conditions is
rare. We report a female patient with HIV infection
with this syndrome.
Case report
A 40-year-old widow with retroviral disease on
treatment with tenofovir, lamivudine and efavirenz
(CD4 count – 682 cells/l) presented with multiple
red, flat, painful lesions on both extremities and back,
with raw, painful lesions in the oral cavity associated
with a burning sensation that had lasted for two
months. The patient gave a history suggestive of photo-
sensitivity, with a burning sensation on her face when
exposed to the sun. There was no history of joint pain,
chest pain, breathlessness, palpitation, abdominal dis-
tention, pedal oedema or seizure disorder. Four months
beforehand, she had experienced multiple violaceous
scaly papules and plaques on the upper trunk and extre-
mities, with oral mucosal involvement (Figure 1(a), (b)
and (c)), with a biopsy suggesting LP (Figure 1(d)). She
was treated with oral prednisolone in tapering doses
over three months and oral dapsone and topical
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mometasone furoate 0.1% cream, without much
improvement. There was a history of LP five years
prior, treated with oral corticosteroids and dapsone,
with lesions healing with hyperpigmentation. The
patient was diagnosed with retroviral disease 10 years
ago and has been on antiretroviral therapy for nine
years. Her baseline CD4 count before initiation of anti-
retroviral therapy was 279. Table 1 depicts her CD4
count over time and the development of cutaneous
and mucosal lesions.
On cutaneous examination, there were multiple
erythematous to depigmented atrophic scaly plaques
with peripheral hyperpigmentation on extensors of
both upper and lower extremities and back
(Figure 2(a) and (b)). Examination of the oral cavity
revealed buccal erosions with scalloped borders and
also diffuse cheilitis (Figure 2(c)).
The patient had microcytic hypochromic anaemia
(haemoglobin – 10 g/dl), a raised erythrocyte sedimen-
tation rate (40 mm in 1 h), raised T3, T4 with normal
Department of Dermatology, Topiwala National Medical College and
B.Y.L. Nair Hospital, Mumbai, India
Corresponding author:
Priyanka Patil, Department of Dermatology, OPD No. 14, Second Floor,
OPD Building, B.Y.L. Nair Hospital, Mumbai Central, Mumbai 400008,
India.
Email: drpriyankapatil219@gmail.com
2 International Journal of STD & AIDS 0(0)

Figure 1. Four months prior. (a) Multiple erythematous to violaceous scaly papules on dorsum of hand. (b) Reticular pattern in
buccal mucosa. (c) Multiple erythematous to violaceous scaly papules and plaques on leg. (d) Histopathology, H & E, 400, Biopsy
from hypertrophic violaceous plaque on left lower leg showed compact hyperkeratosis, wedge shaped hypergranulosis with evidence
of interface dermatitis – suggestive of lichen planus.

TSH (T3 – 203.7, T4 – 13.6, TSH – 1.8). Anti-nuclear
antibody was positive in a speckled pattern at 1:1000
titres. Anti-dsDNA and anti-histone antibody were
negative. An ultrasound scan of the abdomen and
pelvis revealed an anterior wall heterogeneous fibroid
measuring 3.8  3.4 cm. Tests for HBsAg, anti-HCV
antibody and VDRL were non-reactive. Fundus exam-
ination was normal.
A biopsy from the buccal mucosa revealed a subepi-
dermal cleft (Max Joseph space) with a dense lympho-
histiocytic infiltrate hugging the dermoepidermal
junction (Figure 2(d)). A biopsy from the atrophic
plaque on the back revealed compact hyperkeratosis,
wedge-shaped hypergranulosis and basal cell degener-
ation with interface lymphocytic infiltrate, all suggest-
ive of LP. Features of LE viz. deep perivascular and
periappendageal infiltrate with mucin were also present
(Figure 2(e)).
Direct immunofluorescence from the atrophic lesion
on the back and uninvolved skin from the buttock had
features of both LP and LE. There was a granular base-
ment membrane zone band staining positive for IgM
and C3, with colloid bodies in the papillary dermis
staining positive for IgM, IgA, C3 and epidermal
ANA staining with IgG (Figure 2(f)).
Considering the clinical features and histopatho-
logical and immunofluorescence findings, we reached
a diagnosis of LE-LP overlap syndrome.
The patient underwent a hysterectomy for the
uterine fibroid. She was advised on photoprotection,
prescribed oral chloroquine 250 mg twice daily, topical
mometasone furoate 0.1% cream in morning and top-
ical tacrolimus 0.1% ointment at night for skin lesions,
and topical tacrolimus 0.03% ointment at night for
oral lesions. After one month, there was no improve-
ment in oral and cutaneous lesions, so oral methorexate
10 mg weekly was added. The lesions gradually
healed with hypopigmentation and oral erosions
completely resolved after one month of therapy
(Figure 3(a) and (b)).

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 Patil et al.

Table 1. Correlation of clinical features with CD4 count.

CD4 count
Cutaneous examination Sites Clinical diagnosis (cells/l) ART regimen Skin biopsy

Present episode Multiple erythematous to depigmented Extensors of both upper and LE-LP overlap 682 Tenofovir, Features of
atrophic scaly plaques with periph- lower extremities, dorsae of lamivudine, LP and LE
eral hyperpigmentation hands and feet and back efavirenz
Oral cavity: erosions with scalloped
borders on bilateral buccal mucosa
with diffuse cheilitis
4 months prior Multiple violaceous scaly papules and Upper trunk and both upper and Lichen planus 511 Tenofovir, Lichen
plaques lower extremities lamivudine, planus
Oral mucosa: reticular pattern in bilat- nevirapine
eral buccal mucosa
5 years prior Multiple violaceous scaly papules and Extensors of both upper and Lichen planus 655 Stavudine, Not done
plaques lower extremities, dorsae of lamivudine,
Oral cavity: multiple irregular, atrophic hands and feet and back nevirapine

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plaques with white streaks in lacy
pattern with violaceous hue on
buccal mucosa
10 years prior 279 (baseline) Before initiation
of ART
ART: antiretroviral therapy; LE-LP: Lupus erythematosus-lichen planus.
3
4 International Journal of STD & AIDS 0(0)

Figure 2. Present episode. (a) Multiple erythematous to depigmented atrophic scaly plaques with peripheral hyperpigmentation on
dorsae of hands. (b) Multiple erythematous to depigmented atrophic scaly plaques with peripheral hyperpigmentation on lower
extremities. (c) Erosions with scalloped borders on buccal mucosa with diffuse cheilitis. (d) Histopathology, H & E, 100, Biopsy from
the buccal mucosa revealed Max Josephs space with dense lymphohistiocytic infiltrate hugging the dermoepidermal junction. (e)
Histopathology, H & E, 100, Biopsy from atrophic plaque on back revealed compact hyperkeratosis, wedge shaped hypergranulosis,
basal cell degeneration with interface lymphocytic infiltrate – suggestive of lichen planus with deep perivascular and periappendageal
infiltrate with evidence of mucin, suggestive of discoid lupus erythematosus. (f) Direct immunofluorescence (100) of uninvolved skin
from buttock shows granular BMZ band with IgM and C3, colloid bodies in the papillary dermis staining with IgM, IgA, C3 and
epidermal ANA staining with IgG suggestive of lupus erythematosus and lichen planus.

Figure 3. (a) Lesions on dorsae of hands healed with hypopigmentation. (b) Complete resolution of lesions in oral cavity after one
month of therapy.

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Patil et al.
Discussion
LE-LP overlap syndrome has features of both LP and
LE. Approximately 50 cases have been reported so far.1
Various etiologies postulated for this overlap syndrome
are genetic, autoimmune, viral or drugs.2 Drugs known
to cause LE-LP overlap syndrome are isoniazid,
procainamide and acebutolol. It is seen that in a
genetically predisposed individual, diseases with auto-
immune aetiology may occur simultaneously, as
seen by reported associations with vitiligo,2 pemphigus
foliaceus,3 Hashimoto’s thyroiditis, Reynold’s
syndrome,4 hypothyroidism, cryoglobulinemia and
hypocomplementemia.5
Most of the cases occur between the ages of 25 and
45 years, with slight female preponderance. Cutaneous
lesions mostly affect the distal arms, legs, face and trunk
with palmoplantar involvement. Characteristically, pain-
ful, bluish red, scaly, centrally atrophic plaques and
patches with hypopigmentation and telangiectasia are
commonly seen on extremities, as well as verrucous, papu-
lonodular lesions on the hands and arms. Nail dystrophy,
mucous membrane involvement and scarring alopecia
have also been reported. Classic lesions of LP and discoid
lupus erythematosus are uncommon. Chances of develop-
ing systemic LE are 5–10%.6
Histologically, features of LP and LE are usually
present in the same biopsy.7 In our patient, hyperkera-
tosis, wedge-shaped hypergranulosis, Max Joseph
cleft and dense, band-like lymphocytic infiltrate was
suggestive of LP. The presence of deep perivascular
and peri-appendageal infiltrate with evidence of mucin
suggested LE.
However, the histopathological findings are some-
times insufficient as a distinguishing feature in overlap-
ping cases due to similarities between the two disorders.
Direct immunofluorescence (DIF) is helpful in histolo-
gically doubtful cases. In LP, DIF reveals groups of
cytoid bodies that stain for IgM, IgG and C3 intraepi-
dermally and for fibrin in a fibrillar pattern along the
dermo-epidermal junction. DIF of LE lesions, in add-
ition to IgM staining of cytoid bodies, usually displays
immunoglobulin and complement deposition in a
granular pattern along the dermo-epidermal junction.
Our patient exhibited these mixed features of both
LE and LP. This LE-LP overlap syndrome has a
chronic course with a variable response to treatment.
The medications effective in this syndrome are potent
topical and systemic steroids, oral retinoids,8 ciclos-
porin,9 Hydroxychloroquine10 and topical tacrolimus
0.1% ointment.
To our knowledge, association of LE-LP overlap in
a moderately immunocompromised patient who was
successfully and safely treated with methotrexate is
being reported for the first time.
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5
Our patient was doing well on antiretroviral therapy,
had not suffered from any opportunistic infection and
had severe mucosal and cutaneous involvement not
responding to topical therapy and oral chloroquine.
Financial constraints precluded the use of acetretin;
therefore, we prescribed oral methotrexate.
Methotrexate has been successfully used in reported
cases of HIV-related CD8þ cutaneous pseudolym-
phoma, Burkitt lymphoma, non-Hodgkin lymphoma,
polyarthritis and placenta percreta. Methotrexate in
HIV-positive patients with psoriasis was contraindi-
cated based on reports of Duvic et al.11 in 1987.
However, reports by Maurer et al.12 did not reveal
occurrence of opportunistic infection with moderate
immunosuppression. Currently, according to a recently
published consensus on treatment for psoriasis in
patients with HIV, methotrexate is considered for
severe refractory psoriasis after the failure of first-line
treatment. Methotrexate can be considered in patients
of LE-LP overlap syndrome with HIV who do not
respond to topical therapy and chloroquine in
resource-limited setting.
Acknowledgement
The authors thank Dr Alka Gupta, Professor, Department of
Obstetrics & Gynaecology, King Edward Memorial (KEM)
Hospital, Mumbai.
Declaration of Conflicting Interests
The author(s) declared no potential conflicts of interest with
respect to the research, authorship, and/or publication of this
article.
Funding
The author(s) received no financial support for the research,
authorship, and/or publication of this article.
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