DR [Dermatology Reports 2011; 3:e48] [page 107] Lupus erythematosus and lichen planus overlap syndrome:a case report with a rapid response to topical corticosteroid therapy Gulsen Tukenmez Demirci,1 Ilknur Kıvanç Altunay,1 Sezgi Sarıkaya,1 Damlanur Sakiz2 1Dermatology Department and 2Pathology Department, Sisli Etfal Training and Research Hospital, Istanbul, Turkey Abstract Lupus erythematosus (LE) and lichen planus (LP) may occur as an overlap syndrome. We report the clinical characteristics of a young man with lesions diagnosed as LE and LP by histopathological and direct immunoflurosence examinations. We achieved remarkable clinical response from the treatment with topical corti- costeroids and no recurrence was seen in a 6 months of follow up time. We found this case interesting because of the rapid improvement with corticosteroid and discussed if there is a real overlap or a coexistence according to the literature. Introduction Lupus erythematosus (LE) and lichen planus (LP) are two distinct and well estab- lished dermatoses which occasionally can occur as an overlap syndrome. Overlap syn- drome is characterized by mixed clinical and histopathological features of both LE and LP.1 Although LE and LP are relatively common dis- eases, an overlap is considered as an uncom- mon entity. Approximately 50 cases of LE/LP overlap syndrome have been reported in the lit- erature.2 However, some authors suggest that most of the cases could be missed as a conse- quence of its variable clinical and histopatho- logical appearances.2,3 We report a case diag- nosed as LE/LP overlap syndrome with a rapid improvement to topical corticosteroid treat- ment. There was no recurrence at 6 months. Case Report A 26-year-old male presented to our outpa- tient clinic with a 8-months history of persist- ent, erythematous lesions on his back and widely scattered mildly itching papules on upper and lower extremities. Dermatological examination revealed ery- thematous, slightly scaly, irregularly bordered, infiltrated large plaques with central atrophy on his back, a butterfly type rash involving nose and malar region and erythema on his ears and neck. (Figure 1A and 1B), The skin of his retroauricular regions were intact. He also had widespread violaceous lichenoid papules on his upper and lower extremites (Figure 1C), but no mucosal or nail involvement was noted. Laboratory examinations, including com- plete blood counts, erythrocyte sedimantation rate, routine urine tests were within normal limits except for mild elevations of alanin transaminase and aspartate aminotransferase (44 U/L, 46 U/L; normal limits 0-35 U/L, 0- 45U/L) Antinuclear antibodies, Anti-dsDNA, Anti-ssA, Anti-ssB, Anti-Sm were all negative. Complement C3 and C4 levels were all normal. Two cutaneous biopsies were taken from a plaque on the back and from the dorsum part of one hand. Histopathology of the biopsy speci- men from his back which showed thinning of the epidermis, basal layer vacuolar degenera- tion, perifollicular chronic inflammatory infil- trates and deposition of mucin in the dermis was consistent with subacute cutaneous LE. Mucin deposition was also shown with alcian blue staining. Direct immunofluorescence (DIF) examination of the plaque lesion revealed deposits of immunoglobulin (IgM> IgG, IgA) and C3 forming a granuler pattern (feature of LE) and linear fibrinogen deposi- tion at the basal membrane zone (BMZ) (fea- ture of LP). (Figure 2) A biopsy specimen from the dorsum of the hand was consistent with the diagnosis of LP with hypergranulosis, with a band-like mononuclear infiltrate at the dermo-epidermal junction (Figure 3). The patient was diagnosed as having LE/LP overlap syndrome after clinical, histopatholog- ical and immunohistological examinations. He was treated topically with mometasone furoat 0.1% cream applied twice daily for two weeks. A rapid improvement of the lesions were seen at the end of the second week. Discussion LE/LP overlap syndrome can be diagnosed with the combination of clinical, histopatho- logical and/or immunopathological features of both diseases in the same patient and/or at the same lesion of one patient.2 Histopathological features can be consistent with either LP or LE or both while DIF usually suggests former.4 There is still some controversy regarding the definion of this syndrome. It is suggested that true LE\LP overlap is defined as the presence of LE and LP in the same lesion, whereas the presence of LE features in one lesion and LP features in other one should be considered as a coexistence of LE and LP rather than over- lap.5,6 Nagao et al. reported a true overlapped LE\LP patient presenting with single lesion showing combined features of LE and LP which was confirmed by both DIF and histopathologi- cal studies. Furthermore, in a study conducted by De Jong et al., with the immunohistochem- ical examination of the markers for extracellu- lar matrix proteins, it was asserted that LE/LP overlap syndrome can be considered as LP-like LE rather than as a distinct disease.7 The lesions on the back in our patient were more consistent with LE clinically and histopatho- logically,while those on his hands were consis- tent with LP clinically and histopathologically. DIF taken from his back showed combined immunfluorescence features of LP and LE. In most cases clinical and histopathological fea- tures enable us to differantiate between LE and LP, but it may be difficult where as lesions are less typical or an overlapping occurs. Both diseases may show similar histopathological and immunopathological findings of basement membrane changes and colloid bodies.6,8,9 As for therapy, topical tacrolimus 0.1%, sys- temic retinoids and cyclosporine have been reported to be effective in the treatment of this condition.1,10,11 We only tried topical corticos- teroids. A dramatic improvement occurred in only two weeks. In fact, topical steroids are considered to be inadequate in each disease and systemic therapy is needed. It is interest- ing to obtain a good response with only topical therapy and also in a relatively short period of 2 weeks in our patient. Follow up seems to be mandatory because the chance of conversion of the syndrome into systemic lupus erythe- Dermatology Reports 2011; volume 3:e48 Correspondence: Gulsen Tukenmez Demirci, Sisli Etfal Eğitim ve Araştırma Hastanesi Dermatoloji Kliniği, 19 Mayıs Cad. Etfal sok. Sisli, 34377 Istanbul, Turkey Tel. +90.212.373.5000 - Fax. +90.212.353.5976. E-mail: gulsentukenmez@yahoo.com Key words: cutaneous lupus erythematosus, dis- coid lupus erythematosus, lichenoid eruption. Received for publication: 16 June 2011. Revision received: 4 July 2011. Accepted for publication: 6 October 2011. This work is licensed under a Creative Commons Attribution NonCommercial 3.0 License (CC BY- NC 3.0). ©Copyright G.T. Demirci et al., 2011 Licensee PAGEPress, Italy Dermatology Reports 2011; 3:e48 doi:10.4081/dr.2011.e48 No n- co mm er cia l u se on ly [page 108] [Dermatology Reports 2011; 3:e48] matosus is reported to be about 5-10%.12 We did not see any recurrence during 6 months follow-up. Conclusions We report a case which can be identified as LE/LP coexistence depending on the clinical picture. Although deposition of immunoglobu- lines in a granuler pattern and fibrinogen in a linear pattern at BMZ supports a true overlap- ping, the lack of cytoid bodies staining with IgM prevents us from suggesting true overlap of LE/LP. Additionally, success of local corticos- teroid therapy alone in this combination of two difficult diseases is another debateble issue. We think it is necessary with more data from more patients to delineate LE/LP overlap and coexistence. It seems that the mistery of the disease continues to remain. References 1. Kim H, Pomeranz MK. Lupus erythemato- sus/lichen planus overlap syndrome. J Drugs Dersmatol;3:311-2. 2. Inaloz HS, Chowdhury MMU, Motley RJ. Lupus erythematosus/lichen planus over- lap syndrome with scarring alopecia. J Eur Acad Dermatol Venereol 2001;15:171-4. 3. Mahler V, Hornstein OP, Meyer S, et al. Lupus erythematosus/lichen ruber planus overlap syndrome. 5 cases in a patient sample of the Erlangen University Dermatology Clinic (1894-1895). Hautarzt 1998;49:295-302. 4. James WD, Berger TG, Elston DM. Andrews' Diseases of the Skin: Clinical Dermatology. 10th ed. New York: Elsevier; 2006. 5. Nagao K, Chen KR. A case of lupus erythe- matosus/lichen planus overlap syndrome. J Dermatol 2006;33:187-90. 6. Lever WH, Schaumburg-Lever G. Histopa- thology of the Skin, 7th ed. JB Lippincott Company: Philadelphia; 1990. pp 494–505. 7. De Jong EM, Van der Vleuten CJ, Van Vlijmen-Willems IM. Differences in extra- cellüler matrix proteins, epidermal growth and differentiation in discoid lupus ery- thematosus, lichen planus and the overlap syndrome. Acta Derm Venereol 1997;77: 356-60. 8. Romero RW, Nesbitt LT Jr, Reed RJ. Unusual variant of lupus erythematosus or lichen planus. Clinical, histopathologic and immunofluorescent studies. Arch Dermatol 1977;113:741-8. 9. Camisa C, Neff JC, Olsen RG. Use of indi- rect immunofluorescence in the lupus ery- thematosus/lichen planus overlap syn- drome: an additional diagnostic clue. J Am Acad Dermatol 1984;11:1050-9. 10. Grabbe S, Kolde G. Coexisting lichen planus and subacute cutaneous lupus ery- thematosus. Clin Exp Dermatol 1995;20: 249-54. 11. Tursen U, Oz O, Ikizoglu G, et al. A case of lichen planus lupus erythematosus overlap syndrome with eyelid involvement. Eur J Ophthalmol 2002;12:244-6. 12. Jablonska S, Blaszozyk M. Lupus erythe- matosus. What’s new? J Eur Acad Derma- tol Venereol 2000;15:103-5. Case Report Figure 2. Deposition of Immunoglobulin G, Immunoglobulin M, C3 and fibrinogen in Lupus erythematosus (LE) and lichen planus (LP) lesions on the back has been shown by direct immunofluorescent (Direct immunofluorescence ¥ 200). Figure 3. Histopathologic features of the specimen from the dorsum of the hand. The presence band-like mononuclear cell infiltration and hypergranulosis (Hemato- xylin and eosin stain ¥ 100). Figure 1. A) Erythematous, slightly, scaly, irregularly bordered, infiltrated three large plaques with central atrophy on the back. B) Butterfly type rash involving the nose and malar region. C) Violaceous lichenoid papules on the upper and lower extremities. A B C No n- co mm er cia l u se on ly