Toward a Better Understanding of the Spectrum of Morphea

STUDY

ONLINE FIRST High Frequency of Genital Lichen Sclerosus in a Prospective Series of 76 Patients With Morphea Toward a Better Understanding of the Spectrum of Morphea

Virginie Lutz, MD; Camille Francès, MD, PhD; Didier Bessis, MD; Anne Cosnes, MD; Nicolas Kluger, MD; Julien Godet, PhD; Erik Sauleau, PhD; Dan Lipsker, MD, PhD

Objective: To compare the frequency of genital lichen diagnosis was 54 (13-87) years. Forty-nine patients had sclerosus (LS) in patients with morphea with that of con- plaque morphea, 9 had generalized morphea, and 18 had trol patients. linear morphea. Three patients (3%) in the control group and 29 patients (38%) with morphea had LS (odds ra- Design: A prospective multicenter study. tio,19.8; 95% CI, 5.7-106.9; PϽ.001). Twenty-two patients with plaque morphea (45%) and only 1 patient with Setting: Four French academic dermatology depart- linear morphea (6%) had associated genital LS. ments: Strasbourg, Montpellier, Tenon Hospital Paris, and Henri Mondor Hospital Cre´teil. Conclusions: Genital LS is significantly more frequent in patients with morphea than in unaffected individu- Patients: Patients were recruited from November 1, 2008, als. Forty-five percent of patients with plaque morphea through June 30, 2010. Seventy-six patients with mor- have associated LS. Complete clinical examination, in- phea and 101 age- and sex-matched controls, who un- cluding careful inspection of genital mucosa, should there- derwent complete clinical examination, were enrolled. fore be mandatory in patients with morphea because genital LS bears a risk of evolution into squamous cell Interventions: A complete clinical examination and, if deemed necessary, a cutaneous biopsy. carcinoma and thus needs treatment with topical corti- costeroids. Main Outcome Measure: The frequency of genital LS. Arch Dermatol. 2012;148(1):24-28. Results: There were 58 women and 18 men (a 3:1 ra- Published online October 17, 2011. tio) with a median age of 59 years. Mean (range) age at doi:10.1001/archdermatol.2011.305

ORPHEA AND LICHEN been described.21 Morphea involves the sclerosus (LS) are 2 skin, but the extremities, the face, and the entities that are char- aerolar area are usually spared, while LS acterized clinically by usually involves the genital mucosa. Skin plaques of indurated, involvement can occur in LS but is rare. sclerotic,M and dyschromic skin and pathologically by an inflammatory dermal in- CME available online at filtrate and dermal fibrosis. Their cause is www.jamaarchivescme.com largely unknown, although both genetic Although there are some similarities be- See Practice Gaps tween morphea and LS, their exact rela- at end of article tionship remains debated. Some au- thors22 consider that LS is a superficial factors, such as predisposing HLA alleles, variant of morphea occurring mostly in the and environmental factors, such as infec- genital area, whereas others23-25 consider tion with Borrelia burgdorferi, have been that they are 2 unrelated entities. In some involved in some cases.1-15 Autoimmune cases involving the skin, referred to as diseases and/or stigmata are more fre- “white spot disease,” the differential di- quent in patients with morphea or LS than agnoses between morphea and LS can be in unaffected persons.1,16-20 impossible. However, lesions occurring on Author Affiliations are listed at Different clinicopathologic variants of genital mucosa are usually considered syn- the end of this article. morphea, summarized in Table 1, have onymous with LS. Yet, to our knowl-

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©2012 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/25/2021 Corrected on February 10, 2012 edge, no study has evaluated the frequency of LS in patients with typical morphea. Furthermore, in clinical Table 1. Classification of Morpheaa practice, genital examination is not systematically performed in patients with morphea. Neither is it recom- Type Variant Common Denomination mended in most dermatology textbooks.24,26,27 How- Plaque morphea Superficial Common form, guttate ever, it is important to diagnose genital LS if present, morphea, atrophoderma of because this entity bears a significant risk of squamous Pasini and Pierini 28-30 cell carcinoma. This risk can probably be reduced by Deep Common morphea, early and sustained treatment with topical corticoste- nodular/keloid roids.31-33 Thus, the aim of this study was to evaluate the morphea, frequency of genital LS in patients with morphea. Schulman’s fasciitis Linear/segmentary Facial, monomelic, Progressive hemifacial morphea dimetic, truncal, atrophy of Parry and METHODS hemicorporeal Romberg, “en coup de sabre” morphea Generalized Usually deep Generalized morphea This is a prospective multicenter study. Patients were recruited from November 1, 2008, through June 30, 2010, in the a According to Barete et al.21 departments of dermatology from 4 French university hospi- tals: Strasbourg, Montpellier, Tenon Hospital Paris, and Henri Mondor Hospital Cre´teil. Patients were included if the diag- (range) age was 54 (13-87) years. The mean (range) du- nosis of morphea was confirmed clinically by an experienced ration of morphea was estimated to be 7.9 years (6 dermatologist or after a skin biopsy. Data were collected on a months–36 years), but it was not specified in 46 cases. standardized questionnaire and included age and sex. The vari- The diagnosis was made on typical clinical findings in ous forms of morphea were specified according to the classi- 50 patients and was confirmed by a biopsy in 26. fication used in this study (Table 1): plaque, linear, and/or gen- One hundred one controls were included (68 women eralized. The number of plaques, their size, their location, their and 33 men). The mean (range) age was 57 (1-87) years. clinical description, and the functional consequences were re- There was no significant statistical difference between the ported. When patients had both plaque morphea and linear le- patients and the control group for age (P=.44) and sex sions, they were classified as having linear morphea. The geni- (P=.30). tal area was examined in every patient by an experienced dermatologist to search for signs of LS. The diagnosis of LS was Forty-nine patients had plaque morphea, 18 had lin- accepted in the case of typical clinical and/or histopathologic ear morphea, and 9 had generalized morphea. In only 1 findings. Patients with systemic sclerosis as defined by the cri- patient, the clinicopathologic findings were typically those teria of the American College of Rheumatology were not in- of extragenital LS. This patient had pathologically con- cluded in this study. firmed plaques of both morphea and extragenital LS. The The control patients in our study consisted of patients seen clinical characteristics in these patients are summarized in the dermatology department for a reason other than mor- in Table 2. phea and who had a complete skin and mucosal examination. Most were followed up as part of surveillance of cutaneous ma- FREQUENCY OF GENITAL LS lignancies. Some had inflammatory diseases, such as psoriasis or lupus erythematosus. Statistical analyses were performed in collaboration with the Three patients (3%) in the control group and 29 pa- Secteur Biostatistiques et Me´thodologies at the Universite´de tients (38%) with morphea had LS. Thus, compared with Strasbourg. Before starting the study, we estimated that the num- the frequency in the control group, genital LS is signifi- ber of patients to be included, assuming that the incidence of cantly more frequent in patients with morphea, with an LS in the general population is 1 in 300 to 1 in 1000 and that odds ratio of 19.8 (95% CI, 5.7-106.9; PϽ.001). the expected difference in prevalence would be 10%, would be The frequency of LS according to type of morphea is 50 to 80 patients with a power of 95%. illustrated in Figure 1. Forty-five percent of patients with The main objective was to compare the frequency of geni- plaque morphea and only 6% of patients with linear mor- tal LS in patients with morphea with that of the control group. phea had genital LS (PϽ.001). For this purpose, a comparison test was conducted between the Twenty percent of patients had genital pruritus. In- frequency of genital LS in these 2 groups. The odds ratio was calculated by the Fisher exact test. We compared the age and terestingly, none of the patients spontaneously com- sex between case patients and the control group by the ␹2 plained about this symptom, which was always revealed method. Under French law, this type of study, which does not through specific questioning. involve any invasive investigation but relies on a questionnaire performed during a regular consultation, does not need INCIDENT CASES the approval of the institutional review board. Twenty-seven patients were incident cases of morphea who RESULTS did not see a dermatologist before this study and in whom diagnosis of morphea was previously not established. Their mean (range) age was 50.8 (13-84) years, and the female EPIDEMIOLOGY to male ratio was 21:6. The mean (range) duration of morphea was estimated as 4.6 years (6 months–33 years). Of We included 76 patients with morphea. This group con- these 27 patients, 13 (48%) had genital LS. The mean sisted of 18 men (24%) and 58 women (76%). The mean (range) age of the 13 patients with genital LS was 64.9 (14-

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©2012 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/25/2021 Corrected on February 10, 2012 Table 2. Clinical Characteristics of Patients With Morphea

Type Characteristic No. of Patients Female/Male Sex Mean Age, ya Plaque morphea (n=49) Common variant 32 27/5 61.3 Atrophoderma of Pasini and Pierini 10 9/1 46.0 Keloid morphea 1 1/0 62.0 Guttate morphea 1 1/0 26.0 Deep morphea 3 2/1 68.0 Schulman’s fasciitis 2 1/1 64.5 Linear morphea (n=18) Band 6/2 38.0 Upper limbs 1 Lower limbs 4 Upper and lower limbs 2 Hemicorporeal 1 En coup de sabre 6 5/1 Parry Romberg syndrome 4 2/2 Generalized morphea . . . 9 4/5 62.6

a At inclusion.

A 49 Had plaque morphea 22 Had LS

76 Patients 18 Had linear morphea 1 Had LS

9 Had generalized morphea 6 Had LS

Figure 1. Frequency of genital lichen sclerosus (LS) according to type of morphea.

84) years. Ten of the 13 patients had plaque morphea (Figure 2) and 3 had generalized morphea.

COMMENT

B This study shows that genital LS is significantly more frequent in patients with morphea than in controls and that LS is found with an unexpected high frequency of 38% in patients with morphea. The prevalence of LS in the population is difficult to evaluate, but it is estimated from 1 in 300 to 1 in 1000. Thus, the frequency of LS in the controls of this study is ten times higher than the frequency usually estimated to occur in the general population. This can be explained by a selection bias in our control group of patients mainly recruited in a dermatology department and examined by experienced dermatologists. However, it should also raise some doubt about the usually published data regarding prevalence rates of LS in the general population. It might Figure 2. A 66-year-old patient with multiple plaques of morphea (A) and biopsy-proven genital lichen sclerosus (B). be that the exact prevalence of LS in the general population is largely underestimated. Indeed, evaluation of prevalence is difficult because the manifestation may be asymp- quency of genital LS between controls and patients with tomatic, patients do not consult for discomfort because of morphea. This was not the case because there is still a large the genital location of the lesions, and many physicians and significant difference. are not familiar with this entity and are thus unable to cor- Genital LS was significantly more frequent in pa- rectly diagnose it. Only a systematic genital examination tients with plaque morphea than in patients with linear by an experienced practitioner will provide valuable preva- morphea. This observation further supports the fact that lence rates. In any case, this unusually high frequency of those 2 entities might result from different pathomecha- LS in the control group could have harmed this study by nisms. Only 1 patient with linear morphea had associ- lowering our ability to demonstrate a difference in the fre- ated LS. There were, however, too few patients with lin-

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©2012 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/25/2021 Corrected on February 10, 2012 ear morphea included in this study to draw any definitive quisition of data: Lutz, Francès, Bessis, Cosnes, Kluger, conclusion about a possible association. Patients with and Lipsker. Analysis and interpretation of data: Lutz, plaque morphea are those with the highest risk of asso- Godet, Sauleau, and Lipsker. Drafting of the manuscript: ciated genital LS, with occurrences in about 45% of Lutz, Sauleau, and Lipsker. Critical revision of the manu- patients. Furthermore, when we restrict our analysis to script for important intellectual content: Lutz, Francès, Bes- patients with incident morphea, that is, those who were sis, Cosnes, Kluger, Godet, Sauleau, and Lipsker. Statis- not diagnosed with morphea before entering this study, tical analysis: Godet and Sauleau. Study supervision: we find a comparatively high frequency of LS of 48%. This Francès, Bessis, Cosnes, and Lipsker. fact could suggest that LS usually precedes morphea. The Financial Disclosure: None reported. association between morphea and LS has been reported Additional Information: Drs Lutz, Francès, Bessis, Cosnes, previously, mainly in patients with plaque morphea,34-40 and Lipsker are members of the Study Group of Sys- but it was reported only once in a case of linear mono- temic Diseases in Dermatology, France. melic morphea in a young boy.40 In most cases, how- 35,37-39,41 35 ever, the LS was extragenital. Only 1 study re- REFERENCES ported 7 cases of patients with morphea and genital LS. The fact that 38% of patients with morphea have asso- 1. Powell JJ, Wojnarowska F. Lichen sclerosus. Lancet. 1999;353(9166):1777-1783. ciated genital LS strongly supports the fact that these 2 2. Peterson LS, Nelson AM, Su WP, Mason T, O’Fallon WM, Gabriel SE. The epi- diseases share common pathogenetic pathways, and pos- demiology of morphea (localized scleroderma) in Olmsted County 1960-1993. sibly a common genetic background, if they are not one J Rheumatol. 1997;24(1):73-80. 3. Aberer E, Stanek G, Ertl M, Neumann R. Evidence for spirochetal origin of cir- and the same disease. Indeed, both entities are chronic cumscribed scleroderma (morphea). Acta Derm Venereol. 1987;67(3):225- inflammatory skin diseases characterized by dermal fi- 231. brosis. Lichen sclerosus could be the genital manifesta- 4. Eisendle K, Grabner T, Zelger B. Focus floating microscopy: “gold standard” for tion of morphea. cutaneous borreliosis? Am J Clin Pathol. 2007;127(2):213-222. 5. Eisendle K, Grabner T, Zelger B. Morphoea: a manifestation of infection with Bor- In conclusion, this study clearly shows that LS is sig- relia species? Br J Dermatol. 2007;157(6):1189-1198. nificantly more frequent in patients with morphea. This 6. Buechner SA, Winkelmann RK, Lautenschlager S, Gilli L, Rufli T. Localized sclero- finding should definitely affect our clinical practice. In- derma associated with Borrelia burgdorferi infection: clinical, histologic, and immu- deed, it is not the standard of care to completely un- nohistochemical observations. J Am Acad Dermatol. 1993;29(2, pt 1):190-196. dress patients with morphea, and this practice is not rec- 7. Schempp C, Bocklage H, Lange R, Ko¨lmel HW, Orfanos CE, Gollnick H. Further 24,26,27 evidence for Borrelia burgdorferi infection in morphea and lichen sclerosus et ommended in the major dermatology textbooks. The atrophicus confirmed by DNA amplification. J Invest Dermatol. 1993;100(5): results of this study, however, show that it is mandatory 717-720. to perform a complete examination, including the geni- 8. Lecerf V, Bagot M, Revuz J, Touraine R, Dournon E. Borrelia burgdorferi and lo- tal mucosa. That will allow us to diagnose a substantial calized scleroderma. 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The high rate of familial lichen sclerosus suggests a genetic contribution: an observa- pitalier Universitaire de Strasbourg, Strasbourg; Service tional cohort study. J Eur Acad Dermatol Venereol. 2010;24(9):1031-1034. de Dermatologie, Hoˆpital Tenon, Universite´ Pierre et Ma- 15. Gao XH, Barnardo MC, Winsey S, et al. The association between HLA DR, DQ rie Curie-Paris 6, Paris (Dr Francès); Universite´ Mont- antigens, and vulval lichen sclerosus in the UK: HLA DRB112 and its associated pellier I, Service de Dermatologie, Centre Hospitalier Uni- DRB112/DQB10301/04/09/010 haplotype confers susceptibility to vulval lichen sclerosus, and HLA DRB10301/04 and its associated DRB10301/04/DQB10201/ versitaire de Montpellier, Montpellier (Dr Bessis); and 02/03 haplotype protects from vulval lichen sclerosus. J Invest Dermatol. 2005; Service de Dermatologie, Hoˆpital Henri Mondor, Cre´- 125(5):895-899. teil (Dr Cosnes), France; and Departments of Dermatol- 16. Leitenberger JJ, Cayce RL, Haley RW, Adams-Huet B, Bergstresser PR, Jacobe ogy, Allergology and Venereology, Institute of Clinical HT. Distinct autoimmune syndromes in morphea: a review of 245 adult and pe- Medicine, University of Helsinki, Skin and Allergy Hos- diatric cases. Arch Dermatol. 2009;145(5):545-550. 17. Majeed M, Al-Mayouf SM, Al-Sabban E, Bahabri S. Coexistent linear sclero- pital, Helsinki University Central Hospital, Finland (Dr derma and juvenile systemic lupus erythematosus. Pediatr Dermatol. 2000; Kluger). 17(6):456-459. Correspondence: Dan Lipsker, MD, PhD, Clinique Derma- 18. Bonifati C, Impara G, Morrone A, Pietrangeli A, Carducci M. Simultaneous oc- tologique, 1, Place des l’Hoˆpital, BP 426, 67 091 Strasbourg currence of linear scleroderma and homolateral segmental vitiligo. J Eur Acad Dermatol Venereol. 2006;20(1):63-65. CEDEX, France ([email protected]). 19. Meyrick Thomas RH, Ridley CM, McGibbon DH, Black MM. Lichen sclerosus et Author Contributions: Study concept and design: Lutz, atrophicus and autoimmunity—a study of 350 women. Br J Dermatol. 1988; Francès, Bessis, Cosnes, Kluger, Sauleau, and Lipsker. Ac- 118(1):41-46.

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©2012 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/25/2021 Corrected on February 10, 2012 20. Aslanian FM, Marques MT, Matos HJ, et al. HLA markers in familial lichen sclerosus. 32. Pugliese JM, Morey AF, Peterson AC. Lichen sclerosus: review of the literature J Dtsch Dermatol Ges. 2006;4(10):842-847. and current recommendations for management. JUrol. 2007;178(6):2268- 21. Barete S, Lipsker D, Francès C. Morpheés. In: Allanore Y, Cabane J, Mouthon L, 2276. eds. Scle´rodermies [in French]. 2nd ed. Paris, France: Med-Line; 2011:209-226. 33. van de Nieuwenhof HP, van der Avoort IA, de Hullu JA. Review of squamous pre- 22. Peterson LS, Nelson AM, Su WP. Classification of morphea (localized scleroderma). malignant vulvar lesions. Crit Rev Oncol Hematol. 2008;68(2):131-156. Mayo Clin Proc. 1995;70(11):1068-1076. 34. Tremaine R, Adam JE, Orizaga M. Morphea coexisting with lichen sclerosus et 23. Sehgal VN, Srivastava G, Aggarwal AK, Behl PN, Choudhary M, Bajaj P. Local- atrophicus. Int J Dermatol. 1990;29(7):486-489. ized scleroderma/morphea. Int J Dermatol. 2002;41(8):467-475. 35. Farrell AM, Marren PM, Wojnarowska F. Genital lichen sclerosus associated with 24. Puzenat E, Humbert Ph, Aubin F. Scle´rodermies localiseés: les morpheés. In: Sau- morphoea or systemic sclerosis: clinical and HLA characteristics. Br J Dermatol. rat JH, Lachapelle JM, Lipsker D, Thomas L, eds. Dermatologie et Infections Sex- 2000;143(3):598-603. uellement Transmissibles [in French]. 5th ed. Paris, France: Masson; 2009:364-374. 36. Wu KH, Dai YS, Tsai MJ, et al. Lichen sclerosus et atrophicus, bullous morphea, 25. Freedberg IM, Eisen AZ, Wolff K, et al. Scleroderma. In: Freedberg IM, Eisen AZ, and systemic lupus erythematosus: a case report. J Microbiol Immunol Infect. Wolff K, Austen KF, Goldsmith LA, Katz S, eds. Fitzpatrick’s Dermatology in Gen- 2000;33(1):53-56. eral Medicine. Vol 1. 3rd ed. New York, NY: McGraw-Hill; 1999:1842-1850. 37. Kim DH, Lee KR, Kim TY, Yoon MS. Coexistence of lichen sclerosus with mor- 26. Champion RH, Burton JL, Burns DA, Breathnach SM. Perniosis: localized morphea. phoea showing bilateral symmetry. Clin Exp Dermatol. 2009;34(7):e416-e418. In: Rook A, Wilkinson DS, Ebling FJG, eds. Rook-Wilkinson-Ebling Textbook of Der- doi:10.1111/j.1365-2230.2009.03396.x. matology. Vol 3. 6th ed. Oxford, England: Blackwell Science Ltd; 1998:2502-2512. 38. Connelly MG, Winkelmann RK. Coexistence of lichen sclerosus, morphea, and 27. Yu BD, Eisen AZ. Morphea. In: Freedberg IM, Eisen AZ, Wolff K, Austen FK, Gold- smith LA, Katz S, eds. In: Fitzpatrick’s Dermatology in General Medicine. Vol II. lichen planus: report of four cases and review of the literature. J Am Acad Dermatol. 6th ed. New York, NY: McGraw-Hill; 2010:1709-1718. 1985;12(5, pt 1):844-851. 28. Derrick EK, Ridley CM, Kobza-Black A, McKee PH, Neill SM. A clinical study of 23 39. Sawamura D, Yaguchi T, Hashimoto I, Nomura K, Konta R, Umeki K. Coexis- cases of female anogenital carcinoma. Br J Dermatol. 2000;143(6):1217-1223. tence of generalized morphea with histological changes in lichen sclerosus et 29. Sergeant A, Vernall N, Mackintosh LJ, McHenry P, Leman JA. Squamous cell atrophicus and lichen planus. J Dermatol. 1998;25(6):409-411. carcinoma arising in extragenital lichen sclerosus. Clin Exp Dermatol. 2009; 40. Mensing H, Schmidt KU. Diffuse fasciitis with eosinophilia associated with mor- 34(7):e278-e279. doi:10.1111/j.1365-2230.2008.03195.x. phea and lichen sclerosus et atrophicus. Acta Derm Venereol. 1985;65(1):80- 30. Wang SH, Chi CC, Wong YW, Salim A, Manek S, Wojnarowska F. Genital verru- 83. cous carcinoma is associated with lichen sclerosus: a retrospective study and 41. Lampert A, Fortier-Beaulieu M, Thomine E, Young P, Lauret P. Association sur review of the literature. J Eur Acad Dermatol Venereol. 2010;24(7):815-819. un membre d’un lichen scle´reux et d’une scle´rodermie monome´lique [associa- 31. Cooper SM, Gao XH, Powell JJ, Wojnarowska F. Does treatment of vulvar lichen tion of lichen sclerosus and monomelic scleroderma] [in French]. Ann Dermatol sclerosus influence its prognosis? Arch Dermatol. 2004;140(6):702-706. Venereol. 1995;122(3):102-104.

PRACTICE GAPS

Missing Genital Lichen Sclerosus in Patients With Morphea Don’t Ask? Don’t Tell?

ichen sclerosus (LS) is an autoimmune inflam- The practice gap of failing to diagnose genital LS in matory dermatosis that most commonly afflicts patients with morphea likely begins with an incomplete L the female genitalia, with an estimated preva- history. Dermatologists may be reluctant to inquire lence of 1 in 300 to 1 in 1000. An increased frequency of about genital symptoms out of fear of “opening Pando- autoimmune disorders, including thyroid disease, viti- ra’s box.” Patients may not consider their genital symp- ligo, alopecia areata, and pernicious anemia, occurs in toms to be relevant to their dermatologic evaluation. Fi- patients with vulvar LS compared with controls.1 A di- nally, patients may be reluctant to report genital agnosis of LS should prompt evaluation for other auto- symptoms. Lutz et al found that 20% of their patients immune diseases, but what conditions should prompt with genital LS had genital pruritus when questioned, physicians to investigate for genital LS? but none spontaneously disclosed this symptom. A The article by Lutz et al2 demonstrates an increased brief discussion between patient and physician regard- frequency of genital LS in males and females with plaque, ing the rationale for questioning about genital symp- linear, or generalized morphea. More than 40% of pa- toms will ease the anxiety of those involved and facili- tients with plaque morphea and two-thirds of patients tate a focused interview. with generalized morphea were found to have genital LS. Patients with genital LS most commonly present with Morphea and LS may exhibit overlapping clinical and his- genital pruritus. Dysuria, dyspareunia, painful defeca- topathologic features. Whether morphea and LS repre- tion, and constipation also occur. Genital LS may, how- sent a disease continuum remains debatable, but the pres- ever, be asymptomatic even in the setting of advanced dis- ence of morphea as a marker for genital LS should no ease. Therefore, thorough examination is paramount to longer be overlooked. making the diagnosis. Dermatologists should become com-

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