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Toward a Better Understanding of the Spectrum of Morphea

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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 pa- tients 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 geni- tal 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. Msclerotic, and dyschromic skin and patho- logically 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- ARCH DERMATOL/ VOL 148 (NO. 1), JAN 2012 WWW.ARCHDERMATOL.COM 24 ©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 pa- tients with typical morphea. Furthermore, in clinical Table 1. Classification of Morpheaa practice, genital examination is not systematically per- formed 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 re- cruited 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 question- naire 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 mor- phea 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- ARCH DERMATOL/ VOL 148 (NO. 1), JAN 2012 WWW.ARCHDERMATOL.COM 25 ©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 .
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