Original articleCoexistence of lichen sclerosus and morphea: A retrospective analysis of 472 patients with localized scleroderma from a German tertiary referral center
Section snippets
Patients
A retrospective chart review of patients with LoS who presented at the outpatient clinic for connective tissue diseases of the Department of Dermatology at Ruhr University Bochum was performed. Observation period was 11 years (January 1, 2000, through December 31, 2010). A protocol on the evaluation of connective tissue diseases including LoS and LiS (individual ethics submission code: 2851) was approved by the ethics review board of the Ruhr University Bochum.
Clinical examination and classification of LoS subtypes
To be eligible for this
Demographic characteristics
A total of 472 patients with LoS were given a diagnosis and treated at the Department of Dermatology at Ruhr University Bochum from January 1, 2000, through December 31, 2010. The demographic characteristics are detailed in Table I. Similar to previous reports, there was a much higher proportion of women affected by the disease, with an overall female to male ratio of 3.5:1 in adults and 8:1 in children.
Distribution of LoS subtypes
The distribution of LoS subtypes in the overall cohort, and in adults and children is
Discussion
This retrospective analysis of a large cohort of patients with LoS demonstrates that an essential proportion of these patients have concomitant LiS. The demographics and subtype distribution of our cohort largely confirms previous investigations, showing that the majority of patients with LoS are women, that the plaque type (morphea) is most frequent in adults, and that linear types of LoS dominate in childhood.2, 3, 4
The possible coexistence of both LoS and LiS has been well known for many
Conclusions
Accumulating evidence exists that a subset of patients with LoS, namely those with morphea and generalized LoS, have a significantly increased risk for the development of concomitant LiS. This high LiS prevalence in LoS is suggestive for a common pathogenetic background. Moreover, patients with LoS frequently present other autoimmune disorders. These findings should definitively have an impact on our practical management. Clinical examination of patients with LoS should include inspection of
References (17)
- et al.
Pediatric morphea (localized scleroderma): review of 136 patients
J Am Acad Dermatol
(2008) - et al.
Update on morphea, part I: epidemiology, clinical presentation, and pathogenesis
J Am Acad Dermatol
(2011) - et al.
Lichen sclerosus
Lancet
(1999) - et al.
Autoantibodies to extracellular matrix protein 1 in lichen sclerosus
Lancet
(2003) - et al.
Morphea and lichen sclerosus et atrophicus: clinical and histopathologic studies in patients with combined features
J Am Acad Dermatol
(1980) - et al.
AWMF guideline No. 013/066: diagnosis and therapy of circumscribed scleroderma
J Dtsch Dermatol Ges
(2009) - et al.
Distinct autoimmune syndromes in morphea: a review of 245 adult and pediatric cases
Arch Dermatol
(2009) - et al.
Juvenile localized scleroderma: clinical and epidemiological features in 750 children: an international study
Rheumatology (Oxford)
(2006)
Cited by (97)
Juvenile Localized Scleroderma: Updates and Differences from Adult-Onset Disease
2021, Rheumatic Disease Clinics of North AmericaCitation Excerpt :The Pediatric Rheumatology European Society criteria (Fig. 2) performed better than the Mayo Clinic or German criteria in classifying 944 adults and children.19 Table 1 presents data from 10 pediatric rheumatology and/or dermatology cohorts,4–6,20–26 including the largest international jLS cohort (750 patients)2 and 5 pediatric and adult-onset cohorts.4,6,20–22 Most patients with jLS (56%) had linear scleroderma, with trunk or limb more often affected than the head (Table 2).
Clinical characteristics and histopathologic changes of morphea: A single-center, retrospective study of 137 patients
2021, Journal of the American Academy of DermatologyAutoimmune and Cutaneous Inflammatory Comorbidities in Adult-Onset Morphea in the All of Us Research Program
2024, American Journal of Clinical DermatologyS2k guideline: Diagnosis and therapy of localized scleroderma
2024, JDDG - Journal of the German Society of Dermatology
Funding sources: None.
Conflicts of interest: None declared.