Plaque Morphea Research Articles

A Report of a Case of Reactivation of Plaque Morphea After Sunlight Exposure and a Literature Review

A Report of a Case of Reactivation of Plaque Morphea After Sunlight Exposure and a Literature Review

Novel multispectral imaging to predict disease progression in pediatric morphea.

Morphea, or localized scleroderma, is an inflammatory, fibrosing skin disorder that can be progressive and debilitating. Infrared thermography frequently has false positive results. The aim of this study was to assess the ability of multispectral imaging to predict disease progression in children with morphea. Children with morphea were recruited between 2016 and 2022. Multispectral images of affected and matched contralateral unaffected sites were obtained using the Antera™ 3D camera. Clinical assessment was performed using the Localized Scleroderma Assessment Tool (LoSCAT). Children were followed up every 3 months for imaging and clinical review. The main outcome measurement was correlation of hemoglobin gradient between affected and matched contralateral unaffected tissue and progression. Of 17 children, the average age was 12 years (range 6-18 years); most were female (76.5%) and white (94.1%). Nearly two-thirds (64.7%) had linear morphea, 35.2% had plaque morphea; 58.8% had been treated with systemic agents. The average LoSCAT score was 20.6 (range 5-73). The average hemoglobin gradient between affected and matched contralateral unaffected skin was four times higher in those who had progression (average differential 0.3, range 0.1-0.4) compared to those who did not (average differential 0.08, range 0.02-0.15). Using a cut off of a 0.18 hemoglobin gradient between affected and unaffected skin, the sensitivity of multispectral imaging for detecting progression in pediatric morphea is 90% with specificity of 100%. Multispectral imaging is a novel assessment tool with promising accuracy in predicting progression as an adjunct to clinical assessment in pediatric morphea. Further research should examine its performance against thermography.

A Case of Hidradenitis Suppurativa with Plaque Morphea: Association or Chance?

Abstract Hidradenitis suppurativa (HS) is a chronic inflammatory disease of the apocrine glands characterized clinically by recurrent nodules, abscesses, and discharging sinuses which heal with a bridge or rope-like scars in the axilla, groin, and perineum. HS is seen to be associated with several other autoimmune and pilosebaceous structural disorders. We present the case of a 50-year-old obese female, known case of HS for 3 years who developed hyperpigmented indurated plaques over the breasts for the past 1 year. Histopathology from the plaques showed thickened and homogenized collagen bundles. A diagnosis of plaque morphea was made based on the clinical and histopathological findings. There are few case reports of HS associated with connective tissue diseases (CTDs) such as systemic lupus erythematosus, Sjogren’s syndrome, and systemic vasculitis. We discuss the shared pathogenesis of HS and CTDs which may have led to morphea developing in our patient.

Plaque psoriasis and morphea in a patient on ustekinumab: A case report

A large number of autoimmune and fibrosing conditions associated with psoriasis and morphea have been reported in the literature. Currently, the relationship between psoriasis and morphea is unknown, and the coexistence of these conditions is quite rare. Due to the few patients affected by both conditions, and the lack of knowledge concerning their pathogenic mechanisms, the cause behind this co-occurrence is poorly understood. Here, we present a case of morphea under a plaque of psoriasis in a patient managed on ustekinumab.

Nevoid telangiectasia patches in a 17-year-old boy

A 17-year-old-boy presented to the Dermatology service with a 5-month history of mildly pruritic rash over his trunk and limbs. On examination, several erythematous telangiectatic patches were noted over his chest, bilateral flanks, hips, and lower limbs (Fig 1, A and B). These were accompanied by tenosynovitis over bilateral hands. 5 months after the initial review, the telangiectatic patches regressed with development of hypopigmented sclerotic plaques over these sites (Fig 1, C-E). A skin biopsy was arranged, and histology revealed thick dermal collagen fibers, with a perivascular and peri-adnexal lymphohistiocytic infiltrate within the superficial to deep dermis (Fig 2, A and B). Question 1: What is the most likely diagnosis for these telangiectatic patches ?A.Port wine stainB.Unilateral nevoid telangiectasiaC.Telangiectasia macularis eruptiva perstansD.MorpheaE.Benign hereditary telangiectasia Answer:A.Port wine stain – Incorrect. These present from birth as erythematous or violaceous patches.B.Unilateral nevoid telangiectasia – Incorrect. These may be congenital or acquired, and are characterized by multiple, unilateral and linearly arranged, blanchable telangiectases in a dermatomal (most frequently involving the third and fourth cervical dermatomes) or Blaschkoid pattern.1Kreft B. Marsch W. Wohlrab J. Unilateral nevoid telangiectasia Syndrome.Dermatology. 2004; 209: 215-217https://doi.org/10.1159/000079892Crossref PubMed Scopus (27) Google ScholarC.Telangiectasia macularis eruptiva perstans – Incorrect. This is characterized by brownish erythematous macules and telangiectasia usually located at the chest and limbs.2Costa D.L. Moura H.H. Rodrigues R. Pineiro-Maceira J. Ramos-E-Silva M. Telangiectasia macularis eruptiva perstans: a rare form of adult mastocytosis.J Clin Aesthet Dermatol. 2011; 4: 52-54PubMed Google ScholarD.Morphea – Correct. This is consistent with the skin biopsy results. This case also highlights telangiectatic patches as a unique early presentation of morphea, before its evolution to well-recognized hypopigmented sclerotic plaques.E.Benign hereditary telangiectasia – Incorrect. Distribution of telangiectasia is generalized or in photo-exposed areas. Patients do not have any systemic involvement. Morphea, also known as localized scleroderma, is an autoimmune condition characterized by inflammation and sclerosis of the skin and underlying soft tissues.3Abbas L. Joseph A. Kunzler E. Jacobe H.T. Morphea: progress to date and the road ahead.Ann Transl Med. 2021; 9: 437https://doi.org/10.21037/atm-20-6222Crossref PubMed Google Scholar It is characterized by distinctive cutaneous inflammation and fibrosis, and damage which produce atrophy. Unlike systemic scleroderma, morphea does not often involve the extra-cutaneous systems and also lacks the specific antibodies as found in systemic scleroderma.3Abbas L. Joseph A. Kunzler E. Jacobe H.T. Morphea: progress to date and the road ahead.Ann Transl Med. 2021; 9: 437https://doi.org/10.21037/atm-20-6222Crossref PubMed Google Scholar While early presentations are often of an erythematous patch or edematous plaque, morphea can also appear as telangiectatic patches. Question 2: What subtype of morphea does this patient have?A.CircumscribedB.GeneralizedC.LinearD.PanscleroticE.Mixed Answer:A.Circumscribed – Incorrect. Otherwise also known as plaque morphea, it begins as an erythematous-to-violaceous, edematous plaque of several centimeters and is most often located on the trunk with 3 or less lesions.B.Generalized – Correct. Defined as 4 or more morpheaform plaques involving at least 2 different anatomic sites.C.Linear – Incorrect. One or more linear streaks of cutaneous induration that may involve the dermis, subcutaneous tissue, muscle, and underlying bone.D.Pansclerotic – Incorrect. A very rare variant presenting with extensive full-thickness skin involvement sparing acral skin.E.Mixed – Incorrect. A combination of 1 or more morphea subtypes. The more modern and clinically appropriate classification, the ‘Pauda criteria’, divides morphea into circumscribed, generalized, linear, pansclerotic, and mixed subtypes. Each subtype has different clinical manifestations and degree of involvement of the subcutaneous soft tissues.4Florez-Pollack S. Kunzler E. Jacobe H.T. Morphea: current concepts.Clin Dermatol. 2018; 36: 475-486https://doi.org/10.1016/j.clindermatol.2018.04.005Abstract Full Text Full Text PDF PubMed Scopus (49) Google Scholar The patient’s findings were consistent with generalized morphea. Question 3: Which is the most common extracutaneous manifestation associated with morphea?A.GastrointestinalB.NeurologicalC.Musculoskeletal/articularD.VascularE.Respiratory Answer:A.Gastrointestinal – Incorrect. This includes gastrointestinal reflux.B.Neurological – Incorrect. Such as in the en coup de sabre type, neurological abnormalities can include migraines, trigeminal neuralgia, seizures, behavioral changes, and learning disability.5Zulian F. Vallongo C. Woo P. et al.Localized scleroderma in childhood is not just a skin disease.Arthritis Rheum. 2005; 52: 2873-2881https://doi.org/10.1002/art.21264Crossref PubMed Scopus (260) Google ScholarC.Musculoskeletal/articular – Correct. This is the most common extracutaneous manifestation of morphea and includes sacroiliitis, generalized synovitis, and inflammatory arthritis.D.Vascular – Incorrect. This can include Raynaud’s phenomenon, deep vein thrombosis, and vasculitis.E.Respiratory – Incorrect. This includes restrictive pulmonary disease, bronchopneumonia. In addition to involvement of joints and deeper subcutaneous areas underlying the lesions of morphea, patients may also experience other non-localized musculoskeletal manifestations. In a study of 750 patients with morphea, 22.4% had extracutaneous manifestations which included articular (47.2%), neurologic (17.1%), vascular (9.3%), ocular (8.3%), gastrointestinal (6.2%), respiratory (2.6%), cardiac, and renal.5Zulian F. Vallongo C. Woo P. et al.Localized scleroderma in childhood is not just a skin disease.Arthritis Rheum. 2005; 52: 2873-2881https://doi.org/10.1002/art.21264Crossref PubMed Scopus (260) Google Scholar Our patient’s rheumatological manifestation, with his initial presentation of tenosynovitis, likely represented early extra-cutaneous manifestations of morphea. None disclosed.

Morphea disease activity during pregnancy: A case series.

Little is known about pregnancy in women with morphea. We aimed to describe the clinical presentation, pregnancy outcomes, and medical management of morphea during pregnancy. We conducted a case series of female patients of reproductive age (18-49 years) who were part of the longitudinal MAC (Morphea in Adults and Children) cohort seen in the outpatient dermatology clinic at the University of Texas Southwestern from July 2007 to February 2022. Women who were pregnant during research visits and had at least 6months of follow-up in the MAC cohort were included. Data collected included demographics, morphea characteristics, pregnancy outcomes, and medication history. Median clinical disease activity and damage scores using the Localized Scleroderma Cutaneous Assessment Tool were recorded. Ten patients were pregnant during the study period. Five patients had pediatric-onset morphea and five had adult-onset morphea. Eight patients had linear and two had plaque morphea. Six patients had at least one morphea lesion on their abdomen. Median age at first pregnancy was 31 years (interquartile range [IQR], 26.0-35.8 years) and median duration of morphea was 13.5 years (IQR, 4.8-19.0 years). In seven patients, damage scores were stable with no increased morphea activity. Three patients (30%) experienced reactivation of morphea activity during pregnancy with a median Localized Scleroderma Activity Index of 4 (IQR, 2.5-10). Only one patient required immunosuppressive therapy during pregnancy for her morphea diagnosis. Seven of 10 patients had cesarean deliveries and one adverse fetal outcome was reported. In this small series, most patients maintained stability of their morphea and there were no adverse pregnancy outcomes directly related to morphea.

Painful and oozing bullous lesions in a 74‐year‐old woman

Painful and oozing bullous lesions in a 74‐year‐old woman

AB012. Clinical and demographic features of morphea patients with mucocutaneous involvement: a cross sectional study from the morphea of adults and children (MAC cohort)

While many clinical findings of morphea have been described by our group and others, mucocutaneous findings, including genital or oral lesions, have not been well characterized. To date, we don’t know the frequency, demographic and clinical features associated with mucocutaneus lesions. To address this knowledge gap, we performed a cross-sectional analysis of patients enrolled in the Morphea in Adults and Children Registry from 2007 to 2018. Of the 737 patients analyzed, 48% (n=353) had linear morphea, 31% (n=232) had generalized morphea, 12% (n=87) had plaque morphea and 6% (n=45) had mixed. Oral lesions were present in 23 patients (3%), out of which 20 (87%) had linear morphea, nine had En Coup de Sabre (39%) and 12 had Parry Romberg syndrome (PRS) (52%). Genital lesions were present in 28 patients, the majority of which (24/28, 86%) had generalized morphea. Patients with oral involvement had a younger age of onset when compared to patients with genital involvement (12 and 58 years old, respectively; P<0.001). Seventy-nine percent (n=22) of morphea patients with genital involvement had lichen sclerosus et atrophicus (LsA) overlap, compared to 17% (n=4) with oral involvement (P<0.001). Eighty-three percent (n=19) patients with oral involvement were classified as having morphea profunda, or deep involvement, compared to 14% (n=4) of patients with genital involvement (P<0.001). Median mLoSSI ad PGA-A scores for patients with oral involvement (0, IQR 0–4 and 0, IQR 0–24, respectively)) was lower than patients with genital involvement (10, IQR 6–27 and 23, IQR 15–40, respectively) (P<0.001 and P=0.002, respectively). PGA-D scores were higher in patients with oral involvement (50, IQR 30–60) than in patients with genital involvement (20, IQR 10–25) (P<0.001). In conclusion, have demonstrated that mucocutaneous involvement of morphea lesions occurs most frequently in patients with linear and generalized morphea. Providers may not be aware of the prevalence of these findings and thus they may go undiagnosed for many months or years, leading to the development of potentially permanent and devastating sequalae.

An Evaluation of the Performance of Current Morphea Subtype Classifications

Numerous classification systems for morphea subtypes exist, but none have been systematically evaluated for their ability to categorize patients with morphea into demographically and clinically coherent groups. Although some subtypes, such as linear morphea, are present across all the classification schemes, others list unique subtypes. This creates confusion among investigators and practitioners and impairs accurate categorization of patients for study and clinical evaluation. To evaluate how frequently the commonly used morphea classification systems categorize patients with morphea into clinically relevant subtypes using cross-sectional analysis of 2 large patient cohorts. This cross-sectional study comprised 944 adults and children from 2 prospective cohorts-the Morphea in Adults and Children at the University of Texas Southwestern Medical Center (Dallas, Texas), which enrolled participants from July 20, 2007, to September 21, 2018, and the National Registry for Childhood-Onset Scleroderma at the University of Pittsburgh (Pittsburgh, Pennsylvania), which enrolled participants from October 23, 2002, to November 13, 2018. Patient demographic characteristics, morphea subtype, quality-of-life measures, disease activity, and damage as measured by Localized Scleroderma Cutaneous Assessment Tool scores during initial visits. A total of 944 participants (444 patients with adult-onset morphea and 500 patients with pediatric-onset morphea; 741 female participants [78%]; median age at onset, 16 years [interquartile range, 8-44 years]) were included in this study. Most participants were White (723 [77%]) and had the linear (474 [50%]) or generalized subtype of morphea (244 [26%]). With the use of the previously published Padua criteria, most patients were classified to have linear morphea (474 [50%]), followed by generalized morphea (244 [26%]), plaque morphea (141 [15%]), mixed morphea (38 [4%]), and pansclerotic morphea (3 [0.3%]). Overall, the Padua criteria successfully classified 900 patients (95%) in comparison with the Peterson criteria (533 [56%]) and the European Dermatology Forum classification (487 [52%]). In this cross-sectional study of morphea subtype classification systems, the Padua criteria performed best in classifying patients into subgroups with cohesive demographic and clinical features, supporting its widespread use. However, they have ambiguities that might lead to misclassification, particularly in terms of generalized and pansclerotic morphea and descriptors such as morphea profunda. Consensus-based approaches are needed to address these ambiguities and develop a unified classification scheme.

Plaque Morphea at Distance from Breast Cancer Radiotherapy: A Cost of the Treatment?

Introduction. Morphea is a rare dermatological disease with no known cause, characterized by persistent inflammation, collagen deposition, fibrosis, and atrophy, as well as a considerable reduction in quality of life. Case presentation. An 89-year-old female patient, with a history of arterial hypertension, right mastectomy, and radiation therapy for breast cancer 10 years before, presented for the appearance of two erythematous plaques in the right submammary groove and right flank. The plaques are well-defined, with irregular margins, and have dimensions of 10/5 cm, and 13/8 cm respectively, were infiltrated, with areas of skin retraction and visible capillary network, itchy and painful. They were observed six months before presentation, as a single hyperpigmented lesion that gradually evolved into its current appearance and was treated as a subcutaneous hematoma, without resolution. Laboratory tests showed a nonspecific inflammatory syndrome and an increase in antinuclear antibodies. By corroborating the clinical and paraclinical data, the diagnosis of plaque morphea was made, and the systemic treatment with Penicillin G 2mil IU/12h, 311nm UVB phototherapy was initiated, with favorable evolution. At the subsequent hospitalization, after noticing the increase in the size of the plaques, with their extension to the left hemiabdomen, it was decided to start treatment with Methotrexate 7.5 mg/week, with a favorable evolution and the disappearance of fibrous bands. Conclusions. Although radiotherapy may be the trigger factor of morphea, the time until the onset of the disease (9 years) is consistent with the data presented in the literature (9.4 years).

Congenital Morphea: Two Case Reports

Congenital morphea is a rare and underestimated form of localized scleroderma that presents at birth. From the five known subtypes, linear and plaque morphea are the most common and benign forms and are both described in these cases. Extracutaneous manifestations are rare.

543 Clinical and demographic features of morphea patients with mucocutaneous involvement: A cross sectional study from The Morphea of Adults and Children (MAC Cohort)

While clinical findings of morphea have been described by our group and others, genital and oral lesions have not been well characterized. To address this knowledge gap, a cross-sectional analysis was performed of patients enrolled in The Morphea in Adults and Children Registry from 2007 to 2018. Of 737 patients analyzed, 48% (n=353) had linear morphea, 31% (n=232) had generalized morphea, 12% (n=87) had plaque morphea, and 6% (n=45) had mixed. Oral lesions were present in 23 patients (3%), of which 20 (87%) had linear morphea, nine had En Coup de Sabre (39%) and 12 had Parry Romberg Syndrome (PRS) (52%). Genital lesions were present in 28 patients, the majority of which (86%) had generalized morphea. Patients with oral morphea involvement had a younger age of onset compared to genital involvement (12 and 58 years old, respectively; p < 0.001). Genital morphea patients had extra-genital lichen sclerosus et atrophicus (LsA) in 79% (n=22) as compared to 17% (n= 4) with oral involvement (p < 0.001). Morphea profunda, or deep involvement, was seen in 83% (n = 19) patients with oral involvement versus 14% (n=4) of patients with genital involvement (p < 0.001). Median mLoSSI and PGA-A scores for patients with oral involvement (0, IQR 0-4 and 0, IQR 0-24, respectively) was lower than patients with genital involvement (10, IQR 6-27 and 23, IQR 15-40, respectively) (p < 0.001 and p = 0.002, respectively). PGA-D scores were higher in patients with oral involvement (50, IQR 30-60) than in patients with genital involvement (20, IQR 10-25) (p < 0.001). Our results show that while mucocutaneous lesions are rare in morphea, oral involvement is predominant in patients with facial linear lesions, particularly PRS, while genital lesions predominate in post-menopausal women with overlying extra-genital LsA. Given the function limiting nature of these lesions, practitioners should know clinical signs that put patients at risk mucocutaneous morphea.

Update on Management of Morphea (Localized Scleroderma) in Children.

Juvenile localized scleroderma (morphea) is the predominant scleroderma in childhood which affects the skin and may extend to the underlying fascia, muscle, joints and bone. The assessment of activity and damage can be done with a validated instrument like LoSCAT. Disease classified as “low severity” which includes superficial plaque morphea can be treated with topical mid potent- potent steroids, tacrolimus, calcipotriol or imiquimod in combination with phototherapy. Methotrexate is recommended for linear, deep and generalized morphea. Steroids are effective in the early inflammatory stage and used in combination with methotrexate. Methotrexate is continued for at least 12 months after adequate response is achieved. Mycophenolate mofetil is given in cases where methotrexate is contraindicated or for those who do not respond to methotrexate. There are also reports of improvement of disease with ciclosporine and hydroxychloroquine. In severe cases, recalcitrant to standard therapy there may be a role for biologics, JAK inhibitors, and IVIG. Supportive measures like physiotherapy and psychiatric counseling are also important in the management of morphea. Orthopedic surgery and other measures like autologous fat transfer may be advocated once the disease is inactive.

Clinico-epidemiological Study of Morphea from a Tertiary Care Hospital.

Morphea is an uncommon disease that presents with skin induration and sclerosis. The disease is common in Caucasians and there are few studies describing the clinicoepidemiological profile of these patients from the Indian subcontinent. This prospective study was conducted during a three year period from June 2014 to May 2017. All patients of morphea presenting to the dermatology outpatient department were evaluated for parameters like age, sex, duration, age of onset, clinical subtype and possible disease associations and triggering factors. The data was analysed, tabulated and mean, standard deviation and percentages calculated. 47 patients were incorporated into the study. These comprised of 10 (21.28%) males and 37 (78.72%) females. The average age of the patients was 23.92 ± 12.07 years with a mean age of onset being 22.13 ± 12.51 years. 22 (46.80%) patients presented within one year of onset of disease. Plaque morphea was the commonest type seen in 31 (65.96%) patients followed by linear morphea in 9 (19.15%) patients. We found preceding trauma in 3 patients and morphea developed following herpes zoster and intramuscular injection in one patient each. Morphea is an uncommon disease that is seen predominantly in females and young individuals. Circumscribed plaque morphea is the commonest variant. Certain predisposing factors like trauma can precede it.

High-frequency ultrasonography (HFUS) as a useful tool in differentiating between plaque morphea and extragenital lichen sclerosus lesions.

IntroductionMorphea and lichen sclerosus (LS) are chronic inflammatory diseases that may pose a diagnostic challenge for a physician. High-frequency ultrasonography (HFUS) is a versatile diagnostic method utilized in dermatologic practice, allowing monitoring the course of the disease, treatment response and differentiation between certain skin disorders.AimTo prove the usefulness of HFUS in differentiating between plaque morphea and extragenital LS lesions.Material and methodsWe examined 16 patients with plaque morphea and 4 patients with extragenital LS using 20 MHz taberna pro medicum TM (Germany) device.ResultsInvestigations revealed hyperechogenic entrance echo in both morphea and LS lesions, whereas a distinct polycyclic surface of the entrance echo was detected exclusively in LS.ConclusionsHigh-frequency ultrasonography is a current diagnostic modality that may prove useful in differentiating between morphea and LS lesions.

Fractional carbon dioxide laser versus low-dose UVA-1 phototherapy for treatment of localized scleroderma: a clinical and immunohistochemical randomized controlled study

Morphea is a rare fibrosing skin disorder that occurs as a result of abnormal homogenized collagen synthesis. Fractional ablative laser resurfacing has been used effectively in scar treatment via abnormal collagen degradation and induction of healthy collagen synthesis. Therefore, fractional ablative laser can provide an effective modality in treatment of morphea. The study aimed at evaluating the efficacy of fractional carbon dioxide laser as a new modality for the treatment of localized scleroderma and to compare its results with the well-established method of UVA-1 phototherapy. Seventeen patients with plaque and linear morphea were included in this parallel intra-individual comparative randomized controlled clinical trial. Each with two comparable morphea lesions that were randomly assigned to either 30 sessions of low-dose (30J/cm2) UVA-1 phototherapy (340-400nm) or 3 sessions of fractional CO2 laser (10,600nm-power 25W). The response to therapy was then evaluated clinically and histopathologically via validated scoring systems. Immunohistochemical analysis of TGF-ß1 and MMP1 was done. Patient satisfaction was also assessed. Wilcoxon signed rank test for paired (matched) samples and Spearman rank correlation equation were used as indicated. Comparing the two groups, there was an obvious improvement with fractional CO2 laser that was superior to that of low-dose UVA-1 phototherapy. Statistically, there was a significant difference in the clinical scores (p = 0.001), collagen homogenization scores (p = 0.012), and patient satisfaction scores (p = 0.001). In conclusion, fractional carbon dioxide laser is a promising treatment modality for cases of localized morphea, with proved efficacy of this treatment on clinical and histopathological levels.

Generalized morphea as the first sign of breast carcinoma: a case report

Generalized morphea is a rare idiopathic form of scleroderma that literally means "hard skin." Morphea is usually considered an isolated event that is not associated with malignancy. However, case reports of lung, hematologic, and breast cancer occurring simultaneously with large plaque morphea have caused dermatologists to question whether a work-up for malignancy is appropriate. We highlight a case of generalized morphea that preceded invasive ductal carcinoma of the breast and provide a discussion about the possible paraneoplastic origin of generalized morphea and systemic sclerosis (SSc).

284. A Study of Morphea in Systemic Sclerosis: Defining Links Between Localized and Systemic Sclerosis

Background: Some patients with SSc develop skin features of localized scleroderma. One clinical subtype, keloid/nodular morphea, is particularly challenging to treat and may offer insight into key pathogenetic mechanisms. We have identified a cohort of SSc cases with concurrent localized scleroderma and examined their clinical and laboratory features and undertaken gene expression analysis to define the underlying pathogenic mechanism. Methods: We examined a clinical database of 2200 patients with SSc to identify all patients with a clinical or histological diagnosis of localized scleroderma and SSc overlap. The demographics, clinical features and serology of these patients were examined in detail. We undertook gene expression analysis of forearm and keloid/nodular morphea lesional skin biopsies from six patients by RT-PCR for matrix genes including COMP and thrombospondin-1, skin biopsies from patients with early or established diffuse cutaneous SSc provided a control group. Results: 49 patients (2.2%) had evidence of localized scleroderma and SSc. Demographic and clinical data from this group were broadly comparable to the SSc cohort, excepting higher prevalence in females (female: male ratio 11:1). Limited: diffuse cutaneous SSc ratio was 2:1 which is representative of this SSc cohort. Three subtypes of localized scleroderma were prevalent in this patient group: plaque morphea in 32 patients (67%); linear morphea of trunk and limbs in 4 patients (8.0%) and keloid/nodular morphea in 12 patients (25%). 28 out of 32 patients with plaque morphea and 3 out of 4 patients with linear morphea had limited cutaneous SSc, while 11 out of 12 subjects with keloid/nodular morphea had diffuse cutaneous SSc (P<0.001). There were no clear serological associations when correcting for subset. Black ethnicity, as could be expected, was prevalent in patients with keloid/nodular morphea (58% of keloid patients compared with 4% in our SSc cohort). Keloid nodules were localized on the upper chest, breast and upper limbs. Demographic, clinical and serological data are summarized in Table 1. Gene expression analysis defined key markers of matrix over expression compared with early stage or established SSc lesional skin. Conclusion: This is the largest analysis of localized scleroderma in SSc to be presented. There are particular associations of subtypes of localized scleroderma with subsets of SSc which are not related to serology. Paired gene expression of keloid and non-keloid SSc skin will contribute to an understanding of pathogenesis of this rare but important complication. Disclosure statement: The authors have declared no conflicts of interest.

Circumscribed morphea and breast asymmetry in an adolescent.

Morphea is a rare fibrosing disorder of the skin and underlying tissues. Circumscribed morphea presents with less than three discrete indurated plaques and breasts are commonly affected in women. We report the case of a 12-year-old female with a right infra-areolar, nontender, brownish patch and asymmetry of the right breast with 2 years of evolution. Skin biopsy showing thickening of the dermal collagen bundles confirmed the clinical diagnosis of morphea. After a 3-year follow-up period without progression of disease, reconstructive surgery is scheduled. Plaque morphea can involve all layers of the skin but associated breast deformity is rare. It can mimic benign and malignant breast disorders justifying the benefit for early tissue biopsy. Breast morphea generally has a good prognosis but hyperpigmentation and breast deformity in young girls have been rarely described. An early diagnosis can possibly lead to a therapeutic intervention with a different outcome, as it can be the source of severe psychological and social issues in a delicate period of development such as adolescence.

Plaque morphea with neurological involvement—an extraordinary uncommon presentation

Localized scleroderma is traditionally considered to be limited to the skin, subcutaneous tissue, underlying bone, and in the craniofacial subtype, also nervous system involvement. However, recent studies have also described other systemic manifestations in these patients. Despite many reports of neurological involvement in patients with the craniofacial linear localized scleroderma, it is extremely rare in patients with the other subtypes of localized scleroderma. Here, we report an extraordinary case of localized scleroderma en plaque (classic morphea), located to the upper trunk and neck, associated with neurological manifestations presented as seizures. Magnetic resonance imaging of the brain showed focal lesions on the contralateral side to the skin involvement. This case is extremely relevant not only due to its rarity, but also because it supports the idea that the pathogenesis of the localized scleroderma is related to a systemic autoimmune process.