Localized scleroderma: clinical spectrum and therapeutic update

Careta, Mariana Figueiroa; Romiti, Ricardo

doi:10.1590/abd1806-4841.20152890

Cited by 177 publications

(232 citation statements)

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“…The incidence of LoSc is 0.4-2.7 cases/100,000 persons/year, with a peak age of onset at 20-40 years. It is more common in the Caucasian population, and in women (female-to-male ratio -4 : 2) [1][2][3]. Children under 10 years of age represent approximately 15% of the patient population [4].…”

Section: Review Article/praca Poglądowamentioning

confidence: 99%

“…Szacuje się, że zapadalność na LoSc wynosi 0,4-2,7 przypadku/ 100 000 osób/rok, a szczyt zachorowań przypada między 20. a 40. rokiem życia. Choroba częściej wystepuje u osób rasy kaukaskiej i płci żeńskiej (kobiety : mężczyźni -4 : 2) [1][2][3]. Około 15% chorych stanowią dzieci do 10. roku życia [4].…”

Section: Wstępunclassified

“…The lesions are distributed symmetrically within the trunk, thighs and in the lumbosacral region [2,6], and easily distinguishable from the surrounding healthy skin (Figs. 4 A-D) [1,3]. The disease is characterized by rapid progression and needs to be differentiated from systemic sclerosis.…”

Section: Generalized Form Of Localized Sclerodermamentioning

confidence: 99%

“…Wykwity są dobrze odgraniczone od skóry zdrowej (ryc. 4 A-D) [1,3]. Choroba cechuje się szybką progresją i wymaga różnicowania z twardziną układową.…”

Section: Twardzina Ograniczona Uogólnionaunclassified

“…Choroba cechuje się szybką progresją i wymaga różnicowania z twardziną układową. W przeci- in the nail-bed capillaries, Raynaud phenomenon, ulceration and reabsorption of the phalanges, or facial skin involvement [1,3]. Muscle and joint pain, and fatigue may be present, and laboratory tests may be positive for ANA and eosinophilia [3,9].…”

Section: Twardzina Ograniczona Uogólnionaunclassified

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Localized scleroderma – classification and tools used for the evaluation of tissue damage and disease activity/severity

Wolska-Gawron¹,

Krasowska²

2017

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Localized scleroderma is a rare autoimmune disorder affecting the dermis, subcutaneous tissue and deeper structures. The course of localized scleroderma includes three stages: early inflammation, progressive sclerosis and atrophy. The active stage of the disease is characterized by erythema ('lilac ring'), skin induration, and appearance of new or expansion of pre-existing skin lesions during the preceding month. The most recent localized scleroderma classification recognizes five types of the disease: localized, generalized, linear, deep and mixed. Various methods have been proposed for the evaluation of disease activity or severity and tissue damage, including ultrasonography, thermography, durometry, computed tomography and magnetic resonance imaging. However, all of them have their limitations, as they only make it possible to assess one component of the disease: activity or severity or tissue damage. The LS Cutaneous Assessment Tool (LoSCAT) questionnaire is a promising method of patient evaluation because of its availability, repeatability of results and sensitivity to changes resulting from treatment. STRESZCZENIETwardzina ograniczona jest rzadką chorobą autoimmunologiczną, która przebiega z zajęciem skóry, tkanki podskórnej i tkanek głębiej położonych. Choroba klinicznie ma trzy fazy: wczesną zapalną, postępującego stwardnienia i zanikową (atroficzną). Wykładnikami aktywnego procesu chorobowego są: rumień (lilac ring), stwardnienie skóry, pojawianie się nowych wykwitów skórnych lub powiększenie wcześniej istniejących zmian w ciągu ostatniego miesiąca. Najnowsza klasyfikacja wyróżnia pięć typów twardziny ograniczonej: ograniczona, uogólniona, linijna, głęboka i mieszana. Zaproponowano wiele metod oceny aktywności lub nasilenia choroby oraz uszkodzenia tkanek, takich jak ultrasonografia, termografia, durometria, tomografia komputerowa czy rezonans magnetyczny. Ograniczenia powyż-szych badań wynikają z możliwości oceny tylko jednego wykładnika -aktywności lub nasilenia choroby albo uszkodzenia tkanek. Przedstawiona metoda ewaluacji pacjentów oparta na zastosowaniu kwestionariusza do oceny aktywności i nasilenia choroby (LoSCAT) jest godna uwagi ze względu na dostępność narzędzia, powtarzalność wyników, a także czułość formularza na zmiany wynikające z zastosowanego leczenia.

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Section: Review Article/praca Poglądowamentioning

confidence: 99%

Section: Wstępunclassified

Section: Generalized Form Of Localized Sclerodermamentioning

confidence: 99%

“…Wykwity są dobrze odgraniczone od skóry zdrowej (ryc. 4 A-D) [1,3]. Choroba cechuje się szybką progresją i wymaga różnicowania z twardziną układową.…”

Section: Twardzina Ograniczona Uogólnionaunclassified

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Localized scleroderma – classification and tools used for the evaluation of tissue damage and disease activity/severity

Wolska-Gawron¹,

Krasowska²

2017

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Corrugated Indurated Short Cords on the Neck of a Teenage Girl

Zhang

2021

JAMA Dermatol

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A teenage girl presented with a 1-year history of multiple indurated cords on the anterior neck area. The lesions appeared without an identifiable cause and spread around the neck. The cords eventually covered the entire anterior neck and upper and lower clavicle areas. The skin lesions formed a corrugated pattern at the time of presentation. The patient had previously been treated with moisturizers at a local clinic for 2 months without any signs of improvement. The patient denied any manipulation or trauma at those sites. There was no personal or family history of keloid formation. The girl was otherwise healthy and met normal developmental milestones.On examination of the patient, there were many short, skin-colored, parallel cords on the patient's anterior neck areas (Figure , A and B). These cords were indurated, slightly raised, and well defined with a shiny surface. The cords had a uniform width of approximately 2 mm, and the lengths varied from 3 mm to 2 cm. During the course of the examination, an ill-defined sclerotic plaque surrounding the underside of the clavicle was found. It had a shiny surface, irregular shape, and measured 10 × 4 cm. No similar lesions were found on any other part of the patient's body. Inspection of the patient's skin revealed no abnormal nail fold capillary dilation or hemorrhage; no sclerodactyly, distal digital pitting, or Raynaud disease; and no extracutaneous involvement. A punch biopsy specimen from the affected area was obtained (Figure, C and D). A Corrugated indurated short cords on neck B Closer view of corrugated indurated short cords on neck Original magnification ×40 C Original magnification ×200 D Figure. The patient has a sclerotic plaque surrounding the underside of the clavicle (A) and indurated, slightly raised, and well-defined cords with a shiny surface (B). Histopathologic findings include inflammatory infiltrate around blood vessels and closely spaced thick bundles of collagen (C) and hyalinosis of collagen (D) (hematoxylin-eosin). WHAT IS YOUR DIAGNOSIS? A. Pseudoxanthoma elasticum B. Morphea C. Hypertrophic scar or keloid D. Connective tissue nevus Quiz at jamacmelookup.com Clinical Review & Education

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Clinical, Histologic, and Transcriptomic Evaluation of Sequential Fat Grafting for Morphea

Liu,

Wang,

Zhang

et al. 2024

JAMA Dermatol

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ImportanceMorphea is a rare disease of unknown etiology without satisfactory treatment for skin sclerosis and soft tissue atrophy.ObjectiveTo provide clinical, histologic, and transcriptome evidence of the antisclerotic and regenerative effects of sequential fat grafting with fresh fat and cryopreserved stromal vascular fraction gel (SVF gel) for morphea.Design, Setting, and ParticipantsThis single-center, nonrandomized controlled trial was conducted between January 2022 and March 2023 in the Department of Plastic and Reconstructive Surgery of Nanfang Hospital, Southern Medical University and included adult participants with early-onset or late-onset morphea who presented with varying degrees of skin sclerosis and soft tissue defect.InterventionsGroup 1 received sequential grafting of fresh fat and cryopreserved SVF gel (at 1 and 2 months postoperation). Group 2 received single autologous fat grafting. All patients were included in a 12-month follow-up.Main Outcome and MeasuresThe primary outcome included changes in the modified Localized Scleroderma Skin Severity Index (mLoSSI) and Localized Scleroderma Skin Damage Index (LoSDI) scores as evaluated by 2 independent blinded dermatologists. The histologic and transcriptome changes of morphea skin lesions were also evaluated.ResultsOf 44 patients (median [IQR] age, 26 [23-33] years; 36 women [81.8%]) enrolled, 24 (54.5%) were assigned to group 1 and 20 (45.5%) to group 2. No serious adverse events were noted. The mean (SD) mLoSSI scores at 12 months showed a 1.6 (1.50) decrease in group 1 and 0.9 (1.46) in group 2 (P = .13), whereas the mean (SD) LoSDI scores at 12 months showed a 4.3 (1.34) decrease in group 1 and 2.1 (1.07) in group 2 (P &lt; .001), indicating that group 1 had more significant improvement in morphea skin damage but not disease activity compared with group 2. Histologic analysis showed improved skin regeneration and reduced skin sclerosis in group 1, whereas skin biopsy specimens of group 2 patients did not show significant change. Transcriptome analysis of skin biopsy specimens from group 1 patients suggested that tumor necrosis factor α signaling via NFκB might contribute to the immunosuppressive and antifibrotic effect of sequential fat grafting. A total of 15 hub genes were captured, among which many associated with morphea pathogenesis were downregulated and validated by immunohistochemistry, such as EDN1, PAI-1, and CTGF.Conclusions and RelevanceThe results of this nonrandomized trial suggest that sequential fat grafting with fresh fat and cryopreserved SVF gel was safe and its therapeutic effect was superior to that of single autologous fat grafting with improved mLoSSI and LoSDI scores. Histological and transcriptomic changes further support the effectiveness after treatment.Trial RegistrationChinese Clinical Trial Registry identifier: ChiCTR2200058003

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Localized scleroderma: clinical spectrum and therapeutic update

Cited by 177 publications

References 123 publications

Localized scleroderma – classification and tools used for the evaluation of tissue damage and disease activity/severity

Localized scleroderma – classification and tools used for the evaluation of tissue damage and disease activity/severity

Corrugated Indurated Short Cords on the Neck of a Teenage Girl

Clinical, Histologic, and Transcriptomic Evaluation of Sequential Fat Grafting for Morphea

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