A case of guttate morphea devoid of plaque morphea

( Dongyoung Roh ),( Hyunju Jin ),( Hyang Suk You ),( Woo Haing Shim ),( Jeong Min Kim ),( Gun Wook Kim ),( Hoon Soo Kim ),( Hyun Chang Ko ),( Byung Soo Kim ),( Moon Bum Kim ) 대한피부과학회 2016 대한피부과학회 학술발표대회집 Vol.68 No.1

Morphea is a rare inflammatory disorder of the skin characterized by sclerotic change in deep dermis. It is clinically divided into several subtypes according to morphology, number, or extent of lesions. Among them, guttate morphea is an extremely rare subtype of morphea and tends to be less infiltrated on palpation and to appear at an earlier age. It presents small, ‘drop like’ and atrophic skin lesions resembling those of lichen sclerosus et atrophicus. A 27-year-old woman presented with multiple whitish atrophic macules on both arms and thighs for 4 months. Compared to the normal skin, the histopathologic examination showed increased thick collagen bundles in lower dermis, minimal perivascular inflammatory infiltrations, and a reduced number of appendages. Changes in the amount of pigmentation and the number of melanocytes were not prominent. We diagnosed this patient as guttate morphea based on clinical manifestations and histopathology. Guttate morphea tends to be localized and usually occurs in combination with larger plaque-type lesions. To our knowledge, guttate morphea have been rarely reported in the literature and most of these have been associated with plaque morphea. We encountered a case of guttate morphea which presented without association with plaque morphea.

P047 Clinical and histopathologic study of morphea and extragenital lichen sclerosus et atrophicus

( Wooseok Jeong ),( Woojung Jin ),( Seunghyun Moon ),( Taekgeun Lee ),( Taegwang Kwon ),( Sookkyung Lee ) 대한피부과학회 2016 대한피부과학회 학술발표대회집 Vol.68 No.2

<div style="display:none">fiogf49gjkf0d</div><div style="display:none">fiogf49gjkf0d</div><div style="display:none">fiogf49gjkf0d</div><div style="display:none">fiogf49gjkf0d</div><div style="display:none">fiogf49gjkf0d</div><div style="display:none">fiogf49gjkf0d</div><div style="display:none">fiogf49gjkf0d</div> Background: Differential diagnosis of morphea and extragenital lichen sclerosus et atrophicus(LSA) is occasionally difficult. There have been very few cases reported describing coexistence of morphea and LSA. Objectives: To evaluate the clinical and histopathologic characteristics that differentiate morphea and extragenital LSA. Methods: We have reviewed the medical records, clinical and dermoscopic photographs, and histopathologic slides of 23 patients of morphea and 4 patients of extragenital LSA diagnosed at our clinic between 2006 and 2016. Results: Dermoscopic feature of comedo-like openings was significantly increased in the patients with extragenital LSA compared with morphea. Histopathologic features of hyperkeratosis, epidermal atrophy and homogenized collagen in the upper dermis were significantly increased in the patients with extragenital LSA compared with morphea. There were no statistically significant differences according to the clinical features and autoimmune antibody studies. 2 patients showed overlapping features of both morphea and LSA in the same lesion. Conclusion: Dermoscopy can be useful and there are distinctive histopathologic findings to differentiate the two diseases. Although their typical forms are distint, overlapping features of two lesions appear to represent different presentations along the same disease spectrum. Further studies on a larger group would be needed to confirm and clarify such findings.

증 례 : 선국소피부경화증에서 진행하여 경화 태선을 동반한 전신국소피부경화증

권형일 ( Hyoung Il Kwon ),홍정호 ( Jeong Ho Hong ),김정은 ( Jeong Eun Kim ),고주연 ( Joo Yeon Ko ),노영석 ( Young Suck Ro ) 대한피부과학회 2015 대한피부과학회지 Vol.53 No.9

Morphea is an autoimmune condition characterized by excessive accumulation of collagen in the skin and underlying tissues. Lichen sclerosus (LS) is another connective tissue disease for which an autoimmune cause has been proposed, given the high association with other autoimmune diseases. The coexistence of morphea and lichen sclerosus has been sometimes reported in the literature, and is suggestive of a common pathogenic background between the two diseases. Among various types of morphea, generalized morphea has been associated with an increased rate of autoimmune disease, including LS. We report a case of extragenital LS during the progression of linear morphea into generalized morphea. (Korean J Dermatol 2015;53(9):713∼716)

CD34 stromal expression is inversely proportional to the extent of morphea

( Ji Su Lee ),( Hyun-sun Park ),( Hyun-sun Yoon ),( Soyun Cho ) 대한피부과학회 2017 대한피부과학회 학술발표대회집 Vol.69 No.2

Background: Fibrosis is thought to be the main pathophysiology of scleroderma, and myofibroblasts play a main role in abnormal fibrotic pathologies. Altered distribution of dermal dendrocytes, particularly loss of CD34+ fibrocytes, has been reported in scleroderma. Objective: To investigate the relative expression and distribution of myofibroblast and CD34+ fibrocytes in morphea, we performed immunohistochemical stains using several relevant antibodies. Methods: Skin lesions of 50 patients with morphea and age-, sex- and site-matched normal skin of 50 subjects were evaluated for the following antibodies: SMA, CD34, CD31, and VCAM-1. Results: The patient group showed significantly higher expression of SMA and VCAM-1 stain than the control group (p=0.000 and p=0.027, respectively), while CD34 stromal stain was significantly decreased in the patient group (p=0.000). Within the patient group, CD34 stromal stain had an inverse correlation with SMA and VCAM-1 stains (r=-0.286, p=0.044, and r=-0.296, p=0.037, respectively). In subtype analysis, CD34 stromal expression was lost more in generalized morphea than non-generalized morphea. Limitations: Retrospective nature of the study. Conclusion: Mutually exclusive staining patterns of CD34 stromal and SMA stains suggest a phenotypic change of CD34+ fibrocytes into myofibroblasts with progression of disease in morphea. CD34 may be a useful marker in predicting the extent of morphea.

A case of morphea coexisting with extragenital lichen sclerosus et atrophicus

( Yu Jin Lee ),( Yeon Seok Lee ),( Young Shin Kim ),( Tae Young Han ),( Jae Eun Choi ),( June Hyunkyung Lee ) 대한피부과학회 2021 대한피부과학회 학술발표대회집 Vol.72 No.2

Morphea is a chronic inflammatory disease characterized by hypopigmented sclerotic plaques, which becomes softened and atrophic. Extragenital lichen sclerosus et atrophicus (LSEA) is an inflammatory dermatosis that presents with porcelain-white plaques. Coexistence of morphea and LESA has been occasionally identified in literatures. A 26-year-old woman presented with asymptomatic multiple hyper- and hypopigmented atrophic patches with focal porcelain-white sclerotic lesions on trunk and right axilla since childhood. She denied any traumatic history. Routine laboratory tests were normal except for speckled patterned anti-nuclear antibody (ANA). Histologically, perivascular inflammatory infiltrate of lymphocytes and plasma cells, and swollen collagen bundles were observed, which suggest morphea. In addition, thinned epidermis and subepidermal cleavage with interface change, and a wide band of hyalinization at the dermo-epidermal junction were also observed, which suggest LSEA. According to clinicopathological findings, she was diagnosed with morphea coexisting with extragenital LSEA. The coexistence of both conditions may suggest that these lesions share a spectrum of common pathologic process. While the cause of either disease is unknown, several factors such as Borrelia infection, autoimmunity, and trauma have been considered as etiologies. Herein, we report an uncommon case of a patient with morphea coexisting with extragenital LSEA.

범발성 반상 경피증에서 발생한 편평세포암 치험례 : 증례보고

박대환,장경수 大韓成形外科學會 1995 Archives of Plastic Surgery Vol.22 No.2

Generalized morphea, a generalized form of morphea, is a slowly progressive chronic disease characterized by the fibrosis with sclerosis of the skin. Squamous cell carcinoma arising from the chronic ulcerative lesion of the sclerotic morphea is very rare. Only a few cases were reported in literatures but they were aggressive in nature and had a high frequency of metastasis. The authors have a patient, a twenty-seven year old male, who had been suffering from generalized morphea with scar-like changes and successive involvement of the ankle, limb folds, abdomen and no tendency to heal from the age four. He had a severe sclerotic form of morphea over his entire body along with severe joint contracture, and was diagnosed as pansclerotic generalized morphea. A squamous cell carcinoma, the approximate size of an adult fist (7×6×4cm), with a bleeding tendency, developed from a chronic ulcerative lesion on the posterior aspect of his right ankle at the age of twenty-seven. A biopsy obtained from the tumor revealed a histological typical findings of squamous cell carcinoma grade Ⅱ-Ⅲ. We strongly recommended amputation of the involved lower extremity, but he refused. A wide excision, about 1 cm from the margin of the squamous cell carcinoma, was performed along with a skin graft. A high malignant metastatic lesion occured on the right popliteal fossa three months after first operation. We recommended amputation of the involved lower extremity again, but he refused and died one month later.

A case of generalized morphea misdiagnosed as vitiligo

( Howard Chu ),( Jae Won Lee ),( Do Young Kim ) 대한피부과학회 2015 대한피부과학회 학술발표대회집 Vol.67 No.1

Morphea, or localized scleroderma, is a chronic autoimmune disease characterized by sclerosis of the skin and subcutaneous tissue. Its clinical findings are diverse, resulting in frequent misdiagnosis. We report a case of patient with generalized morphea who was misdiagnosed and treated under the impression of vitiligo at a local oriental clinic. A 29-year-old male presented with multiple hypopigmented and hyperpigmented sclerotic plaques surrounded by violaceous erythematous borders on his whole body, which began 2 years prior to his visit to our clinic. He had been treated at a local clinic under the impression of vitiligo, and was referred to us when no improvement was observed. Biopsy was done, which revealed thickened and sclerotic collagen bundles in the reticular dermis, with direct immunofluorescence study showing weak linear positivity of IgM in dermoepidermal junction. The patient showed no symptoms of sclerodactyly or Raynaud phenomenon, leading to the diagnosis of generalized morphea. Treatment was started with methotrexate and steroid pulse therapy, but due to the delay, sclerosis has progressed, with signs of contracture on his right ankle. The cutaneous manifestation of morphea is diverse, often leading to misdiagnosis. This delay would likely to result in aesthetic and functional complications, in which the treatment is apparently challenging. Earlier diagnosis would help the patients in preventing such complications.

A case of generalized morphea

( Ho Jung Lee ),( Seung Min Lee ),( Sul Hee Lee ),( Young Lip Park ),( Kyu Uang Whang ),( Sung Yul Lee ),( Jung Eun Kim ) 대한피부과학회 2018 대한피부과학회 학술발표대회집 Vol.70 No.1

Morphea, or localized scleroderma, is a chronic autoimmune disease characterized by sclerotic plaques of the skin and subcutaneous tissue. It comprises a number of subtypes due to its clinical diversity. Generalized morphea is a rare subtype of localized scleroderma which occurs in a widespread manner but lacks systemic manifestations. We report a-46-year-old Korean male who presented with multiple hypopigmented and hyperpigmented sclerotic plaques on whole body 3 months ago. The lesions were located on back, abdomen, both lower arms, both axillae, both legs. The lesion was surgically incised under local anesthesia. Histopathological examination with a biopsy specimen from a patch of Right arm showed the characteristic features of morphea, including “square” appearance, interstitial and perivascular inflammatory cell infiltration and collagen bundle swelling and thickening. The patient responded to treatment with topical tacrolimus 0.1%. Topical tacrolimus acts specifically on inflammatory cells, and has anti-inflammatory and immunomodulatory effects that inhibit the synthesis of collagen fibers, thus exerting an effect on morphea. We report a rare case of idiopathic generalized scleroderma treated with topical tacrolimus.

A case of morphea occurring on a scar after laparoscopy

( You Jin Lee ),( Youngkyoung Lim ),( Jae Ho Lee ),( Jong Yoon Chung ),( Ji Hye Park ),( Jong Hee Lee ),( Dong Youn Lee ),( Joo Heung Lee ),( Jun Mo Yang ) 대한피부과학회 2015 대한피부과학회 학술발표대회집 Vol.67 No.1

Morphea (localized scleroderma) is a chronic autoimmune disease characterized by local collagen accumulation in the skin. Several studies suggested that disease begins with unidentified antigens interacting with the host immune system. Recently, Trauma is reported to be a suspected trigger of morphea. Herein, we report a case of morphea occurred on a scar after laparoscopic nephrectomy

방사선 치료로 인한 국소 피부경화증 1예

박상훈 ( Sang Hoon Park ),강유석 ( Yoo Seok Kang ),김성우 ( Sung Woo Kim ),박현수 ( Hyun Su Park ),이운하 ( Un Ha Lee ),장상재 ( Sang Jai Jang ) 대한피부과학회 2009 大韓皮膚科學會誌 Vol.47 No.6

A 58-year-old female with breast cancer, who had undergone breast conserving surgery and radiotherapy, presented with a 7-month history of erythematous to brown, indurated plaque confined to the previous radiation field. The patient had no constitutional symptoms. A skin biopsy showed thickened, hyalinized collagen bundles in the dermis, which was consistent with morphea. Postirradiation morphea is a rare complication of radiotherapy. The clinical feature of postirradiation morphea may be similar to cellulitis or cutaneous metastasis, and the diagnosis of this disease is given histopathologically. We present a case of postirradiation morphea and a review of the literature. (Korean J Dermatol 2009;47(6):752∼754)