{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,9,27]],"date-time":"2025-09-27T13:20:41Z","timestamp":1758979241781},"reference-count":12,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2007,6,21]],"date-time":"2007-06-21T00:00:00Z","timestamp":1182384000000},"content-version":"vor","delay-in-days":781,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Skinmed"],"published-print":{"date-parts":[[2005,5]]},"abstract":"\nThe term \u201cmorphea\u201d includes a wide spectrum of clinical entities, varying from localized plaques of only cosmetic importance to deep lesions resulting in considerable morbidity for the patient. In fact, although survival rates are no different from that of the general population, localized scleroderma May be associated with development of substantial disability, as occurs in deep morphea and in pediatric patients (disabling pansclerotic morphea of children). We report a case of morphea profunda affecting a young man with severe, rapidly progressive, widespread skin involvement and focus on the eventual systemic evolution of such cases. A 40\u2010year\u2010old man was admitted in 2002 for progressive subcutaneous indurations, preferentially involving the right side of the trunk. His health was altogether good, with the exception of a beginning chronic obstructive bronchopneumopathy. There was no family or personal history of dysmetabolic, cardiovascular, neoplastic, or cutaneous disease. Three years earlier, the patient had noted the appearance of two infiltrated, intensely red lesions on the right laterocervical and paraumbilical regions. These had been interpreted as subcutaneous lipomatosis on the basis of an ultrasound scan. The lesions had become progressively larger, while their surface had assumed a scleroatrophic appearance. Thereafter, other lesions had developed on his chest and lower limbs, mostly distributed on the right side of the body. Clinical examination revealed well demarcated, depressed sclerotic plaques with ivory\u2010colored centers and erythematous borders (\u201clilac ring\u201d) localized on the neck, chest, and lower abdomen and limbs (Figure\u20031<\/jats:ext-link>). They were bound to the deeper structures and arranged in a band\u2010like linear distribution on the right side of the chest and abdomen where they extended horizontally for more than 10 cm in diameter. These lesions were totally asymptomatic. In addition, arborizing telangiectasias were evident on the neck and upper chest (Figure\u20032<\/jats:ext-link>). Laboratory investigations provided normal range of erythrocyte sedimentation rat and C reactive protein levels and other inflammation markers. Antinuclear antibody, antidouble\u2010strand DNA, antimitochondrial, anti\u2010extractable antigens (anti centromere, anti\u2010Scl\u201070, anti\u2010U1RNP), and anti\u2010Borrelia burgdorferi antibodies were negative. Circulating immunocomplexes binding C1q were substantially increased. Oesophageal x\u2010rays and lower limb electromyography were within normal limits; ventilatory function testing revealed a mild obstruction consistent with the beginning of chronic obstructive pulmonary disease. Although nailfold capillaroscopy documented nonspecific findings of connective tissue disease (mega\u2010capillaries, segmentary dilatation and destruction), the laser\u2010Doppler flussimetry revealed few signs of microcirculatory abnormalities, in absence of Raynaud's phenomenon. An abdominal wall ultrasonography, performed on a sclerotic plaque, documented thinning of the subcutaneous tissue, with increase of the fibrous component and lower fascia and muscle retraction. The biopsy specimen from the abdominal region included fascia and the subcutaneous tissue (previously obtained from the lower abdomen) with epidermal atrophy, a thickening and homogenization of collagen bundles in the deep dermis and hair reduction. A perivascular lympho\u2010monocytic and plasmacellular infiltration with a dermo\u2010epidermal distribution was present. Moreover, septal fibrosis with a perivascular lymphoplasmacellular inflammatory infiltrate was documented within the abdominal rectus muscle. The diagnosis of morphea profunda was made on the basis of clinical and histopathological findings. A therapeutic regimen based on amino benzoic potassium (Potaba; Glenwood, LLC, Glenwood, NJ), oral prednisone, and topical clobetasol was started. After several months of follow\u2010up, the patient had obtained only moderate improvement of the clinical findings.<\/jats:p><\/jats:disp-quote>\n<\/jats:p> Depressed sclerotic lesion with ivory\u2010colored center and erythematous border in the paraumbilical region<\/jats:italic> <\/jats:p><\/jats:caption>image<\/jats:alt-text><\/jats:graphic><\/jats:fig><\/jats:p> Detail of <\/jats:italic>Figure\u20031<\/jats:italic><\/jats:ext-link> <\/jats:p><\/jats:caption>image<\/jats:alt-text><\/jats:graphic><\/jats:fig><\/jats:p>","DOI":"10.1111\/j.1540-9740.2005.03592.x","type":"journal-article","created":{"date-parts":[[2007,6,21]],"date-time":"2007-06-21T09:59:44Z","timestamp":1182419984000},"page":"188-190","source":"Crossref","is-referenced-by-count":6,"title":["Periodic Follow\u2010Up Is Necessary in Morphea Profunda to Identify Systemic Evolution"],"prefix":"10.1111","volume":"4","author":[{"given":"Lucilla","family":"Melani","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Carla","family":"Cardinali","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Barbara","family":"Giomi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Emiliano","family":"Schincaglia","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marzia","family":"Caproni","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paolo","family":"Fabbri","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2007,6,21]]},"reference":[{"key":"e_1_2_2_2_2","first-page":"2023","volume-title":"Fitzpatrick's Dermatology in General Medicine","author":"Tu JH","year":"1999"},{"key":"e_1_2_2_3_2","doi-asserted-by":"publisher","DOI":"10.1097\/00002281-199609000-00014"},{"key":"e_1_2_2_4_2","first-page":"785","volume-title":"Dermatology","author":"Braun\u2010Falco O","year":"2002"},{"key":"e_1_2_2_5_2","first-page":"898","article-title":"Presence of the antinuclear antibodies and antibodies to Borrelia burgdorferi among patients with morphea en plaque, deep linear scleroder\u2010ma and atrophoderma Pasini\u2010Pierini","volume":"59","author":"Wojas\u2010Pelc A","year":"2002","journal-title":"Przegl Lek"},{"key":"e_1_2_2_6_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-0560.2007.00948.x"},{"key":"e_1_2_2_7_2","first-page":"251","article-title":"Morphea profunda. A new concept and histopathological study of 23 cases","volume":"3","author":"Su WP","year":"1981","journal-title":"Am J Dermatopathol"},{"key":"e_1_2_2_8_2","first-page":"283","article-title":"Morphe\u00e9s: classification and management","volume":"125","author":"Salmon\u2010Ehr V","year":"1998","journal-title":"Ann Dermatol Venereol"},{"key":"e_1_2_2_9_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-4362.1991.tb04356.x"},{"key":"e_1_2_2_10_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2133.1989.tb04171.x"},{"key":"e_1_2_2_11_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1525-1470.1991.tb00936.x"},{"key":"e_1_2_2_12_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2230.2002.10493.x"},{"key":"e_1_2_2_13_2","doi-asserted-by":"publisher","DOI":"10.1053\/sder.2001.23093"}],"container-title":["SKINmed: Dermatology for the Clinician"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fj.1540-9740.2005.03592.x","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/j.1540-9740.2005.03592.x","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/j.1540-9740.2005.03592.x","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/j.1540-9740.2005.03592.x","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,11]],"date-time":"2023-09-11T03:48:10Z","timestamp":1694404090000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/j.1540-9740.2005.03592.x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2005,5]]},"references-count":12,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2005,5]]}},"alternative-id":["10.1111\/j.1540-9740.2005.03592.x"],"URL":"https:\/\/doi.org\/10.1111\/j.1540-9740.2005.03592.x","archive":["Portico"],"relation":{},"ISSN":["1540-9740","1751-7125"],"issn-type":[{"value":"1540-9740","type":"print"},{"value":"1751-7125","type":"electronic"}],"subject":[],"published":{"date-parts":[[2005,5]]}}}