Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0851184 (thinning)
 11,252 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Morphea, or localized scleroderma, of the eyelids is an uncommon disease. Morphea usually involves the thorax, trunk, lower and upper extremities, face, and genitalia. In the present report a patient with a biopsy-proven morphea of both upper eyelids is described. The salient histopathologic features included thinning of the epidermis with thickening and sclerosis of the collagen fibers in both the papillary and reticular dermis. There was a marked decrease in the fibrocytes. The eccrine sweat glands were entrapped by sclerotic collagen fibers. The pilosebaceous units were markedly decreased in number. There was a moderate lymphocytic infiltration in the dermis and a prominent lymphocytic perivasculitis. The clinical and histopathologic features of morphea are compared with those of lichen sclerosus et atrophicus.
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PMID:Morphea of the eyelids. 715 36

The term "morphea" 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-year-old 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-colored centers and erythematous borders ("lilac ring") localized on the neck, chest, and lower abdomen and limbs (Figure 1). They were bound to the deeper structures and arranged in a band-like 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 2). Laboratory investigations provided normal range of erythrocyte sedimentation rat and C reactive protein levels and other inflammation markers. Antinuclear antibody, antidouble-strand DNA, antimitochondrial, anti-extractable antigens (anti-centromere, anti-Scl-70, anti-U1RNP), and anti-Borrelia burgdorferi antibodies were negative. Circulating immunocomplexes binding C1q were substantially increased. Oesophageal x-rays 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-capillaries, segmentary dilatation and destruction), the laser-Doppler 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-monocytic and plasmacellular infiltration with a dermo-epidermal 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-up, the patient had obtained only moderate improvement of the clinical findings.
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PMID:Case study: periodic follow-up is necessary in morphea profunda to identify systemic evolution. 1589 Dec 59

Solitary morphea profunda (SMP) is a distinct form of localized scleroderma, a cutaneous disorder of unknown origin. Here, we describe a patient presenting with noninflammatory cupuliform depressed plaques, without any significant skin induration, pigmentation or texture change, that appeared on the left shoulder at a site of previous intramuscular vaccination. Light microscopy studies revealed excessive dermal collagen deposition with thickened hyalinized collagen bundles. Magnetic resonance imaging studies demonstrated tissue fibrosis with thinning of the subcutaneous fat tissue and involvement of the adjacent deltoid muscle, which was confirmed by electromyographic tests. Borrelia serology was negative. Our observation of SMP differed from previously described cases, since it mimicked localized lipoatrophy. Our observation highlights the wide spectrum of clinical presentations of localized scleroderma. The latter should be considered in the presence of lipoatrophy-like lesions for proper workup and therapy.
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PMID:Primary atrophic solitary morphea profunda. 1859 75

Linear scleroderma is a rare variant of localised scleroderma, which is usually seen in childhood and during the adolescent period, and can cause severe functional morbidity as well as cosmetic and psychological problems. Although its ethiopathogenesis is yet obscure, autoimmunity, local ischaemia and injuries, vaccination, irradiation, vitamin K injections, Borrelia burgdorferi and Varicella infections have been incriminated. A 4-year-old girl who had been followed up for about 18 months with diagnosis of epilepsy had a colour discolouration and depression that first appeared 1 year ago and then progressed on her left frontal region. Her CT scan showed a thinning in the frontal bone and depression in the frontal region. These findings are described as 'en coup de sabre' a rare form of linear scleroderma localised at the frontal region of the scalp. In this paper, we present clinical and radiological findings of a 4-year-old girl with epileptic seizures that started 1 year before the onset of the lesion of linear scleroderma.
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PMID:Frontal linear scleroderma en coup de sabre associated with epileptic seizure. 2323 Feb 61