Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0039483 (giant cell arteritis)
 3,204 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Giant cell arteritis is a systemic disease of the elderly which affects large and medium-sized arteries and which may occur in association with polymyalgia rheumatica. Scalp necrosis is a rare cutaneous complication of giant cell arteritis, and this dermatological presentation is not commonly reported.1-4 Giant cell arteritis should be considered in all elderly patients with scalp ulceration. We describe two patients who presented in this manner.
Clin Exp Dermatol 2003 Sep
PMID:Scalp necrosis in temporal arteritis. 1295 Mar 33

Hypohidrotic ectodermal dysplasia (HED) is a very rare disease characterized by the virtual absence of eccrine glands, dry skin, scanty hair, and dental abnormalities. It is transmitted by an X-linked recessive gene or rarely an autosomal recessive gene. Therefore it is only males who fully express the condition. It is caused by mutations within the ED1 gene, which encodes a protein, ectodysplasin-A (EDA). Typically there is frontal bossing, saddle nose, pointed chin, a prominent supraorbital ridge with periorbital hyperpigmentation, and absence of teeth. Those affected show great intolerance to heat. In the current absence of effective treatment for many hereditary skin diseases, comprehensive, accurate prenatal or postnatal genetic counseling can provide information to parents at risk of having affected children. We report HED in a 6-year-old boy with an Ala349Thr (GCA --> ACA) missense mutation developed de novo. Both parents and a 16-week gestational age fetus were healthy. We thought direct sequencing analysis for the ED1 gene using peripheral blood or amniotic fluid was preferable for an accurate diagnosis of this disease, although there was some risk of not detecting the mutation. After the results of this study were communicated to the parents, the mother was freed of her guilty feelings of the past 6 years and has now delivered a healthy male infant.
Pediatr Dermatol
PMID:Mutation in the ED1 gene, Ala349Thr, in a Korean patient with X-linked hypohidrotic ectodermal dysplasia developing de novo. 1546 65

Pyoderma gangrenosum is a chronic, noninfectious, ulcerating cutaneous disease of unknown etiology usually presenting with multiple lesions on the legs and trunk. Solitary lesions in other locations are rare and therefore hard to diagnose. A 78-year-old and a 81-year-old woman with extensive scalp necrosis were both diagnosed as pyoderma gangrenosum after exclusion of other differential diagnostic possibilities. The separate diagnosis of malignant pyoderma for this disease state no longer seems justified. Scalp necrosis is infrequently seen in dermatological patients; most previous reports describe it in giant cell arteritis. Other differential diagnoses include viral, bacterial and mycotic infections as well as erosive pustular dermatosis of the scalp. In conclusion, we again draw attention to unilesional pyoderma gangrenosum of the scalp as a rare disease entity. Immunosuppressive drugs are an effective treatment modality.
J Dtsch Dermatol Ges 2005 Feb
PMID:[Ulceration of the scalp: a unique manifestation of pyoderma gangrenosum]. 1635 Oct 14

Kaposi sarcoma usually occurs in immunosuppressed patients. A classic type has been reported in elderly men of Jewish and Mediterranean origin. We report a case of an elderly woman with giant cell arteritis (GCA) who developed Kaposi sarcoma while on a double blind trial for GCA with an anti-tumor-necrosis-factor medication. Our patient had none of the risk factors for Kaposi sarcoma, and when she was withdrawn from the study it was found that she was receiving only placebo along with the moderate, tapering doses of corticosteroids.
Dermatol Online J 2006 Oct 31
PMID:Kaposi sarcoma in a patient with giant cell arteritis. 1708 96

Churg-Strauss Syndrome (CSS) is a relatively rare entity characterized by asthma, transient pulmonary infiltrates, eosinophilia and systemic vasculitis. Oral ulceration is a possible clinical manifestation of some systemic vasculitides, such as Wegener's granulomatosis (WG) or giant cell arteritis, but has never been reported with Churg-Strauss syndrome. We report the first observation of a palatine ulceration in a 15-year-old girl with Churg-Strauss syndrome.
J Eur Acad Dermatol Venereol 2007 Aug
PMID:Oral ulceration: an unusual manifestation of Churg-Strauss syndrome. 1765 8

Hypereosinophilic syndrome (HES) is a multisystem disease with a high mortality rate. It is characterized by peripheral blood eosinophilia and eosinophilic infiltration of the skin and many other organs. The commonest cutaneous features include erythematous pruritic maculopapules and nodules, angio-oedema or urticarial plaques. However, some case reports have indicated that eosinophilic cellulitis, cutaneous necrotizing eosinophilic vasculitis, Raynaud's phenomenon and digital gangrene may also occur as cutaneous features of HES. Juvenile temporal arteritis (JTA) of unknown cause is characterized by an asymptomatic nodule in the temporal artery area in young adults. Histologically, the lesion is characterized by a significant intimal thickening with moderate eosinophilic infiltrates, constriction or occlusion of the vascular lumen and absence of giant cells. We report a patient with HES presenting with eosinophilic cellulitis, Raynaud's phenomenon, digital gangrene and JTA. JTA may also be one of the features of HES.
Clin Exp Dermatol 2009 Jul
PMID:Hypereosinophilic syndrome with various skin lesions and juvenile temporal arteritis. 1907 87

Vasculitis comprises a broad group of syndromes characterized by inflammation and necrosis on the walls of blood vessels, resulting in narrowing or occlusion of the lumen. The distribution of involved blood vessel varies considerably and serves as the basis for one classification of vasculitic syndromes: large vessels (Takayasu arteritis, giant cell arteritis); medium and small muscular arteries (polyarteritis nodosa, Churg-Strauss syndrome, Wegener's granulomatosis, vasculitis in rheumatic diseases); and small vessels ("hypersensitivity" vasculitis, Henoch-Schonlein purpura, microscopic polyangiitis, cryoglobulinemia). This article reviews medium and large vessel vasculitis and current treatment options; it also presents a comprehensive approach to diagnosing and treating patients with suspected cutaneous vasculitis.
An Bras Dermatol
PMID:[Medium and large vessel vasculitis]. 1937 60

Giant cell arteritis (GCA) is a systemic vasculitis associated with severe complications such as loss of vision and, rarely, scalp necrosis. We present a patient with GCA who had bilateral scalp necrosis and an erythrocyte sedimentation rate of only 21 mm after the first hour. Therapy with systemic steroids, which were slowly tapered over 1 year, led to secondary wound healing without recurrence. As there are no systematic reviews on the occurrence of scalp necrosis in patients with GCA, we performed a literature research and meta-analysis and discovered 78 cases published between 1946 and 2007. Analysis of the data revealed that GCA with scalp necrosis is associated with a higher incidence of vision loss (32%) and other visual defects (37.3%) than GCA without scalp necrosis (visual disturbances in up to 20%). GCA with scalp necrosis is also associated with an increased mortality (standard mortality ratio [SMR], 4.2) in contrast to GCA without scalp necrosis, which has no significantly higher mortality than age-matched controls (SMR 0.8-1.034). In patients with scalp necrosis, the diagnosis of GCA was made about 1 month later than in patients without scalp necrosis, and scalp necrosis was never reported to occur after onset of therapy with glucocorticoids. Thus, for reasons beyond potential loss of vision, physicians should be alert for symptoms of GCA as only timely diagnosis and immediate therapy may prevent serious complications and increased mortality.
J Am Acad Dermatol 2009 Oct
PMID:Scalp necrosis in giant cell arteritis: case report and review of the relevance of this cutaneous sign of large-vessel vasculitis. 2063 4

Juvenile temporal arteritis (JTA) is a nongranulomatous inflammation of the temporal artery with fragmentation of the internal elastic lamina and no concurrent systemic manifestations. It is a rare clinicopathologic entity with fewer than 20 reported cases, most of which represent localized disease with no recurrence or systemic symptoms at follow-up of up to 2 years. Histopathologic features can include lymphoeosinophilic infiltrate and endothelial proliferation. As the histology may resemble angiolymphoid hyperplasia with eosinophilia or Kimura disease, whether JTA is a discrete localized disease or a manifestation of these systemic conditions has been debated. We present a case of a 36-year-old Jamaican woman with a painful forehead nodule that showed histologic features of JTA, including intimal hyperplasia, lymphoeosinophilic inflammation of the vessel wall, and disruption of the internal elastic lamina; distinctive signet ringlike cytomorphologic alterations of the endothelial cells were noted as well. The lesion also showed extensive subcutaneous lymphoeosinophilic infiltrates and neovascularization with extension into the underlying muscle consistent with angiolymphoid hyperplasia with eosinophilia or Kimura disease. As the connection between JTA and angiolymphoid hyperplasia with eosinophilia and Kimura disease is currently debated and most reported cases of JTA have had only brief follow-up, the long-term sequelae of JTA are not known and careful patient monitoring may be necessary.
J Am Acad Dermatol 2010 Feb
PMID:Juvenile temporal arteritis. 1974 1

Hair loss in elderly women has been becoming a major topic in the daily practice of dermatology. Aging of hair follicles seems to affect hair growth and pigmentation, the molecular mechanisms of which remain to be elucidated. Further senile changes in physiology and immunity may influence the onset and course of hair diseases. Some preexisting diseases such as androgenetic alopecia usually worsen after menopause, while others, like discoid lupus erythematosus, may attenuate. Hormone surveying, especially with regard to internal androgen-producing tumors, is indicated in postmenopausal women with androgenetic alopecia of sudden exacerbation or with unusual manifestation or other virilizing signs. The prevalence of alopecia totalis and alopecia universalis appears to be much lower in postmenopausal ages as compared to earlier onset. Acute or chronic telogen effluvium is not uncommonly superimposed on androgenetic alopecia. Trichotillomania shows a marked female predominance in the senile age group with a higher rate of psychopathology. Worldwide, tinea capitis has been increasingly observed in postmenopausal women. Frontal fibrosing alopecia, giant cell arteritis and erosive pustular dermatosis involve mainly elder women leading to scarring alopecia. Alopecia induced by tumor metastasis to the scalp must be considered in women with underlying neoplasms, especially breast cancer. Overall, hair loss in postmenopausal women is often multifactorial and warrants a close inspection.
Eur J Dermatol
PMID:Hair loss in elderly women. 2017 41


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