Pathophysiology of urticarial vasculitis.
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Antigen-Antibody Complex↗
Biomedical subjects
L E Gibson
Publications and source records attributed to L E Gibson.
Contact hypersensitivity to tixocortol pivalate.
BACKGROUND: Tixocortol pivalate is an established marker to topical corticosteroid allergy. The prevalence of tixocortol pivalate hypersensitivity is well established in Europe, where exposure to this corticosteroid as a therapeutic agent varies. In the United States, tixocortol pivalate is not commercially available and the prevalence of hypersensitivity to it is unknown. OBJECTIVE: We investigated the prevalence of tixocortol pivalate hypersensitivity in our patch-tested population. We further characterized these patients by clinical background, other contact allergens, and the reactivity to other corticosteroids. METHODS: Tixocortol pivalate has been incorporated in our standard 1-52 patch test series since November 1992. We reviewed the histories and patch test results in all patients tested with the standard 1-52 series from November 1992 to December 1996. RESULTS: Of 1536 patch-tested patients, 45 had hypersensitivity to tixocortol pivalate. Dermatitis involving the face was the most common (14 patients). Of the 45 patients, 40 had another allergen identified on patch testing. Eighteen patients underwent further patch testing to an extended corticosteroid panel, and 14 had sensitivity to another steroid agent. CONCLUSION: The 2.9% prevalence of tixocortol pivalate hypersensitivity in our patch test population is within the range reported in Europe. Patients with tixocortol pivalate hypersensitivity tend to have other contact allergens on patch testing. Predisposing factors to tixocortol pivalate hypersensitivity include facial dermatitis and sensitivity to other contact allergens.
Administration, Topical↗
Bullous pemphigoid presenting as exfoliative erythroderma.
Several unusual clinical presentations of bullous pemphigoid have been described such as localized, vesicular, vegetating, nodular, and sine bulla pemphigoid. Patients with erythroderma who fulfill diagnostic criteria for bullous pemphigoid and in whom bullae develop have been reported. We describe a patient with exfoliative erythroderma without any blistering in whom a diagnosis of bullous pemphigoid was based on examination of a biopsy specimen. Circulating antibodies to the basement membrane zone, with an epidermal pattern on salt split skin, and the presence of eosinophilic spongiosis in the skin biopsy specimen supported the diagnosis.
Aged↗
Follicular mucinosis presenting as an acneiform eruption: report of four cases.
Follicular mucinosis can be a primary idiopathic disease or a secondary disease associated with lymphoma. When it appears in childhood or adolescence, it is usually primary and self-limited. We describe four cases of follicular mucinosis occurring in early adulthood that have had protracted courses. Each presented as an unusual acneiform eruption. Two of the cases demonstrated a clonal genetic rearrangement of the T-cell receptor within the cutaneous lymphoid infiltrate, a finding not previously reported. Although its significance is not clear, the clonal lymphocytic expansion indicates a need for continued surveillance of these patients.
Acne Vulgaris↗
Cytosine arabinoside-induced vasculitis.
Dermatologic side effects of cytosine arabinoside (ara-C) are rare and most commonly occur after high-dose (more than 100 to 200 mg/m2 per day) therapy has been administered for several days. Although vascular reactions after combination chemotherapeutic regimens with ara-C have been anecdotally described, they have not been previously reported after single-agent chemotherapy with ara-C. Herein we describe two patients with acute nonlymphoblastic leukemia in whom cutaneous small vessel necrotizing vasculitis developed after high-dose single-agent chemotherapy with ara-C. Cutaneous lesions developed 3 to 5 days after initiation of therapy and resolved spontaneously within a week after the chemotherapeutic regimen was completed. No evidence of systemic vasculitis was present in either patient. Prior experimental data have demonstrated a direct toxic effect of ara-C on endothelial cells, and this outcome may represent the underlying mechanism of vascular injury.
Adult↗
Subcorneal pustular dermatosis: a clinical study of ten patients.
Subcorneal pustular dermatosis of Sneddon and Wilkinson (SPD) is a unique clinical and histologic entity. This disorder has been reported in association with monoclonal gammopathy and IgA pemphigus. We report ten additional cases of SPD and determine the frequency of monoclonal gammopathy and IgA pemphigus in patients with SPD and investigate the relationship of this entity with other dermatoses characterized histologically by a subcorneal pustule. The medical records of patients with SPD evaluated at the authors' institution from 1980 through 1995 were reviewed retrospectively. Medical records of twenty patients with pustular psoriasis were reviewed for comparison. Ten patients met the criteria for SPD. Their average age at diagnosis was 66 years. The characteristic flaccid pustules were often generalized and had a tendency to involve the flexural areas. Serum monoclonal gammopathy was present in four patients: three with IgA and one with IgG. On direct immunofluorescence examination, three patients had IgA deposits in the intercellular spaces. None of the patients exhibited both monoclonal gammopathy and IgA deposits. Repeated direct immunofluorescence studies were necessary to detect IgA pemphigus in one patient. SPD is a distinct clinical entity. The findings of IgA deposits intercellularly on immunofluorescence and monoclonal gammopathy may help to further identify this entity.
Aged↗
Clinicopathologic correlation of hypocomplementemic and normocomplementemic urticarial vasculitis.
BACKGROUND: Urticarial vasculitis is characterized by persistent urticarial lesions with histologic evidence of leukocytoclastic vasculitis. Hypocomplementemic urticarial vasculitis (HUV) is a distinct clinical entity in a subset of patients with urticarial vasculitis. OBJECTIVE: We examined presentation of urticarial vasculitis and factors predictive of connective tissue disease. METHODS: The clinical, histologic, and immunologic characteristics of 132 patients with urticarial vasculitis seen at the Mayo Clinic were examined, and features of the hypocomplementemic patients were compared with those of the normocomplementemic patients. RESULTS: Twenty-four patients (18%) had hypocomplementemia; all were female. Interstitial dermal neutrophilia was seen in 19 biopsy specimens (83%). On direct immunofluorescence (DIF) testing of lesional skin, 23 patients (96%) had a continuous strong granular deposition of immunoreactants along the basement membrane zone compatible with lupus erythematosus in addition to vascular fluorescence. Systemic lupus erythematosus (SLE) was present or occurred in 13 (54%). One hundred eight patients (82%) had normocomplementemia; 65 (60%) were female. Interstitial dermal neutrophilia was seen in 11 of 26 (42%) randomly selected biopsy specimens. On DIF, one patient (1%) had the lupus band. SLE occurred in three patients (3%). CONCLUSION: Patients with HUV were more likely to be female, to have diffuse neutrophilia on biopsy specimens stained with hematoxylin and eosin, to have continuous strong granular deposition of immunoreactants along the basement membrane zone on DIF, and to have SLE than normocomplementemic patients. We submit that HUV represents a subset of SLE with shared clinical, laboratory, and immunologic features.
Adolescent↗
Malignant pyodermas revisited.
The concept of malignant pyoderma (MP) has created controversy since its origin. The distinction of this disease from pyoderma gangrenosum was based on clinical criteria and response to treatment. Herein we discuss our current ideas on this entity and its possible relationship to Wegener's granulomatosis (WG). Follow-up data from the three original cases of MP are reported, as well as additional clinical and laboratory data from cases subsequently thought to represent MP. Many of these cases have similar clinical features such as facial and periauricular ulceration and occasionally signs or symptoms of WG, including positive titers of antineutrophil cytoplasmic antibodies (with a diffuse cytoplasmic staining pattern) (cANCA). MP represents a distinctive clinical disorder and may be a dermal manifestation of WG. Some cases of MP may represent pyoderma gangrenosum or other undefined systemic illnesses. Such cases of WG can be distinguished on the basis of clinical, histopathologic, and laboratory evidence including cANCA titers. MP should no longer be used as a final clinical diagnosis.
Adult↗
Hydroxyurea dermopathy: a unique lichenoid eruption complicating long-term therapy with hydroxyurea.
BACKGROUND: Hydroxyurea is usually a well tolerated antitumor agent. OBJECTIVE: Our purpose was to describe a distinct clinical and histologic eruption in patients receiving long-term hydroxyurea therapy. METHODS: The clinical, histologic, and immunopathologic features of six patients with hydroxyurea dermopathy are described. RESULTS: Three women and three men were identified. The average age was 61 years. Hydroxyurea had been used for an average of 5 years. Lichenoid papules, telangiectasia, and poikilodermatous lesions on the dorsal hands and digits were the most common findings. Interface dermatitis, focal lichenoid reaction with epidermal atrophy, and Civatte bodies were the most common histologic findings. Endothelial swelling also was noted. Cytoid staining with multiple conjugates was the most common immunopathologic finding. Four patients showed significant improvement after discontinuation of hydroxyurea. CONCLUSION: A distinct cutaneous reaction to long-term administration of hydroxyurea has been characterized. Cessation of treatment is necessary for healing or improvement. We have designated this eruption hydroxyurea dermopathy.
Aged↗
Skin necrosis secondary to low-molecular weight heparin in a patient with antiphospholipid antibody syndrome.
Skin necrosis is a rare complication of subcutaneous heparin therapy that usually occurs at injection sites. It occasionally accompanies the heparin-associated thrombocytopenia and thrombosis syndrome. We describe a patient with the antiphospholipid syndrome who had skin necrosis develop from low-molecular weight heparin therapy at sites distant from injection sites.
Adult↗
No evidence for Borrelia burgdorferi infection in lesions of morphea and lichen sclerosus et atrophicus in Spain. A prospective study and literature review.
The possible association of Borrelia burgdorferi with morphea and lichen sclerosus et atrophicus has been the focus of research and discussion in dermatology during the last 10 years. To investigate the etiopathogenic role of B. burgdorferi in morphea and lichen sclerosus et atrophicus lesions in Spain, we studied 14 cases: 8 patients with lichen sclerosus et atrophicus and 6 with morphea. For the whole group, a prospective study was performed, including serologic studies by indirect immunofluorescence, histologic evaluation of skin biopsy specimens, culture studies, and polymerase chain reaction with different primers sensitive for detecting virtually all B. burgdorferi strains tested to date. Although one patient with morphea had positive serologic findings at low titer, we were not able to culture or detect borrelial DNA in any of the specimens. These findings do not confirm an association between B. burgdorferi and morphea and lichen sclerosus et atrophicus.
Adolescent↗
Mucosal, genital, and unusual clinical variants of melanoma.
To demonstrate the need for a through cutaneous and mucosal examination, we discuss and illustrate the spectrum of mucosal melanomas and unusual clinical variants of melanoma. Although cutaneous areas exposed to sunlight are most vulnerable, melanomas can occur in any site on the skin or mucous membranes. Pigmented nevi as well as mucosal and labial melanotic macules are lesions that simulate oral mucosal melanomas but are not associated with such a poor prognosis. In contrast, the 5-year survival rate for patients with malignant melanomas of the oral mucosa is only 5%. Similarly, the prognosis is poor for patients who have malignant melanomas of the vulva, vagina, male genitalia, or anorectal area; most patients with such lesions are 50 years of age or older. Subungual and plantar areas are common sites of malignant melanomas, and involvement of the eyelid margin portends a poor prognosis. Other rare variants-desmoplastic, amelanotic, and polypoid malignant melanomas-are associated with local recurrences and metastatic lesions. Early diagnosis is the key to proper treatment and improved survival rate for patients with these unusual variants of melanoma. Increased awareness of the wide variety of clinical features of melanoma should lead to earlier diagnosis.
Diagnosis, Differential↗
Granulomatous vasculitis associated with herpes virus: a persistent, painful, postherpetic papular eruption.
Cutaneous granulomatous vasculitis manifesting as a postherpetic reaction pattern is uncommon hand has previously been reported as a delayed complication of varicella-zoster virus infection. We describe three patients who had persistent, painful, postherpetic papules in a zosteriform distribution that histologically demonstrated a small vessel granulomatous vasculitis. Herpes simplex virus DNA detected by the polymerase chain reaction technique was demonstrated in two cases.
Aged↗
Cutaneous periarteritis nodosa: a clinicopathological study of 79 cases.
Cutaneous periarteritis nodosa (PAN) is a well-recognized entity characterized by tender subcutaneous nodules and livedo that may ulcerate. The pathogenesis of cutaneous PAN is not known. The objective of the study was to evaluate the clinical and histological features of 79 cases of cutaneous PAN and to investigate any clinical, pathological and immunological differences that may distinguish those cases likely to have a prolonged course. A retrospective analysis of 79 cases was conducted. Thirty-nine patients had ulcers during the course of their illness. Women were affected more than men. Painful nodules on the lower extremities, with oedema and swelling, were the most common clinical finding; 22% of patients had some evidence of neuropathy. Most of the laboratory findings were non-specific. There was no evidence for hepatitis B infection and hepatitis C infection was present in only one patient. Most patients (60%) had no associated medical condition. The disease course was prolonged but benign, and systemic PAN did not develop in any patient. Corticosteroids given systemically induced remission in most acute cases. The ulcerative form of disease was more prolonged and frequently associated with neuropathy.
Adolescent↗
Plantar granulomatous cutaneous T-cell lymphoma.
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Biopsy↗
Chronic hepatitis C, cryoglobulinemia, and cutaneous necrotizing vasculitis. Clinical, pathologic, and immunopathologic study of twelve patients.
BACKGROUND: Several patients with chronic hepatitis C infection and cutaneous vasculitis have been described. OBJECTIVE: The objective of this study was to define better the features of necrotizing vasculitis and mixed cryoglobulinemia in patients with hepatitis C infection. METHODS: A retrospective review of 611 patients with hepatitis C antibodies was conducted. Patients with clinical and histopathologic findings of cutaneous necrotizing vasculitis were identified. Clinical, histologic, and laboratory data were recorded. RESULTS: Twelve patients with necrotizing vasculitis and chronic hepatitis C infection were identified. Palpable purpura was the most common clinical presentation. Onset of skin lesions was usually more than 10 years after infection. The lower extremities were affected in all patients. Cryoglobulinemia of the mixed type II was present in 10 of 11 patients. Liver function tests were evaluated at the time of vasculitis in most of the patients. Rheumatoid factor was elevated in all nine patients tested. Total complement was decreased in seven of nine patients, and C4 was decreased in six of seven patients. CONCLUSION: Cutaneous vasculitis associated with cryoglobulinemia and hypocomplementemia is not uncommon in the course of chronic active hepatitis C infection. The triad of necrotizing vasculitis, chronic hepatitis C infection, and cryoglobulinemia occurs late after initial infection with hepatitis C. Antibodies to hepatitis C virus should be determined in a patient with necrotizing vasculitis, especially if liver function tests are elevated.
Adult↗
Aleukemic monocytic leukemia cutis.
Aleukemic leukemia cutis is a rare condition in which leukemic cells invade the skin before they appear in peripheral blood or bone marrow specimens. Herein we describe a 67-year-old man who underwent assessment because of papules and nodules on his back and lower extremities. A biopsy of these lesions confirmed a dense, predominantly monocytic infiltrate of the dermis and subcutaneous tissue. Immunohistochemical stains were positive for CD43 (Leu-22) as well as monocytic markers. Bone marrow and peripheral blood examinations failed to reveal leukemia. Treatment was based on the results of the skin biopsy, and the patient is doing well 1 year after therapy.
Aged↗
Sudden nodular growth in a congenital facial lesion. Squamous cell carcinoma arising in a nevus sebaceus Jadassohn (NSJ).
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Aged↗