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Background: We encountered a distinct arteriolar histopathologic finding of lymphocytic vasculitis associated with a hyalinized fibrin ring in vessel lumina. Identical histologic findings have previously been described as macular arteritis., Observations: We describe 5 women (mean age, 25 years; age range, 20-34 years) with persistent, slowly progressive, patchy and reticular hyperpigmentation associated with livedo racemosa affecting predominantly the lower limbs. In the biopsy samples, infiltration of muscular vessel wall by inflammatory cells, affecting small arteries of the dermosubcutaneous junction or superficial subcutis, was present. Of the infiltrate, 90% or more consisted of mononuclear cells, mainly lymphocytes with an admixture of histiocytes. Neutrophils and eosinophils were absent or scant. Inflammation was confined to the vicinity of the vessel and the immediate surrounding panniculus. A concentric fibrin ring involving the entire periphery of the lumina of affected vessels was present in all the patients. Laboratory investigation results revealed that 4 patients had antiphospholipid antibodies in their serum. One of these patients had a heterozygous mutation of the factor V Leiden gene. Conclusion We term this arteritis lymphocytic thrombophilic arteritis to reflect the histologic features that combine lymphocytic vascular inflammation with changes representing a thrombophilic endovasculitis.

Background: Urticarial dermatitis may represent a useful term for a subset of a reaction pattern designated most commonly as dermal hypersensitivity by pathologists. The term is not commonly used, and requires definition to determine whether it is clinically relevant., Objectives: To define urticarial dermatitis and distinguish it from other urticarial reaction patterns and to review the frequency with which dermatologists can recognize clinical settings that match the biopsy findings of urticarial dermatitis., Design: Retrospective analysis of clinical and/or histological diagnosis of urticarial dermatitis, applying strict histological criteria in a center using urticarial dermatitis as a diagnostic term in 190 archived reports., Setting: Tertiary referral dermatopathology service reporting for dermatological practices in Sydney, Australia., Main Outcome Measures: The correlation between clinical and histological diagnoses of urticarial dermatitis and alternate diagnoses was analyzed. The frequency of positive immunofluorescence findings for bullous pemphigoid was determined in a subset of patients with urticarial dermatitis in whom this test was ordered to exclude prodromal bullous pemphigoid., Results: Urticarial dermatitis was the histological diagnosis in at least 1 biopsy result in 148 patients, and matched the provisional clinical diagnosis in 49 (33.1%) patients. Urticarial dermatitis was the only diagnosis provided in 21 patients. The main alternate clinical diagnoses provided were early bullous pemphigoid or dermatitis herpetiformis (47 patients [31.8%]), dermatitis (39 patients [26.4%]), drug reaction (35 patients [23.6%]), urticarial vasculitis (24 patients [16.2%]), and urticaria (12 patients [8.1%]). In 91 patients with a clinical diagnosis of urticarial dermatitis, the histological diagnosis in at least 1 biopsy result was matched in 49 patients (53.8%); other histological diagnoses included dermatitis (21 patients [23.1%]), papular urticaria (12 patients [13.2%]), drug reaction (6 patients [6.6%]), and urticaria (3 patients [3.3%]). Review of 38 direct immunofluorescent results for prodromal bullous pemphigoid and a biopsy finding of urticarial dermatitis revealed only 3 positive results (7.9%)., Conclusions: Urticarial dermatitis seems to be a useful histological and clinical term for a subset of the dermal hypersensitivity reaction pattern. Although the clinical presentation is not restricted to a specific entity, eczema and drug reactions seem to be the most frequent clinical associations; and in a subset of patients, urticarial dermatitis remains as a recognizable reaction pattern. Urticarial dermatitis without eosinophilic spongiosis is not a reliable indicator for bullous pemphigoid, because the findings of immunofluorescence are often negative.

Background: Retinoids have the capacity to accelerate the involution of multiple keratoacanthomas, including unusual variants such as keratoacanthoma marginatum centrifugum and keratoacanthoma en plaque that may persist and be associated with progressive growth and provide difficulties in diagnosis and management., Observations: We describe 3 patients who had unusual infiltrated and keratotic plaques affecting the lower legs or nasolabial area that persisted or recurred that may be related to this group of unusual keratoacanthomas. The 3 patients had differing clinical lesions that did not resemble classic keratoacanthomas, but were linked by their biopsy findings of hypertrophic lichen planus-like reaction and pseudoepitheliomatous hyperplasia with a prominent infundibulocystic component that progressed to multiple keratoacanthomas or infundibulocystic squamous cell carcinoma. Polymerase chain reaction analysis of biopsy material from 2 patients failed to detect human papillomavirus. All 3 presentations provided a therapeutic dilemma, but responded rapidly to acitretin treatment at a dosage of 10 to 25 mg daily, which was continued for 15 to 24 months., Conclusions: These cases illustrate an unusual reaction pattern that is hypertrophic lichen planus-like but, instead of evolving to classic lichen planus, progresses to infundibulocystic hyperplasia and the development of multiple keratoacanthomas or infundibulocystic squamous cell carcinomas. Retinoids represent a therapeutic option for this difficult clinical problem and may obviate repeated and extensive surgery.

Objective: To establish a safe and efficacious dosing regimen for the treatment of primary nodular basal cell carcinoma (BCC) using 5% imiquimod cream., Design: Two phase 2 studies were conducted: a 6-week, randomized, open-label, dose-response study evaluating 4 dosing regimens and a 12-week, randomized, vehicle-controlled, double-blind, dose-response study evaluating 4 dosing regimens., Setting: Twenty-four public and private dermatology clinics in Australia and New Zealand (6-week study) and the United States (12-week study) participated., Patients: The study populations comprised 99 patients enrolled in the 6-week study and 92 patients in the 12-week study. Patients were at least 18 years old and had a biopsy-confirmed diagnosis of nodular BCC., Interventions: In the 6-week study, imiquimod was applied once daily for 3 or 7 days per week or twice daily for 3 or 7 days per week. In the 12-week study, imiquimod or placebo cream (vehicle) was applied once daily for 3, 5, or 7 days per week, or twice daily for 7 days per week. The entire tumor area was excised 6 weeks after treatment and examined histologically for evidence of remaining BCC., Main Outcome Measure: The proportion of patients having no histologic evidence of BCC in the posttreatment excision specimen., Results: Dosing once daily for 7 days per week resulted in the highest clearance rate, with 25 (71%) of 35 and 16 (76%) of 21 patients showing clearance of their tumor in the 6- and 12-week studies, respectively., Conclusions: Topical 5% imiquimod cream is well tolerated and most effective in treating nodular BCC when applied once daily for 7 days per week for either 12 or 6 weeks.

Background: Recession of the frontal hairline is a common event in postmenopausal women. This has been shown not to be a marker of gross androgenization, and is usually a progressive nonscarring alopecia. Six postmenopausal women, who developed a progressive frontal scarring alopecia, were studied and their clinical and laboratory data, as well as the results of scalp biopsy specimens in all six patients, were analyzed and compared with recognized forms of scarring alopecia and recently described findings in androgenetic alopecia., Observations: The six postmenopausal women developed a progressive frontal hairline recession that was associated with perifollicular erythema within the marginal hairline, producing a frontal fibrosing alopecia extending to the temporal and parietal hair margins. Scalp biopsy specimens from the frontal hair margin showed perifollicular fibrosis and lymphocytic inflammation concentrated around the isthmus and infundibular areas of the follicles. Immunophenotyping of the lymphocytes showed a dominance of activated T-helper cells. Clinical review of all six cases showed a progressive marginal alopecia without the typical multifocal areas of involvement seen in lichen planopilaris or pseudopelade. None of the patients had mucous membrane or skin lesions typical of lichen planus. Hormonal studies, in five patients, showed no elevated androgen abnormalities., Conclusions: Progressive frontal recession in postmenopausal women may show clinical features of a fibrosing alopecia. The histologic findings are indistinguishable from those seen in lichen planopilaris. However, the absence of associated lesions of lichen planus in all six women raises the possibility that this mode of follicular destruction represents a reaction pattern triggered by the events underlying postmenopausal frontal hairline recession.

The conditions of three patients who had generalized granuloma annulare responded favorably to treatment with alkylating agents. This mode of therapy in two cases was chosen on the basis of recent evidence that implicates delayed hypersensitivity mechanisms as forming part of the pathogenesis of granuloma annulare. Two patients had rapid clearing of their lesions. The response in one of these patients was observed during therapy for a concomitant plasma cell dyscrasia. The third patient had an unusual form of granuloma annulare that had evolved during a period of 28 years, with hand deformities secondary to fascial and tendon involvement and the production of contractures and lymphedema, which responded partially to therapy. The results lend further support to data implicating cellular immunity to granuloma annulare.

In ten cases of neurotropic melanoma, patients presented with nodules composed of amelanotic, deeply infiltrating neuroid fascicles. Only four cases were clinically suggestive of melanoma. In eight of the tumors, a precursor lesion was found histologically and provided a major clue to the diagnosis. In seven cases, the dysplastic precursor was lentiginous, while a superficial spreading pattern was present in one. Initial surgery was often inadequate because of the difficulty in defining tumor margins and the lack of pigment. In seven of the tumors, S100 protein was demonstrated within the invasive spindle cell component by the immunoperoxidase method. This finding was negative in three cases, two of which showed positive staining of the precursor and nerve filaments, indicating that the absence of S100 protein cannot be used as an exclusion criterion for neurotropic melanoma.

Progressive cutaneous papulonodular non-X histiocytosis of the face and proximal parts of the extremities developed in a 28-year-old woman. Histologic study showed masses of benign histiocytes with foci of lymphocytes. Occasional cells contained diastase-resistant cytoplasmic polysaccharide that stained positive with PAS stain. No iron, fat, or Touton or other giant cells were present. Ultrastructural study disclosed laminar bodies but no Langerhans' cells or Langerhans' granules. The process responded to low-dose intravenous vinblastine sulfate treatment.

A 9-year-old girl had a widespread papular eruption that was histologically characterized by benign histiocytes that were acid phosphatase-positive, but that lacked Langerhans' granules ultrastructurally. New lesions had continued to develop since the patient was 3 months old, and individual lesions had resolved over a period of two to three months. The histopathologic, histochemical, and ultrastructural findings in this patient were similar to those previously described for eruptive histiocytomas in adults. Papular histiocytomas in childhood may be found in histiocytosis X, reticulohistiocytoma, xanthogranuloma, xanthoma disseminatum, or eruptive histiocytoma. These diseases may be differentiated on the basis of differing histiocytic populations and the clinical courses of the lesions in each disorder.

Multiple subcutaneous nodules and recurrent flu-like episodes developed in a 60-year-old woman after she had received local intramuscular injections of procaine povidone for a seven-year period. A skin biopsy specimen showed distinctive gray-blue material in the histiocytes in sections stained with hematoxylin-eosin. Povidone is an inert substance that may accumulate in multiple organs, including the skin. Its presence can be verified by its affinity for special stains or by spectrophotometric analysis. In our patient, ultrastructural study showed amorphous inrahistiocytic deposits in the tissues. -revious reports have indicated that this substance may eventually accumulate in noninjected skin sites and may lead to pulmonary and arthritic symptoms in some patients, but no other patients, to our knowledge, have had complications associated with fever.