Lawrence E. Gibson

Urticarial vasculitis: A histopathologic and clinical review of 72 cases

BACKGROUND Urticarial vasculitis is a subset of vasculitis characterized clinically by urticarial skin lesions and histologically by necrotizing vasculitis. OBJECTIVE A review of patients with urticarial vasculitis was undertaken to further characterize the clinical and histologic findings and to differentiate this disorder from urticaria and other types of cutaneous vasculitis. METHODS Seventy-two cases of biopsy-proven urticarial vasculitis were selected for a review of medical records, laboratory data, and histologic findings. Fifty cases of simple urticaria were also reviewed for purposes of comparison. RESULTS Systemic symptoms in patients with urticarial vasculitis included angioedema in 30 patients (42%), arthralgias in 35 (49%), pulmonary disease in 15 (21%), and abdominal pain in 12 (17%). Twenty-three patients (32%) had hypocomplementemia. Forty-six of 72 patients (64%) had lesions that lasted more than 24 hours, 23 of 72 (32%) had painful or burning lesions, and 25 of 72 (35%) had lesions that resolved with purpura. Sixteen biopsy specimens from the 23 patients with hypocomplementemia showed dermal neutrophilia in addition to the perivascular infiltrate. Of the 23 patients with hypocomplementemia, 20 (87%) had fluorescence of the blood vessels and 16 (70%) had fluorescence of the basement membrane zone as determined by routine direct immunofluorescence. CONCLUSION Patients with hypocomplementemia were more likely to have systemic symptoms such as urticaria that resolved with purpura, arthralgias, abdominal pain, and chronic obstructive pulmonary disease. The histologic pattern associated with hypocomplementemia is interstitial neutrophilic infiltrate of the dermis and an immunofluorescent pattern of immunoglobulins or C3 in the blood vessels and along the basement membrane zone.

Sweet's Syndrome: Systemic Signs and Symptoms and Associated Disorders

OBJECTIVE To characterize the findings associated with acute febrile neutrophilic dermatosis (Sweets syndrome [SS]) and the response of SS to treatment. DESIGN We retrospectively reviewed 48 cases of SS encountered at the Mayo Clinic between 1980 and 1992. MATERIAL AND METHODS Histopathologic specimens and medical records were studied to determine initial manifestations, patterns of involvement, systemic signs and symptoms (including mucosal, musculoskeletal, hematologic, pulmonary, hepatic, and renal findings), and conditions associated with SS. RESULTS In patients with SS, the typical manifestations are the acute onset of tender, erythematous or violaceous nodules or plaques in association with fever, leukocytosis, and dermal neutrophilia. In our study group, the cutaneous lesions most frequently involved the arms and legs. Of our 48 patients, 26 (54%) had a hematopoietic, plasma cell, or malignant disorder, and many of these patients had associated anemia, especially the male patients. No single laboratory finding specifically indicated an association with serious systemic disease. Most patients were treated with a tapering dose of prednisone, which yielded a good response. CONCLUSION Clinical acumen and appropriate laboratory tests are the main requirements for detection of hematologic disorders, internal malignant diseases, or other systemic conditions associated with SS.

Erythema elevatum diutinum: a clinical and histopathologic study of 13 patients.

BACKGROUND Erythema elevatum diutinum is a rare condition representing a chronic leukocytoclastic vasculitis. OBJECTIVE Clinical and laboratory features of the disease were reviewed to better understand the disease. METHODS The medical records and histopathologic slides of 13 patients with erythema elevatum diutinum were studied. RESULTS The lesions were violaceous, deep red, or brown and typically were papules or plaques. Lesions were most often located on the extensor surfaces of the extremities. Associated medical problems included hematologic abnormalities in six patients: IgA clonal gammopathies (four), multiple myeloma (one), and myelodysplasia (one). Erythema elevatum diutinum preceded the myeloproliferative disorders by an average of 7.8 years. All patients showed vasculitis. Leukocytoclasia was present in 27 of 35 specimens. The predominant cell type in the inflammatory infiltrate was polymorphonuclear leukocytes or a mixture of polymorphonuclear leukocytes and lymphocytes. CONCLUSION The most significant finding of this study is the association of erythema elevatum diutinum with hematologic disease, most frequently an IgA monoclonal gammopathy.

Increasing Incidence of Melanoma Among Young Adults: An Epidemiological Study in Olmsted County, Minnesota

OBJECTIVE To identify the change in the incidence of cutaneous melanoma over time among young adults. PATIENTS AND METHODS Using Rochester Epidemiology Project data, we identified patients aged 18 to 39 years who had a first lifetime diagnosis of melanoma from January 1, 1970, through December 31, 2009, in Olmsted County, Minnesota. Demographic and clinical information, including survival, was abstracted, and estimates of the incidence of melanoma and overall and disease-specific survival were generated. RESULTS From 1970 to 2009, the incidence of melanoma increased by 8-fold among young women and 4-fold among young men. Overall and disease-specific survival seemed to improve over time; hazard ratios comparing year of diagnosis with mortality were 0.92 and 0.91, respectively. CONCLUSION The incidence of cutaneous melanoma among young adults is rapidly increasing, especially among women. Continued close monitoring of this high-risk population is necessary.

Necrobiotic xanthogranuloma: long-term outcome of ocular and systemic involvement ☆

PURPOSE To report long-term outcomes of patients with necrobiotic xanthogranuloma, to investigate the propriety of therapeutic surgical excision or debulking, and to study tissue specimens by immunoperoxidase staining and in situ hybridization. METHODS Medical records of all patients at the Mayo Clinic, Rochester, Minnesota, with necrobiotic xanthogranuloma between 1980 and 1997 were reviewed. A follow-up letter was sent to each patient inquiring about the current status of the lesions, the treatment regimen, and associated systemic diseases. RESULTS The average age (+/- standard deviation) of the 15 men and 11 women was 56.8 +/- 14.8 years. Of the 26 patients, 21 (81%) had lesions of the ocular adnexa. Ulceration of the lesions occurred in 11 patients (42%). The lesions recurred after surgical removal in 11 patients (42%) and on prior incision sites from unrelated operations in three patients (12%). The average duration of follow-up from the appearance of characteristic skin lesions was 10 +/- 6.1 years. Four patients had multiple myeloma, five had a plasma cell dyscrasia, and one had a lymphoproliferative disorder during this period. Time to development of associated malignancy ranged from 8 years before the skin lesions to 11 years after the skin lesions. Overall survival was 100% at 10 years and 90% at 15 years (95% confidence limit, 0.73 to 1.00). Immunoperoxidase stains demonstrated that most histiocytes are not of Langerhans cell lineage. Monoclonal immune globulins were not identified in tissue specimens. CONCLUSION Care of patients with necrobiotic xanthogranuloma should include avoidance of surgical removal, if possible, and lifelong follow-up to detect the development of associated malignancy.

Folliculotropic Mycosis Fungoides: Single-Center Study and Systematic Review

OBJECTIVES To clarify clinicopathologic features and reconcile discrepancies in previous studies of folliculotropic mycosis fungoides (FMF). DESIGN A single-center retrospective clinicopathologic study and a systematic review of FMF. SETTING Tertiary referral center in the midwestern United States. PATIENTS Patients with clinical and histopathologic evidence of FMF seen at the tertiary referral center during a 12(1/2)-year period. MAIN OUTCOME MEASURES Clinicopathologic features of FMF. RESULTS Fifty patients (32 male [64%] and 18 female [36%]) met study criteria for the clinicopathologic review. Pruritic patches, plaques, and folliculocentric lesions (milia, cysts, and alopecia) on the head, neck, and trunk were common clinical findings. The mean time to diagnosis of FMF was 5.0 years. Diagnostic latency did not affect risk of death. One-year and 5-year overall survival rates were 96% and 62%, respectively. Frequent microscopic features were follicular mucinosis (74%) and epidermotropism (54%). Systematic review of 186 additional patients confirmed male predominance (ratio of men to women, 3.2:1.0), prevalent pruritus (73%), frequent follicular mucinosis (69%) and epidermotropism (37%) microscopically, and common head, neck, and trunk involvement. Combined data demonstrated that 6% of patients with FMF had concurrent non-mycosis fungoides hematologic malignant neoplasms and that the 5-year overall survival rate was 62% to 64%. CONCLUSION Folliculotropic mycosis fungoides has distinct clinical and microscopic features and is associated with a poor 5-year overall survival rate.

Clinical, pathologic, and immunopathologic features of dermatitis herpetiformis: review of the Mayo Clinic experience,*†

Background  Dermatitis herpetiformis (DH) is a cutaneous manifestation of gluten sensitivity, occasionally associated with other autoimmune disorders, and reportedly associated with an increased risk of lymphoproliferative disorders. We describe a series of patients with DH, focusing on associated disorders (particularly celiac disease), incidence of lymphoma, histopathology, and sensitivity of direct immunofluorescence (DIF) testing and serologic testing with antiendomysium antibodies for the diagnosis of DH.

A clinical and histopathologic study of granulomatous rosacea.

A retrospective clinical and histopathologic study of 53 patients with granulomatous rosacea was undertaken. The patients had a broad clinical spectrum of lesions that ranged from primarily erythema to papulonodular lesions. Extrafacial lesions occurred in 15% of patients. Histologic examination showed mixed lymphohistiocytic inflammation (primarily lymphocytic inflammation in 40% of patients and primarily histiocytic with a few giant cells in 34%), epithelioid granulomas in 11% of patients, and epithelioid granulomas with caseation necrosis in 11%. Most patients had a good response to oral antibiotic therapy. Granulomatous rosacea is not a distinct disease but can be regarded and treated as a subtype of rosacea.

Chronic Hepatitis C and Skin Diseases: A Review

OBJECTIVE To emphasize the ongoing role of chronic hepatitis C virus (HCV) infection in the cause or exacerbation of severe dermatologic disorders. DESIGN We present two case reports to outline the pertinent findings in hepatitis C-related cryoglobulinemia, leukocytoclastic vasculitis, and lichen planus and discuss the main disorders associated with chronic HCV infection. RESULTS Chronic HCV infection has recently been recognized in association with various skin disorders. The most commonly reported association is the triad of leukocytoclastic vasculitis, cryoglobulinemia, and chronic HCV infection. Other cutaneous disorders associated with HCV infection include porphyria cutanea tarda, lichen planus, erythema nodosum, urticaria, erythema multiforme, and polyarteritis nodosa. CONCLUSION Patients with onset or exacerbation of these disorders should undergo assessment for HCV infection.

Follicular Mucinosis: Histopathologic Review of 33 Cases

Among 33 patients with the histologic diagnosis of follicular mucinosis (alopecia mucinosa) made at our institution between 1982 and 1989, 9 had mycosis fungoides diagnosed concomitantly. Three other patients had lymphoproliferative disorders, and two had Kaposis sarcoma. Analysis of biopsy features such as epidermal lymphocytic exocytosis, periappendageal infiltrate, and deposition of mucin revealed no predominant finding that distinguished a benign course from mycosis fungoides. A predominance of eosinophils in the infiltrate was suggestive of benign follicular mucinosis rather than mycosis fungoides. Gene rearrangement studies detected three clones in three patients with follicular mucinosis; two were in patients with mycosis fungoides, and one was in a patient with dermatitis. The outcome of these three patients is pending further follow-up. No histopathologic or clinical features distinguished these patients from the others.