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.

Pyoderma gangrenosum: classification and management.

Pyoderma gangrenosum (PG) has four distinctive clinical and histologic variants. Some have morphologic and histologic overlapping features with other reactive neutrophilic skin conditions. PG often occurs in association with a systemic disease, and the specific clinical features of the skin lesion may provide a clue to the associated disease. Management of PG depends on its type and severity and usually requires aggressive local and systemic treatment.

Olmsted syndrome.

Nine cases of Olmsted syndrome have been reported in the world literature. In this syndrome, keratoderma usually starts during infancy on the palms and soles when the baby starts to use the feet for walking and the hands for grasping. Within weeks or months, there is progressive spread of solid, symmetrical, thick hyperkeratotic keratoderma to both palms and soles, surrounded by erythematous margins. Contraction of fingers and deep fissuring of the feet are common complications. Symmetrical, yellow-brown hyperkeratotic plaques and papules are also observed around body orifices such as the mouth, nares, inguinal region, and perianal and gluteal areas. Other clinical manifestations have been reported, including diffuse alopecia, thin nails, leukokeratosis of the oral mucosa, onychodystrophy, hyperkeratotic linear streaks, exaggerated keratosis pilaris, and large verrucous plaques in the axillae. In the differential diagnosis, other keratoderma and hyperkeratotic syndromes should be considered.

Hypertrophic discoid lupus erythematosus resembling squamous cell carcinoma.

BACKGROUND: Hypertrophic discoid lupus erythematosus (HDLE) may resemble cutaneous squamous cell carcinoma (SCC) histologically. OBJECTIVE: The histologic features that help to differentiate HDLE from SCC are reviewed. METHODS: Two patients are described with HDLE. RESULTS: Both patients were treated initially with Mohs surgery for "biopsy-proven" SCC. Subsequent biopsies confirmed the diagnosis of HDLE and the patients responded well to appropriate therapy. No significant complications occurred in either patient. CONCLUSION: Lesions of HDLE may imitate SCC. Strategies are suggested to avoid surgical procedures based on an incorrect biopsy report.

From mercury to malaria to penicillin: the history of the treatment of syphilis at the Mayo Clinic--1916-1955.

Between 1916 and 1955 the Mayo Clinic became recognized as one of the premier institutions specializing in the treatment of syphilis. First under the direction of John H. Stokes (1916-1924) and later Paul A. O'Leary (1924-1953), its Department of Dermatology and Syphilology, together with the members of the Clinical Cooperative Study Group, oversaw the establishment of standardized methods for the administration of the existing arsenicals and the introduction of new therapies. Malaria therapy, heat therapy, penicillin, and oxytetracycline each represented important advances in the treatment of syphilis and were extensively evaluated. Two important ancillary benefits of syphilis treatment were the development of routine intravenous techniques, which would later prove invaluable for the administration of antibiotics and cancer drugs, and the establishment of large cooperative clinical trials, the first of their kind. Under the leadership of Stokes and O'Leary the department produced a stream of pivotal clinical research that contributed to the effective management of syphilis in the United States.

Incidence of psoriasis in Rochester, Minn, 1980-1983.

This population-based study was carried out using the medical records linkage data resource for the population of Rochester, Minn, at Mayo Clinic. There were 132 newly diagnosed cases of psoriasis identified during a 4-year period (1980 through 1983); 88% of the cases had been seen and diagnosed by a dermatologist. The overall crude incidence rate was 57.6 per 100,000 population; for men and women, the rates were 54.4 and 60.2, respectively. The overall sex- and age-adjusted (1980 US white population) incidence rate was 60.4 per 100,000 person-years. The highest rate of occurrence (112.6) was in the 60- to 69-year-old age group. Most of the cases of psoriasis diagnosed in this study (58%) were mild, and the patients had psoriatic lesions on less than 10% of their body. There are no other published incidence rates for this condition with which to make comparisons.

Linear IgA deposition associated with cutaneous varicella-zoster infection: a case report.

The homogeneous linear deposition of IgA along the basement membrane zone of uninvolved skin is a distinguishing feature of linear IgA dermatosis in adults and chronic bullous dermatosis of childhood. Although linear IgA deposition may be seen in other cutaneous diseases, to our knowledge, there are no previous reports of a direct association with cutaneous infection. We describe the finding of intense fluorescence, in a linear pattern, of IgA at the basement membrane zone in an elderly man with disseminated cutaneous varicella-zoster infection.

Scleromyxedema: a scleroderma-like disorder with systemic manifestations.

Scleromyxedema is a rare fibromucinous connective tissue disorder characterized by papular skin lesions associated with sclerosis and a serum monoclonal gammopathy. Little is known about either the natural history or the systemic manifestations of this disease. We reviewed the medical records of 19 patients with biopsy-proven scleromyxedema seen from 1950 to 1985 for evidence of systemic disease. There were 10 males and 9 females with a median age at diagnosis of 53 years. Monoclonal gammopathy was present in 13 patients. Eight patients complained of dysphagia; 3 had proximal esophageal dysfunction and 1 had total esophageal aperistalsis on barium swallow. Proximal muscle weakness was noted in 5, with an inflammatory myopathy in 3. Six patients complained of dyspnea on exertion. Of these, 5 had reduced diffusing capacity, 3 had reduced volumes, and 2 developed cor pulmonale. Pathologic changes characteristic of "scleroderma kidney" were demonstrated in 1 patient at postmortem. One patient had Raynaud's phenomenon and 2 had arthralgias/arthritis with noninflammatory synovial fluids. Although 8 of 12 patients treated with melphalan noted regression of their skin changes, no consistent improvement in the extracutaneous manifestations was demonstrated. Furthermore, 2 patients died of sepsis related to melphalan-induced myelosuppression, and 4 developed hematological malignancies following melphalan therapy. In conclusion, systemic manifestations in scleromyxedema are more prevalent than previously recognized, and can resemble those of scleroderma. Significant toxicity occurred with the use of alkylating agents in these patients, with treatment-related complications developing in 45% of patients treated with melphalan. The lack of definitive data regarding the natural history of this disease complicates the question of optimal therapy, but the use of alkylating agents should be reserved for those patients with severe debilitating skin disease.

Idiopathic gingivostomatitis.

Atypical gingivostomatitis is recognized by a combination of clinical findings, a sharply marginated, deep red edematous quality to the attached gingiva, angular cheilitis, and a sore red tongue. Unfortunately, there are no general laboratory tests that are diagnostic of the condition, although biopsy of the gingiva shows a marked plasma cell infiltrate. The etiology remains obscure, although chewing certain gums or using some types of dentifrices precipitates the problem. The disease seems to involute spontaneously in some patients. Treatment is symptomatic.

Histopathologic and immunopathologic study of pyoderma gangrenosum.

Sixty-three patients with pyoderma gangrenosum were seen and studied at the Mayo Clinic from 1971 to 1980. Biopsies from the erythematous border or necrotic edge of the pyoderma gangrenosum lesions usually demonstrated a characteristic pathogenic morphologic evolution. The early lesions revealed mild to moderate perivascular lymphocytic infiltrate associated with endothelial swelling. The fully developed lesions demonstrated necrosis in addition to a dense lymphocytic infiltration surrounding as well as involving the blood vessels. Extravasation of erythrocytes and thrombosis sometimes were seen. Ulceration, infarction, and abscess formation were found in the later stages of evolution. Direct immunofluorescence results were positive in the blood vessels of 36 of 65 (55%) specimens. IgM, C3, and fibrin were found in the papillary and reticular dermal vessels. IgG and IgA were only occasionally present. Pyoderma gangrenosum appears to be a reactive process that is manifested as a vasculitis. Biopsy material from the advancing active erythematous border has early characteristic dermatopathologic findings of lymphocytic vasculitis. Cutaneous vascular immune deposits suggest an immune pathogenesis of either an immune complex disease or lymphocytotoxic reaction.

Identifying a common--and benign--geriatric skin lesion.

Typical sebaceous hyperplasia is recognized by the yellowish color, lobulated configuration, and central umbilication. Other skin lesions that may be similar in appearance include basal cell epithelioma, molluscum contagiosum, and xanthoma. Chemical cauterization with phenol or trichloroacetic acid is worth trying if the sebaceous hyperplasia is in the middle or upper dermis. When the hyperplasia extends into the subcutaneous tissue, however, these topical agents are less effective.

A skin marker for geriatric cancer.

Dermatomyositis must be differentiated from a number of other diseases, including lupus erythematosus, myxedema, and contact dermatitis. Mixed connective-tissue disease also may be seen as a proximal myopathy and has similar skin findings. Satisfactory treatment of the underlying malignancy often results in disappearance or easier control of the dermatomyositis. The initial treatment step, however, is high-dose corticosteroids combined with oral immunosuppressives to control muscular and cutaneous inflammation.

Alcoholism and psoriasis.

Using criteria for the diagnosis of alcoholism established by the National Council of Alcoholism and by scores on the Self-Administered Alcoholism Screening Test, we compared the prevalence of alcoholism in a group of 99 patients hospitalized for the treatment of psoriasis with that in an age- and sex-matched control group hospitalized for the treatment of other dermatologic disorders. Alcoholism was diagnosed in 11 psoriatic patients and 3 control patients (0.05 less than p less than 0.10); 10 of the 11 psoriatic patients were men, and 1 of the 3 control patients was a man (p less than 0.05). These differences, not noted in certain earlier studies, may reflect a basic association between the two diseases in males or indicate that alcoholism contributes to the morbidity of psoriasis.

Differentiating seborrheic keratosis from skin neoplasm.

The pigmented acanthotic type of lesion may resemble malignant melanoma. When in doubt, a biopsy will usually resolve the question. Seborrheic keratoses may become irritated or inflamed after trauma, biopsy, or exposure to irritants, causing transformation of the basaloid cell type to squamous cells. Such changes may be misinterpreted as diagnostic of basal or squamous epithelioma.

Pyoderma gangrenosum: a review of 86 patients.

The clinical, histologic, and immunofluorescent findings in 86 cases of pyoderma gangrenosum seen at the Mayo Clinic between 1970 and 1983 were reviewed. Males and females were affected in equal numbers. The most frequent site of lesions was the leg. Sixty-seven patients (78 per cent) had associated systemic disease, with arthritis and inflammatory bowel disease being commonest. Cutaneous histopathologic changes varied with the site of biopsy. Lymphocytic vasculitis was predominant in the zone of erythema peripheral to the area of ulceration, while neutrophilic infiltrate and abscess formation were more prominent centrally. In most cases studied, direct immunofluorescence showed immunoglobulins and complement deposited in and around superficial and deep dermal vessels.

Skin lesions from sun exposure: a treatment guide.

Leukoplakia is a clinical term that may histologically represent actinic changes or frank squamous cell carcinoma. Because the nature of dysplasia may differ in various portions of the involved lip, adequate histologic examination is mandatory. Histologic characteristics of squamous cell carcinoma include loss of cell organization and orientation, loss of intercellular bridges, presence of nuclear atypia and mitotic figures, and premature cell keratinization.

Chronic blisters on aging skin: causes and treatment.

Direct immunofluorescence of perilesional tissue in approximately 90% of patients shows a linear or tubular deposition of immunoglobulins--generally IgG and C3--at the basement membrane zone. Mild or localized eruptions can be controlled with topical steroids and wet dressings. If involvement is more extensive, oral prednisone is given in divided doses of 40 to 60 mg/d until the lesions heal.