Sigfrid A. Muller

Incidence of alopecia areata in Olmsted County, Minnesota, 1975 through 1989

OBJECTIVE To assess the incidence and natural history of alopecia areata (AA) among unselected patients from a community. DESIGN We conducted a retrospective population-based descriptive study of AA among residents of Olmsted County, Minnesota, for the period from 1975 through 1989. MATERIAL AND METHODS After identifying 292 Olmsted County residents first diagnosed with AA during the 15-year study period, we reviewed their complete (inpatient and outpatient) medical records in the community and statistically analyzed the effects of gender and age-group. RESULTS The overall incidence of AA was 20.2 per 100,000 person-years and did not change with time. Rates were similar in the two genders and over all ages, and lifetime risk was estimated at 1.7%. Eighty-seven percent of patients were examined by a dermatologist who diagnosed AA, and 29% of cases were confirmed by biopsy. Most patients had mild or moderate disease, but alopecia totalis or universalis developed at some point during the clinical course in 21 patients. CONCLUSION This study of the incidence and natural history of AA in a community shows that this disorder is fairly common and can be seen at all ages. Although spontaneous resolution is expected in most patients, a small but significant proportion of cases (probably approximately 7%) may evolve into severe and chronic hair loss, which may be psychosocially devastating for affected persons.

The clinicopathologic spectrum of lymphomatoid papulosis: study of 31 cases.

Herein we review the Mayo Clinic experience with thirty-one cases of lymphomatoid papulosis seen since 1965. All patients had chronic, recurrent, and self-healing erythematous papulonodular lesions, which often became pustular, ulcerated, and resolved with scarring. The clinical features often corresponded to those seen in Mucha-Habermann disease; however, the predominant histopathologic feature was an infiltrate composed primarily of atypical lymphoid cells suggestive of malignant lymphoma. In six patients, a lymphoproliferative disorder was eventually diagnosed. There were two cases of mycosis fungoides (stage I), one case of nodular sclerosing Hodgkins disease, and three cases of malignant lymphoma--one diffuse mixed large and small cell type with features of T-immunoblastic type, one diffuse large cell type, and one follicular small cleaved cell type. The clinical course of the lymphomatoid papulosis was unaffected by chemotherapy for the lymphoproliferative disorder. Our data indicate that, with sufficient duration of follow-up, malignant lymphoma may develop in some patients with lymphomatoid papulosis.

Ecthyma gangrenosum: Report of clinical, histopathologic, and bacteriologic aspects of eight cases

The records of eight patients with ecthyma gangrenosum who were treated at the Mayo Clinic between 1970 and 1982 were reviewed. All patients had hematologic disease and had received immunosuppressive drugs. Patients with neutropenia that did not resolve by the end of a course of appropriate antibiotic treatment usually died. Solitary lesions of ecthyma gangrenosum were associated with a better prognosis than were multiple lesions. The typical skin findings of ecthyma gangrenosum usually occurred early and were often incorrectly diagnosed, resulting in time lost during which antibiotic therapy could have been instituted. For patients with ecthyma gangrenosum, a systematic procedure is important to establish the diagnosis early, so that appropriate systemic antibiotic therapy can be initiated.

Netherton's syndrome:Report of a case and review of the literature

A patient with Nethertons syndrome who was followed since birth had previously been diagnosed as having Leiners disease and acrodermatitis enteropathica; the disorder was recognized clinically when the patient was 20 years of age. Therapy with 13-cis-retinoic acid significantly aggravated the ichthyosis and induced increased fragility of the skin. This patient also had an intermittent aminoaciduria with clinical investigations that showed normal renal function. Furthermore, the aminoaciduria resolved spontaneously after the discontinuance of topically applied corticosteroids. The normal results of clinical studies and a review of the literature suggest that the aminoaciduria may have been artifactual because excess absorption of topically applied corticosteroids directly affects the renal tubules and impairs renal reabsorption or enhances the free excretion of amino acids. We review forty-two other cases of Nethertons syndrome in the literature and reaffirm the significance of associated ichthyosis, atopy, trichorrhexis invaginata, and other findings in this unique syndrome.

T-cell receptor gene rearrangement analysis: cutaneous T cell lymphoma, peripheral T cell lymphoma, and premalignant and benign cutaneous lymphoproliferative disorders.

T-cell receptor gene rearrangement analysis is a useful technique to detect clonality and determine lineage of lymphoid neoplasms. We examined 103 patients with mycosis fungoides, Sézary syndrome, peripheral T cell lymphoma, potentially malignant lymphoproliferative disorders including pre-Sézary syndrome, large plaque parapsoriasis, lymphomatoid papulosis and follicular mucinosis, and various benign inflammatory infiltrates. A clonal rearrangement was detected in skin samples in 20 of 24 patients with mycosis fungoides and in peripheral blood samples in 19 of 21 patients with Sézary syndrome. A clonal population was also detected in seven of eight cases classified as peripheral T cell lymphoma. The potentially malignant dermatoses tended to have clonal rearrangement, with the exception of large plaque parapsoriasis, and further follow-up is needed to correlate clonality with the disease course. These studies demonstrate the value of molecular genetics as an adjunct to morphology in the examination of patients with cutaneous lymphoproliferative disease.

Clinical dose ranging studies with finasteride, a type 2 5α-reductase inhibitor, in men with male pattern hair loss

BACKGROUND Androgenetic alopecia is a common condition of adult men. Finasteride, a type 2 5alpha-reductase inhibitor, decreases the formation of dihydrotestosterone from testosterone. OBJECTIVE Two separate clinical studies were conducted to establish the optimal dose of finasteride in men with this condition. METHODS Men from 18 to 36 years of age with moderate vertex male pattern hair loss received finasteride 5, 1, 0.2, or 0.01 mg/day or placebo based on random assignment. Efficacy was determined by scalp hair counts, patient self-assessment, investigator assessment, and assessment of clinical photographs. Safety was assessed by clinical and laboratory measurements and by analysis of adverse experiences. RESULTS Efficacy was demonstrated for all end points for finasteride at doses of 0.2 mg/day or higher, with 1 and 5 mg demonstrating similar efficacy that was superior to lower doses. Efficacy of the 0.01 mg dose was similar to placebo. No significant safety issues were identified in the trials. CONCLUSION Finasteride 1 mg/day is the optimal dose for the treatment of men with male pattern hair loss and was subsequently identified for further clinical development.

Detection of HSV-specific DNA in biopsy tissue of patients with erythema multiforme by polymerase chain reaction

Formalin‐fixed paraffin‐embedded skin biopsies of lesions of erythema multiforme (EM) from 32 patients and 13 controls were examined for the presence of herpes simplex virus (HSV) by polymerase chain reaction (PCR) and for histological findings by direct immunofluorescence and staining with haematoxylin and eosin. HSV‐specific DNA was detected in 23 (72%) patients. A history of recurrent skin rash was present in 59% of the PCR‐positive cases, while 55% had had suspected HSV infections. Only two PCR‐positive specimens were found in patients without a history of recurrent rash and/or previous oral lesions. One biopsy was positive for HSV by conventional cell cultures. There was no significant difference in histology between HSV‐related and HSV‐negative cases of EM. In the 13 control specimens [bullous pemphigoid (3), dermatitis herpetiformis (2), lichen planus (1), aphthous ulcer (1), fixed‐drug eruption (1), varicella‐zoster (1), hypereosinophilic syndrome (1), photocontact dermatitis (1), contact dermatitis (1), and cellulitis (1)], no HSV‐DNA was detected.

Incidence of skin cancers in patients with atopic dermatitis treated with coal tar: A 25-year follow-up study

Coal tar ointments have been used for many years in the treatment of chronic eczematoid dermatitis. The possible carcinogenic effect of coal tar ointment and ultraviolet light (Goeckerman regimen), considered individually or in combination, has been of concern to physicians. A 25-year follow-up study was completed on 426 patients with atopic dermatitis and neurodermatitis who were hospitalized and treated with coal tar ointments and ultraviolet light at the Mayo Clinic from 1950 through 1954. These results were compared with those from a similar study that examined patients with psoriasis who also were treated with coal tar ointments and ultraviolet light. The results of this study suggest that the incidence of skin cancer is not significantly increased above the expected incidence for selected populations of the United States. This study offers reassurance about the relatively small risk of skin cancer developing in patients with atopic dermatitis and neurodermatitis treated with coal tar ointments and ultraviolet light (Goeckerman regimen).

Cutaneous carcinosarcoma: adnexal vs. epidermal types define high‐ and low‐risk tumors. Results of a meta‐analysis

Objective:  We report four cases of cutaneous carcinosarcoma (CS) and perform a meta‐analysis of the cutaneous CS literature.

Malignancy‐associated multicentric reticulohistiocytosis: a clinical, histological and immunophenotypic study

The clinical, histopathological, and immunophenotypic characteristics of four cases of malignancy associated multicentric reticulohistiocytosis (MMR) and one case each of diffuse cutaneous reticulohistiocytosis (OCR) and isolated reticulohistiocytoma (IR). are reviewed. In all four cases of MMR the cutaneous lesions and joint manifestations were judged to be concurrent with the diagnosis of malignancy. Malignancies observed included one case each of pancreatic adenocarcinoma, squamous cell carcinoma of the lung, metastatic melanoma and intraperitoneal grade 4 mucinous adenocarcinoma of uncertain origin.