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Biomedical subjects

S A Muller

Publications and source records attributed to S A Muller.

At least 19 recordsLinked to original sources

Malignant lymphoma and leukemia with prominent ulceration: clinicopathologic correlation of 33 cases.

BACKGROUND: The clinical and pathologic findings in patients with malignant lymphomas who presented with prominent cutaneous ulcers are described. OBJECTIVE: Our purpose was to determine the histologic findings, type, and prognosis of lymphomas with cutaneous ulcers. METHODS: Thirty-three patients (20 with cutaneous T-cell lymphomas, 10 with other non-Hodgkin's lymphomas, and 3 with leukemia) were retrospectively studied. RESULTS: All patients had a poor prognosis; 23 died within 9 months after the onset of the ulcers. Patients with non-Hodgkin's lymphoma had a worse prognosis, had a higher incidence of systemic involvement, and more often had ulcers as an initial manifestation of lymphoma than did the patients with cutaneous T-cell lymphoma. The histopathologic findings ranged from a nonspecific inflammatory infiltrate to ulcers with marked adjacent epidermal epidermotropism to ulcers with an angiocentric infiltrate. CONCLUSION: A variety of lymphomas may cause cutaneous ulceration. Adequate sampling of these ulcers is necessary for diagnosis. The average life expectancy after ulcer formation is 21 months.

Adult

Lichen planopilaris: clinical and pathologic study of forty-five patients.

BACKGROUND: We review the findings in a large series of patients with lichen planopilaris. OBJECTIVE: Clinical, histologic, and direct immunofluorescence findings were reviewed in 45 patients. METHODS: Scalp biopsy specimens for routine histologic examination and direct immunofluorescence were reviewed. Clinical data and follow-up were obtained. RESULTS: Women were affected more commonly and had patchy hair loss, with perifollicular erythema, perifollicular spines, and scarring. Half had or developed glabrous skin, mucous membrane, or nail changes typical of lichen planus. Follicular involvement was limited to the infundibulum and isthmus and included lichenoid inflammation and cytoid formation, with few or no changes in interfollicular epidermis. Direct immunofluorescence showed cytoid body staining with anti-IgM and anti-IgA and patchy or linear fibrinogen deposition along the basement membrane zone. The various therapeutic options used were usually unsuccessful. CONCLUSION: To make the correct diagnosis, patients with scarring alopecia should be evaluated histologically and with direct immunofluorescence. They should also be followed up to assess whether lichen planus develops elsewhere.

Adrenal Cortex Hormones

Benign lymphocytic infiltrate of the skin: correlation of clinical and pathologic findings.

In a retrospective study of 137 biopsy specimens of skin from 137 patients (69 men and 68 women) that had been obtained between 1972 and 1989 at our institution and that had perivascular and periappendageal lymphocytic infiltrates characteristic of those described as benign lymphocytic infiltrate (BLI), we determined the specificity of the histologic diagnosis and the correlation with clinical data. The final diagnoses, based on clinical and laboratory data and histologic findings, were BLI (59), possible BLI (7), lupus erythematosus (LE) (12), possible LE (7), procainamide-induced LE (1), insect bites (9), possible insect bites (3), polymorphous light eruption (4), lymphocytoma (4), urticaria (4), and indeterminate or miscellaneous diagnoses (27). BLI is a clinical and histologic syndrome that can be heterogeneous in origin. We recommend careful evaluation to exclude other disorders such as LE, polymorphous light eruption, lymphocytoma, and insect bites. Direct immunofluorescence microscopy and immunophenotypic studies may help distinguish BLI from LE.

Adult

Palisading granulomas caused by infectious diseases.

We recently encountered a palisading granuloma in which an infectious cause was not considered until special stains revealed hyphae and tissue culture revealed organisms of phaeohyphomycosis. A review of more than 2,500 cases of various granulomas revealed 11 cases of palisading-type granuloma caused by infection, including phaeohyphomycosis, nontuberculous mycobacteriosis, papulonecrotic tuberculid, tuberculoid syphiloderm, cat-scratch disease, sporotrichosis, cryptococcosis, and coccidioidomycosis. Infectious disease should be considered in the differential diagnosis of palisading granuloma. Special stains for bacteria, fungi, and acid-fast bacilli should be performed in biopsy specimens that have a palisading granuloma pattern with central necrosis, especially in immunosuppressed patients.

Aged

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.

Adult

Osteoma cutis and hemihypertrophy: a case report.

We report an interesting case of a patient with multiple ipsilateral osteoma cutis lesions and contralateral hemihypertrophy. These two features have not been described together previously. We also review the dermatologic findings described with hemihypertrophy.

Adult

Generalized pustular psoriasis in childhood. Report of thirteen cases.

Generalized pustular psoriasis is rare in children. Less than 100 cases have been reported. We describe 13 children with this type of psoriasis. Seven had acute onset of widespread sterile pustules coalescing into lakes of pus with subsequent exfoliation (the Zumbusch pattern). This usually occurred in infancy and was difficult to control; recurrences developed several times per year. Three had the subacute benign annular pattern. They tended to be older and often had resolution within several years. Three had a mixed pattern with Zumbusch flares preceded by an annular or acral pattern. Most patients had an eruption preceding the generalized pustular psoriasis and often had precipitating factors. Generally, generalized pustular psoriasis has little serious chronic morbidity. The condition in most patients was well controlled with topical therapy. Systemic steroids were not helpful.

Acute Disease

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 Kaposi's 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.

Female

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.

Adolescent

Cutaneous and subcutaneous leiomyosarcoma: a clinicopathological review of 14 cases with reference to antidesmin staining and nuclear DNA patterns studied by flow cytometry.

Among 14 patients (11 female and three male) with cutaneous and subcutaneous leiomyosarcomas, metastatic spread occurred in six of the 13 patients with adequate follow-up. The tumours were classified with regard to tumour grade, number of mitotic figures per 10 high-power fields, reactivity to antidesmin antibodies, and DNA pattern as assessed by flow cytometry. Antidesmin antibody staining was present in only six cases and seemed to be less common in the higher-grade tumours. The DNA pattern was aneuploid in five cases and non-classifiable in one case; all of these cases had metastatic spread. None of the eight remaining diploid cases had metastatic spread. DNA content was a strong predictor of metastatic potential.

Adolescent

Lichen sclerosus et atrophicus in children and young adults.

We performed a retrospective study of 52 children and young adults (average age 18 yrs) with lichen sclerosus et atrophicus. In 56% of patients the eruption was still present after a follow-up of 7.5 years. Younger patients were most likely to show improvement. The histologic appearance of both groups (patients with persistent lesions and those with resolution of lesions) showed classic features of lichen sclerosus et atrophicus: homogenized collagen with an underlying bandlike lymphocytic infiltrate. Epidermal changes such as atrophy, hyperplasia, and follicular plugging were slightly more prominent in patients with persistent lesions. Menarche, pregnancy, and the presence of extragenital lesions had no effect on the prognosis, but since the condition resolved, on average, during adolescence, unknown developmental factors may be implicated.

Adolescent

Generalized pustular psoriasis. A review of 63 cases.

BACKGROUND: Sixty-three patients with generalized pustular psoriasis were hospitalized during a 29-year period. They were classified into four subgroups on the basis of onset and morphologic pattern of disease: acute (von Zumbusch), subacute annular, chronic (acral), and mixed. This division provides a better understanding of the variability of the disease and helps in choosing treatment. OBSERVATIONS: The average age at onset was 50 years; male and female patients were affected about equally. In 11 patients, flares were precipitated by localized infections. Approximately one fourth of the patients had complications; most were superinfections. The average stay in the hospital was 30 days; factors correlating with a long hospitalization were hypocalcemia, female sex, and a previous history of psoriasis vulgaris or pustular psoriasis. CONCLUSIONS: Whereas topical therapy was helpful, systemic medications were often needed. Coal tar, ultraviolet light, and psoralen-ultraviolet A may be effective; however, they must be used with caution, because they may exacerbate the disease.

Acute Disease

Androgenetic alopecia (common baldness).

Alopecia is a common problem affecting the scalp in both men and women. The vast majority of all cases of alopecia are of the androgenetic variety. The clinical and endocrinologic features of this disorder are reviewed. Potential therapies, including surgical and medical modalities, are mentioned.

Aging

Trichotillomania: a histopathologic study in sixty-six patients.

This report reviews the results of scalp biopsies in 66 (38%) of 174 patients with trichotillomania seen at the Mayo Clinic from 1978 through 1988. The most important findings included catagen hairs in 74%, pigment casts in 61%, and traumatized hair bulbs in 21%. Bulbar inflammation and atrophic anagen hairs were not seen. The most characteristic features most often were noted in specimens from areas that had been affected for usually less than 8 weeks. It is important to examine many sections stained with hematoxylin and eosin because only a few sections may show the characteristic changes. The study points out the usefulness of scalp biopsy for making or excluding a diagnosis of trichotillomania.

Adolescent

Incidence of cutaneous T cell lymphoma and other rare skin cancers in a defined population.

Between 1970 and 1984 in Rochester, Minnesota, rare skin cancers developed in 15 local residents: cutaneous T cell lymphoma (six subjects), dermatofibrosarcoma protuberans (four), adenocarcinoma of sweat glands (two), Merkel cell carcinoma (one), liposarcoma (one), and extramammary Paget's disease (one). These cases were identified through a unique computerized retrieval system that is maintained at the Mayo Clinic for the population of Rochester, Minnesota. The annual incidences of these cancers in the Rochester population were 0.9, 0.5, 0.3, 0.2, 0.2, and 0.2 per 100,000 residents, respectively (standardized to 1980 U.S. population). To our knowledge, this is the first report of the incidences of these rare skin cancers in a well-defined population.

Adenocarcinoma

Multicentric cloacogenic carcinoma: report of a case with anogenital pruritus at presentation.

A case of multicentric cloacogenic carcinoma of the perianal skin and vulva in a 79-year-old woman is presented, and the embryologic basis for the multicentricity is discussed. Histologically, cloacogenic carcinoma can be differentiated from other small cell neoplasms that affect the area. Cloacogenic carcinoma should be considered a rare cause of anogenital pruritus. It is important to perform an early biopsy of anogenital lesions that do not respond to conventional therapy.

Aged

Cyclosporine in the treatment of cutaneous T cell lymphoma.

We present our observations on the use of cyclosporine in the treatment of three patients with mycosis fungoides and two patients with Sézary syndrome. The patients with mycosis fungoides showed mild improvement of short duration. One patient with Sézary syndrome had significant improvement during 2 years of treatment. It is unlikely that cyclosporine alone will be sufficient therapy for cutaneous T cell lymphoma. However, lower doses in conjunction with other immunomodulators may minimize potential side effects and lead to longer lasting clinical improvement.

Aged