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Biomedical subjects

J A Doyle

Publications and source records attributed to J A Doyle.

At least 37 records · Page 2Linked to original sources

Sclerodermoid changes of porphyria cutanea tarda: possible relationship to urinary uroporphyrin levels.

From 1950 to 1982, fifteen patients were seen at the Mayo Clinic with a diagnosis of sclerodermoid changes of porphyria cutanea tarda. Fourteen patients had changes similar to scleroderma limited to the skin, and one patient had scleroderma-like skin changes accompanied by visceral abnormalities. Both light-exposed and unexposed areas of the body were affected. Areas of involvement included the chest, the V-shaped area of the neck, and the back, face, and shoulders. In six patients, morpheaform changes represented the presenting cutaneous sign of porphyria cutanea tarda. Follow-up examination, after treatment that included abstinence from alcohol and phlebotomy, revealed that the sclerodermoid skin changes had disappeared in six patients and improved in four. Generally, the degree of improvement of the sclerodermoid changes was proportional to the reduction of the urinary uroporphyrin levels toward normal (p = 0.02).

Adult↗

Genital Paget's disease and urinary tract malignancy.

Eight cases of Paget's disease of genital mucosa with malignancy of the lower urinary tract are described. In five it was apparent that there was concurrence of two separate malignancies. In one patient with long-standing Paget's disease of the genital mucosa, carcinoma of the urethra and bladder developed subsequently in continuity with the genital lesion; the two lesions were indistinguishable histologically, suggesting extension of the Paget's disease into urothelium. In two patients with bladder malignancy, there was histologic evidence of outward pagetoid extension of this process along urothelium and onto the genital mucosa. The significance of genital Paget's disease is discussed in the light of these findings, and the possible origins of Paget cells within the epidermis are reviewed.

Aged↗

Hematologic disease in scleroderma syndromes.

Review of 4 430 patients with a diagnosis of scleroderma, morphea, or eosinophilic fasciitis has revealed 16 patients with one of a variety of serious hematologic diseases. Although it is not possible to comment on the exact relationship between the dermatologic disease and the hematologic disorder, in seven patients there appeared to be a close temporal relationship between the onset of the two conditions. The development of a serious hematologic disease, especially a lymphoproliferative process, may rarely be seen not only in patients with eosinophilic fasciitis but also in those with systemic or localized scleroderma.

Adult↗

Gamma heavy-chain disease involving upper airway.

Gamma heavy-chain disease is a rare disorder. It is a plasma cell dyscrasia, with the patient's age at onset being 9 to 87 years. Identification of IgG heavy chains in the serum and urine is diagnostic. Histopathologic features are nonspecific. Although the clinical presentation is diverse, many signs and symptoms lead to early otorhinolaryngologic consultation. For this reason gamma-heavy-chain disease should be considered in the differential diagnosis of inflammatory lesions of the upper aerodigestive tract.

Heavy Chain Disease↗

Treatment of psoriasis with N-phosphonacetyl-L-aspartate.

Seven patients with severe psoriasis were treated with the aspartate carbamylase inhibitor N-phosphonacetyl-L-aspartate (PALA). Three patients showed a definite improvement lasting up to 3 months after each course of therapy. Three other patients showed more limited responses of shorter duration, and one patient experienced progressive worsening of his psoriasis while on the study. Psoriatic arthritis did not appear to be improved by the PALA therapy. Side effects were usually mild and limited to skin irritation and diarrhea. The development of alternate schedules of administration may lead to a more useful treatment response in patients with severe psoriasis.

Adult↗

Cowden's disease with associated malignant melanoma.

Cowden's disease (multiple hamartoma syndrome), a rare genodermatosis, is inherited in an autosomal-dominant genetic pattern. It is characterized by mesodermal and epithelial hamartomas appearing as verrucous papules occurring predominantly over the central portion of the face, with perioral and acral papular lesions and papillomatosis of the lips, gingiva, and tongue extending through the entire length of the gastrointestinal tract. These skin lesions have been found to be associated with a number of benign and malignant tumors involving the breast, thyroid, skin, ovaries, adipose tissue, and gastrointestinal tract. The authors report the second case of Cowden's disease associated with malignant melanoma and emphasize the need for a high index of suspicion for related benign or malignant tumors in these cases.

Female↗

Thalidomide treatment of prurigo nodularis.

Four patients with classic recalcitrant prurigo nodularis had symptomatic and physical responses to thalidomide with remissions. Three of the four patients had increased IgE levels that decreased during therapy. In two patients, short-term treatment (2 to 3 months) was not sufficient to produce remission, but retreatment was effective. Two patients had long-term remission with more than 6 months of treatment. No significant side effects occurred.

B-Lymphocytes↗

Eosinophilic fasciitis: extracutaneous manifestations and associations.

Eosinophilic fasciitis (EF) is diagnosed by the presence of characteristic alterations in the skin, fascia, and peripheral blood. Direct extension of the fascial inflammation may lead to involvement of the tendons, synovium, and muscle. Visceral changes similar to those seen in localized and systemic scleroderma may occur in the esophagus, lung and heart. Autoimmune phenomena may account for the thyroid and hematologic diseases which may also develop in EF.

Eosinophilia↗

Extracutaneous sporotrichosis.

The typical infection with Sporothrix schenckii is characterized by superficial cutaneous nodules occurring along the lines of lymphatic drainage of the limbs. Review of the medical records at the Mayo Clinic from 1937 to the present disclosed 58 patients with sporotrichosis. Eleven patients had evidence of extracutaneous infection. Eight of the patients were men whose ages ranged from 25 to 71 years; the ages of the three women were 54, 64, and 67. Seven patients had predominantly joint involvement, with the knee and wrist joints being most often infected. Other sites of infection included the mandible and ethmoid sinuses. Three patients had disseminated systemic infection, and one patient died within a year of the initial diagnosis. Nine patients had been in good health before the infection, although five patients were taking systemic corticosteroids before their infection was diagnosed. Therapies included supersaturated potassium iodide, amphotericin B, and 2-hydroxystilbamidine isethionate. Although most commonly seen as a cutaneous disease, sporotrichosis is a potentially disseminated infection with life-threatening consequences.

Adult↗

Carpal tunnel syndrome in cutaneous connective tissue disease: generalized morphea, lichen sclerosus, fasciitis, discoid lupus erythematosus, and lupus panniculitis.

Carpal tunnel syndrome developed concurrently with cutaneous connective tissue disease in five patients. The skin lesions varied from morphea, lichen sclerosus, fasciitis, and discoid lupus erythematosus to lupus panniculitis. Variable and transitory serologic and direct immunofluorescent findings were noted. In two cases, surgical specimens from carpal tunnel operations had lymphoid nodules. Treatment of the cutaneous connective tissue syndrome (antimalarials, four cases; corticosteroids, two cases) brought healing of the carpal tunnel syndrome as well as improvement of the skin lesions.

Adult↗

Chrysiasis resulting from gold therapy in rheumatoid arthritis: identification of gold by x-ray microanalysis.

A patient with blue-gray discoloration of the skin of her face, neck, and hands is described. She was a patient with rheumatoid arthritis who had received a large total cumulative dose of gold. Light microscopy of skin biopsy tissue from the forehead revealed deposits of heavy metal in macrophages. On electron microscopy, the particles were found in the lysosomes of the cell. X-ray microanalysis confirmed the presence of gold. This condition, which is called chrysiasis, may be incorrectly diagnosed as cyanosis.

Arthritis, Rheumatoid↗

Cutaneous and subcutaneous inflammatory sclerosis syndromes.

Systemic scleroderma and localized scleroderma (morphea) show comparable changes on skin biopsy specimens, and a distinction has often been made on the basis of Raynaud's phenomenon, organ involvement, and laboratory abnormalities characteristically seen in systemic scleroderma. Critical evaluation not only of patients with localized scleroderma but also of those with eosinophilic fasciitis, morphea profunda, and acral pansclerotic morphea has disclosed Raynaud's phenomenon, organ involvement, and laboratory abnormalities typical of systemic scleroderma in a small percentage of patients. Histologically, all five conditions show similar inflammation and sclerosis of the skin, the primary difference being the depth at which these changes occur. These conditions may possibly be related, and the clinical and laboratory differences observed may result from variations in depth, nature, and intensity of the cutaneous and subcutaneous inflammatory sclerosis.

Child↗

Lymphoma-associated papulosis: lymphomatoid papulosis associated with lymphoma.

Lymphomatoid papulosis is a condition characterized by the continuing appearance of self-healing inflammatory papulonodules which histologically show atypical cells simulating a malignant process. We describe two patients who had features typical of lymphomatoid papulosis in whom a lymphoreticular malignancy developed. The first patient developed Hodgkin's disease 30 years after the onset of lesions that resembled lymphomatoid papulosis, and the second patient developed a lymphocytic lymphoma 11 months after the appearance of the self-healing papules. While most patients with lymphomatoid papulosis have a benign protracted course, "lymphoma-associated papulosis" represents the malignant end of this spectrum, in which lymphomatoid papulosis is associated with a lymphoma. All patients with lymphomatoid papulosis require close, long-term follow-up.

Biopsy↗

The therapeutic uses of topical vitamin A acid.

Topical vitamin A acid (VAA) has various mechanisms of action which may be responsible for its therapeutic success in many different disorders. Although the absorption, metabolism, and excretion of VAA are not completely understood, VAA appears to remain mainly on the skin surface. The question of carcinogenicity is unresolved, and more research is needed to clarify this problem. This article reviews the literature regarding the therapeutic uses of VAA and summarizes various investigators' experiences with VAA.

Acne Vulgaris↗