John A. Doyle

Prurigo Nodularis: A Reappraisal of the Clinical and Histologic Features

Study of 14 patients with prurigo nodularis revealed that clinically the lesions were multiple, verrucous, and discrete and were set in normal‐appearing skin. Microscopically, the lesions showed a vascular acanthomatous mass. The dermal nerves showed a tendency toward hyperplasia in parallel with the epidermal and vascular changes, but no evidence of neuroma formation was seen. Electron microscopic examination showed disorganization of the normal pattern of the myelin sheaths and axons of the dermal papillary nerves. The presence of epidermal mast cells was noted in the biopsy specimens of prurigo nodules from eight of the 14 patients.

Cutaneous cryptococcosis simulating pyoderma gangrenosum

A 33-year-old white man with a history of chronic ulcerative colitis presented with multiple cutaneous ulcers and an indurated cellulitic area on his right thigh. Clinically, the ulcers were considered to represent pyoderma gangrenosum. However, tissue biopsy revealed copious yeast forms that were identified as Cryptococcus neoformans. The indurated area on the right thigh later ulcerated, and tissue culture of this area also revealed Cryptococcus. This case illustrates that when pyoderma gangrenosum is diagnosed, the possibility of deep fungal infection should be considered and excluded by appropriate studies.

Hyperglucagonaemia and necrolytic migratory erythema in cirrhosis--possible pseudoglucagonoma syndrome.

Necrolytic migratory erythema is the distinctive cutaneous eruption seen with glucagon‐producing tumours of the pancreas. Recognition of this eruption is important because it may lead to the early diagnosis of a glucagonoma. Recently, we saw a patient who had necrolytic migratory erythema, hyperglucagonaemia, and cirrhosis of the liver with no evidence of pancreatic tumour while alive or at autopsy. Serum glucagon levels during the period of observation and during an oral glucose tolerance test suggested that the hyperglucagonaemia was not due to an occult glucagon‐producing tumour but may have been the result of advanced hepatic cirrhosis.

Genital Paget's disease and urinary tract malignancy

Eight cases of Pagets 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 Pagets 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 Pagets 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 Pagets disease is discussed in the light of these findings, and the possible origins of Paget cells within the epidermis are reviewed.

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).

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.