A NEUTROPHILIC REACTION OF SWEET''S SYNDROME TYPE ASSOCIATED WITH THE ORAL CONTRACEPTIVE

A case is presented of a Sweet's syndrome-like eruption in association with the oral contraceptive. A 46 year old caucasian woman developed recurrent episodes of erythematous tender plaques on her trunk six weeks after commencement of the oral contraceptive (OC). Her condition clinically and histologically resembled Sweet's dermatosis. On cessation of the OC there was complete resolution of her lesions and she remains well 12 months later. This is the first report, to our knowledge, of a neutrophilic reaction to the oral contraceptive, and we believe that drugs may be implicated in the aetiology of atypical neutrophilic reactions simulating Sweet's syndrome in patients who are otherwise well.     

RECURRENT NEUTROPHILIC DERMATOSIS OF THE FACE—A VARIANT OF SWEET''S SYNDROME

SUMMARY.— A syndrome which he named acute febrile neutrophilic dermatosis was described by Sweet in 1964.
Two cases are reported which seem to fall into the same category, but showed recurrent involvement of the face only. One of these patients and another similar case in the literature are the only males so far recorded.     

ACUTE FEBRILE NEUTROPHILIC DERMATOSIS (SWEET'S SYNDROME) WITH MYELOID PROLIFERATION

A case of acute febrile neutrophilic dermatosis in a 21-year-old man is presented. Onset of the dermatosis followed sore throat, gastro-enteritis treated with sulphonamides and trimethoprim, and signs of meningism. Accompanying the pronounced neutrophilic leucocytosis and dermal polymorph infiltrate, there was marked myeloblastic proliferation in the bone marrow. While distinction from acute myeloid leukaemia was difficult, there was a rapid response to steroid therapy. The findings emphasize that myeloid proliferation may be a central feature in at least some cases of acute neutrophilic dermatosis.     

ACUTE FEBRILE NEUTROPHILIC DERMATOSIS (SWEET'S SYNDROME)

Background. Sweet's syndrome is well recognized and not infrequently diagnosed in Spain; however, the range of clinical and pathologic expression may not have been fully realized. Methods. We reviewed 30 consecutive Spanish cases of Sweet's syndrome diagnosed in our department from 1979 to 1990, with special attention to clinical and histopathologic findings. Results. Distinctive clinical features in our series included oral mucosa lesions in four patients (13%), development of pathergy phenomenon in one case, concurrent nodular lesions resembling erythema nodosum on the limbs in nine cases (30%), and lung involvement in two patients. Infectious disease and drug treatment were recorded as possible triggering factors of Sweet's syndrome in eight and seven patients respectively. Associated underlying systemic disorders were present in 15 (50%) of our patients. The most frequent associations were hematologic neoplasia in four patients, solid neoplasia in two, and chronic idiopathic inflammatory bowel disease in three patients. Dressler's syndrome and sicca syndrome were found in one patient each. Histopathologic studies of skin biopsy specimens obtained at presentation disclosed typical features of Sweet's syndrome in all cases. Epidermal involvement, with variable degrees of spongiosis, exocytosis of polymorphonuclear leukocytes and keratinocyte necrosis, was a prominent feature in 83% of biopsy specimens. Conclusions. Further characterization of the clinicopathologic spectrum of Sweet's syndrome is necessary as the recognition of the full spectrum of this syndrome will improve our diagnostic abilities and provide a solid clinical basis for prospective studies that allow dissection of the intricate pathomechanisms involved in this fascinating disorder.     

SWEET''S SYNDROME ASSOCIATED WITH ADENOCARCINOMA OF THE PROSTATE

Sweet's syndrome can occur in association with malignancy, in particular acute myeloid leukaemia. It has rarely reported with solid tumours. A case of recurrent Sweet's syndrome in a 75 year old man with metastatic prostatic carcinoma is presented.     

HISTOLOGIC SPECTRUM OF CUTANEOUS INVOLVEMENT IN PATIENTS WITH MYELOGENOUS LEUKEMIA INCLUDING THE NEUTROPHILIC DERMATOSES

Background. Cutaneous manifestations of myeloid leukemia can be specific or nonspecific. The study was designed to determine the prevalence and histologic appearance of cutaneous lesions in patients with myeloid leukemia and various myeloproliferative disorders. Methods. The histologic changes of cutaneous lesions in 52 patients with myelodysplastic syndrome, polycythemia vera, and myeloid, myelomonocytic, or monocytic leukemia are presented in this study. Results. Two types of cellular infiltrates were identified. In the first group, the most common pattern was a diffuse involvement by the leukemic cells through the entire dermis with preservation of a “grenz zone” in the superficial dermis. Two cases exhibited a Kaposi's sarcoma-like pattern, with prominent slit-like blood-filled spaces lined by myeloblasts against a fibrocellular stroma. The second group of lesions was characterized by dense, neutrophilic dermal infiltrates resembling acute neutrophilic dermatosis (Sweet's syndrome) or pyoderma gangrenosum. In two of these cases, scattered immature blast cells admixed with the mature neutrophilic elements were identified. Conclusions. Awareness of these different morphologic features and application of special stains are of value in the evaluation of suspicious cutaneous infiltrates in patients with myeloid leukemia and various myeloproliferative disorders.     

STEVSENS-JOHNSON SYNDROME ASSOCIATED WITH MYCOPLASMA PNEUMONILAE INFECTION

SUMMARY.— Two cases of Stevens-Johnson syndrome associated with pneumonia, in which high titres to Mycoplasma pneumoniae were present, are described. In one case M. pneumoniae was isolated from the sputum.     

DETERMINATION OF HYALURONIC ACID IN THE URINE OF A PATIENT WITH WERNER'S SYNDROME

A 54-year-old Japanese male with Werner's syndrome was reported. We found increased excretion of hyaluronic acid in the urine, although the content and analyses of the glycosaminoglycans in the urine eluted by Dowex-1 column chromatography was shown to be hyaluronic acid by comparison with standard hyaluronic acid. The Sephadex G-200 gel filtration of the 0.5 M NaCl fraction caused separation into two fractions. It was revealed that there was not only an excessive production of hyaluronic acid, but also an excretion of lower molecular weight hyaluronic acid in the urine of this syndrome.     

SYSTEMIC SCLEROSIS ASSOCIATED WITH CARCINOMA OF THE LUNG

SUMMARY.— A case of systemic sclerosis associated with squamous cell carcinoma of the lung is presented. The relationship between malignant disease and systemic sclerosis is briefly discussed. Haemoptysis occurilng in a patient with systemic sclerosis may not be caused by the disease but be due to neoplastic change in the lung, and therefore merits full investigation.