Intestinal heterotopia: an unusual cause of vulval ulceration. Case report.
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Biomedical subjects
Publications and source records attributed to S Kossard.
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A 45-year-old woman developed simultaneously a form of palmar and digital fibromatosis and an unusual polyarticular disorder with painful capsular contraction. The features of the bilateral shoulder involvement were consistent with the adhesive capsulitis/frozen shoulder syndrome. The other affected joints were painfully contracted and tender but without signs of inflammation in synovial fluid (knee) or associated abnormalities in hematologic status. Histologically, there was extensive fibrosis with increased numbers of fibroblasts, dilated blood vessels and scant perivascular lymphocytic infiltration. The clinical and pathological features were similar to cases described as palmar fasciitis and polyarthritis occurring in association with malignant tumors and with antituberculous chemotherapy. Several months after removal of an endometrial cyst of the ovary and after symptomatic treatment, the palmar fibromatosis and polyarticular disorder had almost completely resolved.
In ten cases of neurotropic melanoma, patients presented with nodules composed of amelanotic, deeply infiltrating neuroid fascicles. Only four cases were clinically suggestive of melanoma. In eight of the tumors, a precursor lesion was found histologically and provided a major clue to the diagnosis. In seven cases, the dysplastic precursor was lentiginous, while a superficial spreading pattern was present in one. Initial surgery was often inadequate because of the difficulty in defining tumor margins and the lack of pigment. In seven of the tumors, S100 protein was demonstrated within the invasive spindle cell component by the immunoperoxidase method. This finding was negative in three cases, two of which showed positive staining of the precursor and nerve filaments, indicating that the absence of S100 protein cannot be used as an exclusion criterion for neurotropic melanoma.
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Nine patients developed multiple areas of eczema surrounding centrally located pigmented nevi. There was no significant history of atopy or evidence of external contact factors to account for the reaction. The eczema did not appear to influence the central melanocytic nevi, which persisted after resolution of the inflammation. The pathogenesis of this striking phenomenon remains unclear but differs from that associated with classical halo nevi.
Bindii dermatitis is a distinctive eruption occurring in some Australian children during spring and early summer and characterized by discrete erythematous papules with puncta, concentrated over the palms, soles, and knees. In three affected individuals (but not in thirteen controls), pricking the skin with bindii seed produced an erythematous, indurated papule that appeared within 48 hours of intradermal pricking and simulated the primary lesion. Pathogenesis may involve an allergic reaction with a retained depot of antigen rather than a nonspecific foreign body injury reaction.
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Progressive cutaneous papulonodular non-X histiocytosis of the face and proximal parts of the extremities developed in a 28-year-old woman. Histologic study showed masses of benign histiocytes with foci of lymphocytes. Occasional cells contained diastase-resistant cytoplasmic polysaccharide that stained positive with PAS stain. No iron, fat, or Touton or other giant cells were present. Ultrastructural study disclosed laminar bodies but no Langerhans' cells or Langerhans' granules. The process responded to low-dose intravenous vinblastine sulfate treatment.
The question of whether epidermolysis bullosa acquisita (EBA) is a distinct entity prompted a review of the Mayo Clinic experience. Twelve patients with this diagnosis were seen from 1955 through 1979, and their records were reviewed. Particular attention was directed to immunofluorescence microscopy (IF), which had been performed in seven patients. Direct IF was positive in seven patients, and indirect IF was positive in four patients. Although many cases of EBA may remain distinct, we believe that the morphologic appearance known as EBA may represent a final common pathway of disease expression in a heterogeneous group of patients with a variety of more specific diseases and that trauma serves as a localizing factor. Our findings indicate that cicatricial pemphigoid probably is the most common specific diagnosis but that all cases of EBA are not represented by this diagnosis. Furthermore, we conclude that when IF is performed, exclusion of other bullous diseases, which is a key criterion for the diagnosis of EBA, is more difficult than previously recognized.
Eight patients had multiple xanthomatous plaques and subcutaneous nodules that had a predilection for the periorbital area, flexures, and trunk and that tended to ulcerate. Skin biopsy specimens showed a combination of xanthogranulomatous nodules with necrobiosis. All patients had an accompanying dysproteinemia, which was a monoclonal IgG paraprotein in six. Hyperlipidemia, low serum complement, and cryoglobulinemia were variable features. Five patients had leukopenia. Bone marrow examination showed myeloma in two patients, a lymphoproliferative process in one, and some atypical plasma cells in two. Cutaneous necrobiotic xanthogranuloma is a distinctive histologic pattern most frequently related to plasma cell dyscrasias, and it should be distinguished from normolipemic plane xanthoma and other necrobiotic granulomas.
Nine biopsy specimens taken from the scalps of five children and four adults with subcutaneous necrobiotic granulomas were reviewed. This histopathologic features were similar in both groups. Four of the five children presented clinically with multiple, firm, bound-down subcutaneous nodules limited to the scalp but no associated systemic disease. In addition to the scalp nodules, one child had typical cutaneous lesions of granuloma annulare over the acral areas. Two of the adult patients, however, had severe rheumatoid arthritis with multiple rheumatoid nodules on the arms in addition to the scalp nodules; one of them had rheumatoid vasculitis. Another adult patient had allergic granulomatosis,and one had granuloma annulare lesions involving the scalp, face, and left ankle. Excision biopsy specimens should be taken in all patients--both children and adults--with multiple, deep, bound-down nodules of the scalp. If the specimen shows subcutaneous necrobiotic granuloma, probably no treatment is indicated in children because, in our experience, there is no associated systemic disease. However, in the adult patient with similar scalp lesions, the possibility of systemic disease should be explored.
Forty-one (8%) of 500 randomly selected biopsy specimens in verruca vulgaris showed evidence of inflammation. The inflammation was lymphocytic and lichenoid in 21 specimens and was associated with papillary hemorrhage and infarction in 23. Clinical review indicated that patients of all ages were represented but that there was a predominance of verrucae of the extremities in older patients. The data suggest the possibility of a distinct solitary acral verruca in the elderly.
A 9-year-old girl had a widespread papular eruption that was histologically characterized by benign histiocytes that were acid phosphatase-positive, but that lacked Langerhans' granules ultrastructurally. New lesions had continued to develop since the patient was 3 months old, and individual lesions had resolved over a period of two to three months. The histopathologic, histochemical, and ultrastructural findings in this patient were similar to those previously described for eruptive histiocytomas in adults. Papular histiocytomas in childhood may be found in histiocytosis X, reticulohistiocytoma, xanthogranuloma, xanthoma disseminatum, or eruptive histiocytoma. These diseases may be differentiated on the basis of differing histiocytic populations and the clinical courses of the lesions in each disorder.
Multiple subcutaneous nodules and recurrent flu-like episodes developed in a 60-year-old woman after she had received local intramuscular injections of procaine povidone for a seven-year period. A skin biopsy specimen showed distinctive gray-blue material in the histiocytes in sections stained with hematoxylin-eosin. Povidone is an inert substance that may accumulate in multiple organs, including the skin. Its presence can be verified by its affinity for special stains or by spectrophotometric analysis. In our patient, ultrastructural study showed amorphous inrahistiocytic deposits in the tissues. -revious reports have indicated that this substance may eventually accumulate in noninjected skin sites and may lead to pulmonary and arthritic symptoms in some patients, but no other patients, to our knowledge, have had complications associated with fever.
In a patient with primary inflammatory macular atrophy, immunofluorescence studies disclosed both C'3 and IgM in a granular and fibrillar pattern in the dermis between the coagen fibers and at the basement membrane zone. Localization of C'3 to autofluorescent elastic fibers was demonstrated. Ultrastructural examination showed the presence of activated macrophages enveloping fragmented elastic fibers. Immunologic mechanisms may play a participatory role in some forms of macular atrophy.