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.

Immunopathology of oral mucosal inflammatory diseases.

Many inflammatory conditions of oral mucous membranes are the end result of altered immune reactivity. Lesions from these disorders, as well as a wide variety of infectious, irritant, and idiopathic conditions, appear clinically similar in the mouth and are difficult to differentiate. New and improved immunologic laboratory techniques have helped to further our understanding of the pathogenesis of many of these disorders and have proved to be valuable in clarifying the diagnosis underlying many of these difficult dermatoses.

Henoch-Schönlein vasculitis: direct immunofluorescence study of uninvolved skin.

Henoch-Schönlein purpura is a multisystem disease believed to be a consequence of entrapment of circulating IgA-containing immune complexes in blood vessel walls throughout the skin, kidney, and gastrointestinal tract. In this direct immunofluorescence study, twenty-five skin biopsy specimens from twenty patients with Henoch-Schönlein purpura were examined (9 from uninvolved, normal-appearing skin). A distinct stippled pattern of vascular fluorescence was found in 87% of lesion biopsies; 75% of these contained deposits of IgA. In uninvolved skin, seven (78%) showed immunoglobulin in vessel walls and six (67%) contained IgA, suggesting that immune complexes are deposited with equal frequency in normal-appearing and lesional skin of patients with Henoch-Schönlein purpura. Biopsy of uninvolved, rather than of purpuric, skin for direct immunofluorescence studies may be more helpful in confirming the diagnosis of Henoch-Schönlein purpura because tissue morphology is usually of better quality.

Direct immunofluorescence for the study of cutaneous drug eruptions.

Forty-one patients with drug eruptions were retrospectively evaluated to correlate clinical, direct immunofluorescence, and dermatopathologic findings. Forty-six biopsy specimens were obtained for immunofluorescence in addition to specimens for dermatopathologic study. Eighteen patients had blood vessel fluorescence. Fourteen patients had basement membrane zone fluorescence. Eleven patients had dermatopathologic evidence of lichenoid changes; 8 of these had basement membrane zone fluorescence. Triamterene-hydrochlorothiazide caused the most eruptions (six patients). Positive direct immunofluorescence findings may aid the diagnosis of drug eruptions, especially in the presence of a nonspecific clinical picture or histopathologic findings.

Nodular lymphoid disease of the head and neck: lymphocytoma cutis, benign lymphocytic infiltrate of Jessner, and their distinction from malignant lymphoma.

Skin biopsy specimens from six patients with nodular lymphoid disease of the head and neck were studied by routine histology, direct immunofluorescence microscopy, and leukocyte monoclonal antibodies to T and B cell subsets and monocytes. Initially, these lesions were clinically considered to be benign lymphocytic infiltrates of Jessner, lymphocytoma, or lymphoma. Direct immunofluorescence was negative or showed nonspecific staining in all four patients in whom it was performed. Leukocyte monoclonal antibody stains revealed two distinct patterns of lymphocytes. Lymphocytoma was represented by nodular masses of B lymphocytes with peripheral and intervening zones of T cells. The second pattern consisted of solid nodular masses of T lymphocytes occupying the dermis and subcutaneous tissue. In the specimens interpreted as benign lymphocytic infiltration, the T cells were composed equally of helper and suppressor cells.

Clinical and histopathologic spectrum of necrotizing vasculitis. Report of findings in 101 cases.

Clinical and histopathologic features of 101 cases of necrotizing vasculitis were selected on the basis of the following histopathologic criteria: fibrinoid necrosis of blood vessel walls, endothelial cell hyperplasia, and an infiltrate within and around the blood vessel walls predominantly of polymorphonuclear leukocytes. There were three clinical patterns of vasculitis: (1) associated with other coexistent disease, (2) associated with known precipitating events, and (3) idiopathic. Two histologic features were particularly notable in view of the clinical findings. First, vasculitis extending deep into the reticular dermis or subcutaneous tissue seemed to be associated more often with systemic disease such as malignancy or connective tissue disease. Second, in biopsy specimens from patients with hypocomplementemia, the inflammatory infiltrate was composed almost exclusively of neutrophils, as compared with the mixed infiltrate seen in normocomplementemic vasculitis.

Pacemaker contact sensitivity.

In a patient who had 4 cardiac pacemakers implanted and removed, pruritus, redness, and swelling of the skin overlying the pacemaker developed at intervals of 6 weeks to 17 months after insertion. Patch testing showed a 2+ reaction to titanium. The positive result of this test, the titanium case of the generator, and the history of multiple local reactions around the generator site pointed toward contact sensitivity to the pacemaker. Although a review of the literature indicates that this problem is rare, it is of extreme importance to the patient with pacemaker contact dermatitis.

Dermal ultrastructure in leprosy.

We studied the ultrastructure of the dermal inflammatory response in 18 patients with leprosy. Biopsy specimens from 14 lepromatous patients, including four with Lucio's phenomenon and four with erythema nodosum leprosum, were compared with biopsy specimens from one borderline lepromatous and three borderline tuberculoid patients. In all, the dermal infiltrate consisted of macrophages, lymphocytes, and mast cells. This infiltrate was predominantly perivascular, and chronic reactive changes were found in the small dermal vessels. The macrophages contained phagocytized organisms within membrane-bound vacuoles and a wide variety of lysosomal residual dense bodies. Intraendothelial organisms were occasionally seen, especially in biopsy specimens from the patients with Lucio's phenomenon. The greatest number of mast cells were also seen in the infiltrate in those cases. The frequent close association of macrophages with lymphocytes and mast cells suggests an interrelationship between these cells that appears typical of the host response to leprosy.

Ultrastructure of the dermal microvasculature in leprosy.

Infection with M. leprae may lead to the presence of the organism within the dermal vascular endothelium, a phenomenon most pronounced in lepromatous leprosy. In order to study the ultrastructural features of the dermal microvasculature in leprosy, biopsies from 18 patients with lepromatous (14), borderline lepromatous (1) and borderline tuberculoid (3) leprosy were examined. Four patients with Lucio's phenomenon and four with erythema nodosum leprosum were included. The ultrastructural changes in the dermal microvasculature included endothelial swelling and hypertrophy, increased endothelial and pericytic cytoplasmic processes, and pronounced basal lamina reduplication. Occasional large, pale, endothelial cells with widely dispersed organelles were encountered. Phagocytized, membrane-bound intraendothelial organisms were found, similar in appearance to those within dermal macrophages. The predominantly perivascular dermal inflammatory infiltrate consisted of lymphocytes, macrophages and mast cells. The observed ultrastructural changes in the dermal microvasculature are similar to those previously described in the endoneurial vessels. While reflecting nonspecific responses of the dermal microvasculature in chronic inflammation, the findings support a possible role of the small dermal vessels in the chronic nature of the host's response to infection with M. leprae.

Malignant fibrous histiocytoma, myxoid variant metastatic to the oral cavity. Report of a case and review of the literature.

Primary and metastatic malignant fibrous histiocytoma (MFH) of the oral cavity is exceedingly rare. A review of the literature yielded only nine cases of primary MFH and no reports of MFH metastatic to this area. We report a case of recurrent malignant fibrous histiocytoma of the myxoid type arising in the soft tissue of the lower extremity and metastasizing to the gingiva as the typical storiform fibrous variant.

Neuroblastoma and adrenal morphologic features in anencephalic infants.

At autopsy, an incidental congenital neuroblastoma was found in the adrenal gland of an 8-day-old full-term anencephalic baby. This previously unreported coincidence of neuroblastoma and anencephaly prompted a retrospective study of adrenal morphologic features in 20 anencephalic patients and 20 matched controls. Anencephalic adrenal glands were smaller and the fasciculoreticular zone was thinner (average 32%) than in the normal controls (average 76%). The neural crest-derived cells represented an average of 2% of the total gland area in anencephalic patients and 0.5% in the controls. These findings, statistically significant and compatible with previous studies, verify the deficient development of the fetal cortex in anencephalic patients. Furthermore, the neural component in anencephalic patients may be delayed in its maturation by the abnormal cortex, and thus may be prone to undergo malignant transformation and become a neuroblastoma.