Biomedical subjects
Publications and source records attributed to K Soltani.
Seckel's syndrome consists of multiple congenital anomalies, including bird-headed dwarfism, mental deficiency, and skeletal and ophthalmic defects. We report a patient with this syndrome who demonstrated pigmentary changes, including streaks of brown pigmentation on the neck, groin, and axillae. Histologic examination revealed pigment incontinence.
Angioimmunoblastic lymphadenopathy (AIL) is an uncommon immunoproliferative disorder with a presentation similar to malignant lymphoma but with a benign histopathologic picture. We report a case of a 49-year-old woman with AIL who manifested a pruritic maculopapular eruption as her presenting complaint and whose disease pursued an aggressive clinical course. Forty-four percent of patients with AIL experience a nonspecific dermatitis that in general is maculopapular and precedes other clinical symptoms by at least several weeks. AIL should be included in the differential diagnosis of any maculopapular eruption of unknown etiology accompanied by lymphadenopathy.
A patient with erythema multiforme developed many nevocytic nevi, including a large cluster of such lesions at the sites of healed blisters. The development of eruptive nevocytic nevi in this pattern suggests that cutaneous injury plays a role in their pathogenesis.
Bullous eruption of diabetes is a cutaneous sign of diabetes mellitus. Patients with this disorder manifest a sudden onset of intraepidermal or subepidermal bullae, which are primarily confined to the extremities and heal within several weeks without scarring. Our patient had severe diabetes and experienced two episodes of bullae associated with intense, ultraviolet light exposure. Negative immunofluorescence, early disappearance of anchoring filaments and half-desmosomes between cell membrane and basal lamina, and the absence of urinary uroporphyrins separate this entity from certain similar-appearing conditions. Cation imbalance, precipitated by renal failure, could be a possible causal factor.
There is increasing evidence that, as in systemic lupus erythematosus (SLE), deposition of immune complexes plays a role in the pathogenesis of dermatitis herpetiformis (DH). Dermatitis herpetiformis and SLE were diagnosed in a 15-year-old girl with Marfan's syndrome who died of cardiac tamponade secondary to cystic medial necrosis of the ascending aorta. The concurrence of these diseases suggests that predisposition to immune-mediated disorders may be associated with the expression of multiple clinical entities.
Shulman's syndrome includes fasciitis of rapid onset, sclerodermoid skin changes, absence of Raynaud's phenomenon, absence of visceral manifestations of progressive systemic sclerosis, hypergammaglobulinemia, transient eosinophilia, and a dramatic response to systemic corticosteroid therapy. Although this syndrome may be a variant of scleroderma, its dramatic responsiveness to corticosteroid therapy is a particularly distinguishing feature.
By direct immunofluorescence, with use of fluorescein-labeled sonified Treponema pallidum, specific antibodies were detected in the tissue infiltrates of cutaneous syphilomas in rabbits. Specimens from cutaneous and mucosal inflammatory lesions induced by intradermal injection of a keratinous substance from an epidermal cyst served as controls. Granular fluorescence was detected in the dermis of 11 of the 12 syphiloma specimens and corresponded to areas of heavy plasma cell infiltrates, and some fluorescence was found directly on plasma cells identified by subsequent staining with hematoxylin and eosin. This fluorescence could be blocked by preabsorption. Control slides did not show any fluorescence. Immunopathologic techniques using labeled antigens may be of diagnostic value in syphilis and other infectious disorders which feature specific infiltrates.
Punch biopsies were examined by indirect immunofluorescence for immune complex deposits containing C-type viral antigen. Antisera specific for immunoglobulins and HEL-12 virus mediated fluorescence at the dermal-epidermal junction and in vessel walls of 16 of 16 biopsies involved skin from patients with systemic lupus erythematosus (SLE). Preimmune sera did not mediate fluorescence and gradient purified HEL-12 virus, simian sarcoma virus and baboon endogenous virus but not Rous sarcoma virus blocked the reaction of anti-HEL-12 virus serum with SLE tissue. Ten biopsies from uninvolved skin of the patients with SLE did not react with the antiviral serum, nor did tissue from 9 patients with discoid lupus erythematosus, psoriasis, bullous pemphigoid or normal skin. These data support the hypothesis that C-type viral immune complexes participate in the pathogenesis of SLE.
Human alpha-fetoprotein (HAFP) was demonstrated in clusters of atypical cells of an extensive Bowen's disease involving the pubic area. Frozen sections were studied by indirect immunofluorescence using monospecific rabbit anti-HAFP antiserum or purified rabbit anti-HAFP antibody followed by staining with fluorescein isothiocyanate (FITC)-labeled goat anti-rabbit gamma-globulin and by a direct technique using FITC-labeled rabbit anti-HAFP Fab2 fragments. Sections of fetal liver served as positive controls and sections of adult human skin and liver were used as negative controls. Preabsorption of the anti-HAFP antibodies with purified HAFP blocked the fluorescence. This finding adds Bowen's disease to the list of premalignant disorders which possess HAFP as an oncofetal marker of their malignant potential. HAFP might possibly enhance malignant behavior through interference with cellular immunosurveillance mechanisms.
Myasthenia gravis in a woman was followed by the development of severe erosive lichen planus and thymoma. Lichen planus is not ordinarily associated with other cutaneous or systemic disorders. The reported immunologic findings in some patients with lichen planus and especially the occasional association of lichen planus with certain disorders linked with immunologic disturbances suggest an autoimmune pathogenesis for this disorder.
Specific antitreponemal antibodies have been demonstrated by immunofluorescence techniques in the lymphoplasmocytic infiltrates which characterize early symphilitic lesions. A purified suspension of Nichols strain Treponema pallidum was sonified and labeled with fluorescein isothiocyanate and applied to cryostat sections of 12 biopsy specimens from the cutaneous lesions of 11 patients with proven secondary syphilis, using a modified direct immunofluorescence procedure. Specimens from various inflammatory dermatoses processed similarly served as controls. Granular fluorescence was noted in the dermis in 9 of the 12 specimens corresponding to areas of heavy plasma cell infiltration and some fluorescence was found directly on plasma cells which were identified by subsequent hematoxylin and eosin staining. This fluorescence could be blocked by prior incubation of the sections with unlabeled sonified treponemal suspension. Control slides did not reveal any fluorescence. The use of labeled treponemal antigen may aid the tissue diagnosis of early syphilitic lesions which can mimic a variety of dermatological disorders.
In fetal life the development of bullous pemphigoid antigen is preceded by a morphologically detectable basement membrane zone (BMZ). The present study investigated the possibility that similar morphologic and antigenic changes of the BMZ occur in short term organ culture of human skin. Twenty small pieces from each of four neonatal foreskin specimens were cultured under standard conditions. At regular intervals two pieces from each specimen were removed. One piece was frozen and cryostat sections were processed for indirect immunofluorescence using a patient's serum that was proven to have circulating pemphigoid IgG antibodies. Additional sections were stained with periodic acid-Schiff and hematoxylin and eosin. The other piece was processed for electron microscopic examination. Morphologically the BMZ started degenerating after 24 hours. Regeneration started on the 4th day and reached an optimal level on the 5th day. Chance of the immunofluorescence line at the BMZ began with a slight weakening at 24 hours followed by progressive widening and breakage until the 4th day when fluorescence was no longer detectable. Except for a few punctate areas immunofluorescence remained absent on the 5th day. Sixth day specimens, however, revealed reappearance of the immunofluorescence line in a continuous pattern. By the 7th day a second morphologic and antigenic degeneration started at the BMZ. This lag between the morphologic and antigenic regeneration of the BMZ in organ culture although shorter is comparable to the one observed during the development of the BMZ in fetal life.
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Semipermanent mounting media and postfixation of immunofluorescence (IF) slides in alcohol have been effective in preserving specific fluorescence (SF). In the present study the efficacy of such mounting was compared with a simpler technique of sealing the routinely mounted IF slides with nail polish. Frozen sections of skin lesions of systemic lupus erythematosus and lichen planus, and patients' sera known to have pemphigus or pemphigoid antibodies were used for IF procedures. SF was equally detectable with both techniques for over 8 months. Control slides similarly processed but not sealed lost most of their SF within a few weeks.
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Flavins are reported to protect cellular DNA against UV irradiation injury in vitro. The possible photoprotective effects of riboflavin in vivo on UV-induced carcinogenesis were studied in three groups of HR-hairless mice. Group I served as control. Group II was painted daily with a 15 mg per ml solution of riboflavin. In Group III, drinking water was replaced with a 15 mg per ml solution of riboflavin in water. All three groups were simultaneously irradiated in a light box with two Westinghouse FS20 sunlamps from a distance of 30 cm for 5 min daily 6 days a week throughout the experiment. By the 11th month all surviving mice developed several histologically proven squamous cell carcinomas. The total numbers and times of onset of tumors did not vary in the three groups. Thus, no protective effect of massive amounts of riboflavin on ultraviolet-induced carcinogenesis in the hairless mouse could be demonstrated when such high dose rates of UV were used.