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Biomedical subjects

J J Gilkes

Publications and source records attributed to J J Gilkes.

At least 19 recordsLinked to original sources

Pemphigus vulgaris: the manifestations and long-term management of 55 patients with oral lesions.

Perhaps surprisingly, the manifestations and management of patients with pemphigus vulgaris and oral lesions have been detailed only infrequently. The present study has examined the clinical features, diagnosis and management of a cohort of 55 patients, including three adolescents, with pemphigus vulgaris predominantly affecting the oral mucosa. There was about a 6-month delay from the onset of symptoms until presentation for diagnosis, longer in men than in women. Patients typically had multiple lesions affecting mainly the buccal and/or palatal mucosae, and over half the patients had lesions affecting non-oral mucosal sites. Nearly one-quarter (24%) had cutaneous involvement. Most patients were otherwise healthy with no other autoimmune disorders. Classical histopathological features of pemphigus vulgaris were present in all patients, as well as IgG intraepithelial deposits in all patients tested and circulating epithelial antibodies in most. Thirty-two patients were treated in the clinic, four responding to topical immunosuppressive therapy, the remainder needing and responding, at least in part, to systemic immunosuppression. Systemic corticosteroids often with adjunctive immunosuppressives, particularly azathioprine, were required in 87% of patients. In 18% of the patients, the disease resolved in 3 months, but 76% had recalcitrant disease. Adverse effects were seen in 78%, and two patients died, at least one as a consequence of immunosuppressive therapy. It is concluded that pemphigus vulgaris affecting the oral mucosa is still diagnosed only after considerable delay because patients, especially men, present late; it has a chronic course; it is often associated with lesions in other mucosae and/or skin; it can be resistant to currently available therapies; and immunosuppressive therapy frequently produces adverse effects, occasionally lethal.

Administration, Topical↗

Systemic corticosteroids in the phenytoin hypersensitivity syndrome.

A patient is described with the characteristic features of phenytoin hypersensitivity syndrome (PHS) including fever, erythroderma, tibial and facial oedema, pinhead-sized facial pustules and abnormal liver function tests. The use of systemic corticosteroids in this condition is not established. We report the resolution of all the features of PHS with systemic corticosteroids and recurrence on withdrawal, and further remission on reinstitution of systemic corticosteroids.

Anti-Inflammatory Agents↗

Acrodermatitis chronica atrophicans: a case report and review of the literature.

We report a 55-year-old woman with acrodermatitis chronica atrophicans (ACA) and a peripheral sensory neuropathy. ACA is an uncommon late cutaneous manifestation of Lyme disease, which follows disseminated Borrelia burgdorferi infection. This is the second published case from the U.K. since serological diagnosis has been available. In this patient the diagnosis was confirmed by serology using a sensitive enzyme-linked immunosorbent assay and immunoblotting techniques. B. burgdorferi DNA was demonstrated in the affected skin using the polymerase chain reaction, although staining and cultures for the organism were negative. Recommended treatment of ACA is with oral doxycycline 100 mg twice daily for 28 days, but our patient did not respond well to this regimen. She was therefore treated with ceftriaxone intravenously for 21 days, which resulted in a rapid symptomatic and clinical response.

Acrodermatitis↗

Pyostomatitis vegetans: report of three cases and review of the literature.

Pyostomatitis vegetans is a rare condition of the mouth frequently associated with chronic inflammatory bowel disease. It is characterized by miliary abscesses and superficial erosions affecting a hyperplastic, soft and friable oral mucosa. Three cases are presented here and used as a basis for reviewing the literature and discussing the differential diagnosis, etiology and treatment of this unusual condition.

Adult↗

Natural progression of male pattern baldness in young men.

Twenty-six men who presented with male pattern baldness (androgen-dependent alopecia), were quantitatively evaluated for scalp hair variables and compared with 13 age-matched controls. Compared to controls, significant mean differences for hair variables were found in the frontal-vertex area, while in the occipital area, a reduction in total hair density (hairs/cm2) was the only significant (P less than 0.05) finding. A large proportion (48.5%) of meaningful hair (non-vellus hair) was less than or equal to 40 mm in length, yet had diameters similar to hairs growing much longer. In controls, these hairs accounted for only 12.2% of the total population. Compared to baseline, mean values from the frontal-vertex area of subjects with androgen-dependent alopecia were significantly lower for total hair density, meaningful hair density (non-vellus hairs/cm2) and percentage of hair in the anagen growth phase, 12 and 24 months later. During this time, total hair density decreased by 6.5% after 12 months and by 11.9% after 24 months. Similarly, meaningful hair density declined at 12 months by 10.8% and by 22.7% after 24 months. No change in any hair variable was detected in controls after 12 or 24 months. Our findings suggest that medications capable of maintaining the existing hair population should be regarded as effective treatments for this condition. Left untreated androgen-dependent alopecia progressively deteriorates. The induction of non-vellus hairs less than or equal to 40 mm in length to grow longer, would substantially improve the aesthetic profile without the need to generate new hair.

Adult↗

Biochemical and trichological characterization of diffuse alopecia in women.

One hundred women who presented with diffuse alopecia, were quantitatively evaluated for hair variables and compared with 20 controls. Fifty were selected for biochemical and haematological investigations, 44 of whom also underwent endocrine evaluation. Compared to controls, significant changes in hair values were found in the frontal area of all subjects, while 84% had significant changes in the occipital area. A biphasic distribution of hair diameter was evident in subjects who had percentages of vellus hair and telogen hair less than or equal to 30 mm in length that were above the control ranges. No significant difference between the mean hormonal values of women with diffuse alopecia and controls could be found. No correlation between hair values and individual or combined hormonal levels could be established. In 18 subjects (40.9%) hormonal values were within the control ranges and these apparently normal findings were often associated with adverse hair profiles. A raised dihydrotestosterone was found in 13 subjects (29.5%) and was the most frequently elevated androgenic finding. Seventeen (34.0%) had changes in iron metabolism, while in 36 (72.0%) serum ferritin levels were below the lowest control value. All had a decrease in the percentage of hair in the anagen growth phase compared to controls. The hair changes were similar to those observed in genetic hair loss in men, a proven androgen-dependent condition. We propose that diffuse androgen-dependent alopecia is the appropriate name to describe this condition in these women.

Adolescent↗

Severe polymorphic eruption of pregnancy occurring in twin pregnancies.

We describe three women pregnant with twins who developed severely symptomatic polymorphic eruption of pregnancy. In all of these women oral prednisolone treatment was indicated and administered to two of them with resulting remission. Both these women breast fed their twins and relapsed in the puerperium requiring further systemic steroid therapy. The third woman was cured by early elective delivery. It has not been previously suggested that the severity of polymorphic eruption is related to multiple pregnancy and this possibility is discussed. The safety of oral prednisolone in pregnancy and during lactation is also reviewed.

Adult↗

Polyostotic fibrous dysplasia associated with extensive linear epidermal naevi.

Albright's syndrome is characterized by the combination of polyostotic fibrous dysplasia, precocious puberty and café-au-lait spots. We describe a patient with polyostotic fibrous dysplasia who also had extensive linear epidermal naevi, an association that has not been previously described in the English literature.

Adult↗

Skin and nail changes in the arthritic foot.

The arthritic process is unlikely to be confined to the foot; similarly the cutaneous lesions associated with the arthritic foot are often widespread. Careful examination of the skin and nails, particularly the finger nails, may be helpful in the differential diagnosis when the patient presents with a painful foot joint. Conversely, certain cutaneous lesions may alert the physician to the possibility of joint disorders presenting at some later date. In this chapter, it is not possible to mention every skin lesion associated with an arthropathy. Some skin lesions are specific but many are non-specific and occur in several rheumatic diseases. The rheumatologist and dermatologist work in closest co-operation when managing patients with lupus erythematosus and psoriatic arthritis and it is for this reason there is particular emphasis on these two diseases. Patients with rheumatoid arthritis and gout usually come within the province of the rheumatologist, but there are often many characteristic dermatological features to these diseases. This chapter also includes some more esoteric diseases such as Familial Mediterranean fever, Behçet's syndrome, disseminated gonococcal infection and Lyme disease which may present a diagnostic problem to the general physician, rheumatologist or dermatologist.

Adult↗

Dermatology.

Disease processes occur in the skin and its appendages, as in other organs, against the background of age-related and degenerative changes. The skin is additionally influenced by the cumulative effect of environmental factors, especially solar radiation.

Aged↗

Relationship between the serum autoantibody titers and the clinical activity of pemphigus vulgaris.

With human tonsillar epithelium as a substrate for the indirect immunofluorescence assay, a statistically significant relationship was demonstrated between the clinical activity of the disease and the autoantibody titers in seventy-eight serum samples obtained from patients with pemphigus vulgaris. High pemphigus antibody titers were associated with severe ulceration, intermediate titers with moderate lesions, and low titers with mild pemphigus. However, the sequential studies revealed that this relationship was not always consistent throughout the course of the disease, and in three out of fourteen patients the correlation was either poor or negative. These results indicate that serial pemphigus antibody titers may not be consistent enough to be used reliably as the sole guide for monitoring disease activity.

Autoantibodies↗