Stephen Lee

Merkel Cell Carcinoma: Assessing the Effect of Wide Local Excision, Lymph Node Dissection, and Radiotherapy on Recurrence and Survival in Early-Stage Disease—Results From a Review of 82 Consecutive Cases Diagnosed Between 1992 and 2004

BackgroundWide surgical excision, lymph node dissection, and radiotherapy have been used with varying efficacy in the management of early-stage Merkel cell carcinoma.MethodsRecords of 82 patients with early-stage Merkel cell carcinoma between 1992 and 2004 were reviewed.ResultsForty-two patients developed a recurrence, and 44 died during the study period. Twenty-nine patients presented with regional lymph node disease, which was independently associated with diminished survival (hazard ratio [HR], 4.08; 95% confidence interval [CI], 1.55–10.75; Pxa0=xa0.005). Lymphadenectomy was independently associated with prolonged disease-free survival (median, 28.5 vs. 11.8 months; HR, .46; 95% CI, .22–.94; Pxa0=xa0.034) but not overall survival (Pxa0=xa0.25). Margin-negative excision of the primary tumor (60 of 73) was not significantly associated with either prolonged disease-free survival (median, 16 vs. 14 months) or overall survival (median, 54 vs. 34 months). Forty-eight patients received radiotherapy: 36 to the primary site and 31 to the regional lymph nodes. Radiotherapy to both sites was associated with a longer median time to first recurrence (primary site, 24.2 vs. 11.8 months; regional lymph nodes, 46.2 vs. 11.3 months) and survival (primary site, 53.9 vs. 45.7 months; regional lymph nodes, 103.1 vs. 34.2 months). Administration of any radiotherapy was significantly associated with a prolonged time to first recurrence (HR, .39; 95% CI, .20–.75; Pxa0=xa0.004) and survival (HR, .39; 95% CI, .18–.82; Pxa0=xa0.013) on the Cox regression multivariate analyses.ConclusionsAdjuvant radiotherapy to the primary site after surgical excision is recommended in early-stage disease. Involved regional lymph nodes should be treated with radiotherapy with or without lymphadenectomy.

Clinical manifestations and outcomes in 17 cases of Stevens-Johnson syndrome and toxic epidermal necrolysis.

The clinical features and outcomes of 17 patients with Stevens–Johnson syndrome (SJS) or toxic epidermal necrolysis (TEN) were retrospectively reviewed. There were 11 males and six females with an average age of 61.5 years. Ten patients with SJS (seven males, three females) and seven patients with TEN (four males, three females) were identified. Antibiotics, mainly β‐lactams, were the most common cause of SJS/TEN in this series. The mean skin loss in TEN was 45.7% total body surface area in contrast to the lesser skin loss (<10%) observed in three patients with SJS. Complications included septicaemia, pneumonia and multi‐organ failure, mainly in the TEN group. Two patients died from TEN‐related complications and one patient with SJS died from unrelated causes. Ocular involvement and skin pigmentary changes represented the most significant long‐term sequelae.

Effect of the glycemic index of carbohydrates on Acne vulgaris.

Acne vulgaris may be improved by dietary factors that increase insulin sensitivity. We hypothesized that a low-glycemic index diet would improve facial acne severity and insulin sensitivity. Fifty-eight adolescent males (mean age ± standard deviation 16.5 ± 1.0 y and body mass index 23.1 ± 3.5 kg/m2) were alternately allocated to high or low glycemic index diets. Severity of inflammatory lesions on the face, insulin sensitivity (homeostasis modeling assessment of insulin resistance), androgens and insulin-like growth factor-1 and its binding proteins were assessed at baseline and at eight weeks, a period corresponding to the school term. Forty-three subjects (n = 23 low glycemic index and n = 20 high glycemic index) completed the study. Diets differed significantly in glycemic index (mean ± standard error of the mean, low glycemic index 51 ± 1 vs. high glycemic index 61 ± 2, p = 0.0002), but not in macronutrient distribution or fiber content. Facial acne improved on both diets (low glycemic index −26 ± 6%, p = 0.0004 and high glycemic index −16 ± 7%, p = 0.01), but differences between diets did not reach significance. Change in insulin sensitivity was not different between diets (low glycemic index 0.2 ± 0.1 and high glycemic index 0.1 ± 0.1, p = 0.60) and did not correlate with change in acne severity (Pearson correlation r = −0.196, p = 0.244). Longer time frames, greater reductions in glycemic load or/and weight loss may be necessary to detect improvements in acne among adolescent boys.

Drug-related pemphigus and angiotensin converting enzyme inhibitors

A 105‐year‐old woman developed pemphigus foliaceus. She had been on fosinopril, an angiotensin‐converting enzyme inhibitor (ACE inhibitor) for 4 years. Anti‐intercellular cement substance antibodies were positive with titre > 160. She died during admission of an unrelated illness. A 57‐year‐old man developed pemphigus vulgaris after 11 months treatment with quinapril. At 14 months after developing pemphigus, this man continues on prednisone and azathioprine. We speculate that these are cases of ACE‐inhibitor‐related pemphigus and we review ACE‐inhibitor‐related pemphigus.

TOXIC EPIDERMAL NECROLYSIS/STEVENS–JOHNSON SYNDROME: CURRENT TRENDS IN MANAGEMENT

Background:u2003 Toxic epidermal necrolysis (TEN) is an uncommon but potentially life‐threatening condition that involves sloughing of the skin at the dermoepidermal junction. TEN is a well‐recognized syndrome and is part of a range of severe mucocutaneous intolerance reactions, mostly elicited by drugs and/or their metabolites. Reported mortality rates vary widely from 20 to 75%. Several systemic treatment protocols for TEN have been published; however, none has been formally standardized in a randomized controlled trial. The present study documents the current management principles and trends seen in 16 patients admitted with TEN or Stevens–Johnson syndrome over a 5‐year period at the Concord Repatriation General Hospital Burns Unit.

Dysplastic naevi: To shave, or not to shave? A retrospective study of the use of the shave biopsy technique in the initial management of dysplastic naevi

The management of dysplastic naevi is a controversial subject. This study sought to assess the usefulness of the shave biopsy technique in the initial management of dysplastic naevi, and to demonstrate the advantages over the punch biopsy technique. We report a retrospective observational study of histopathology specimens examined in one histopathology practice over a 14‐month period. Patients who had a clinical diagnosis of ‘dysplastic naevus’, which had initially been biopsied using either a shave or punch biopsy, and then followed up with a full‐thickness elliptical excision, were included in the study. Histopathological concordance between the shave and punch biopsy specimens and their respective follow‐up elliptical excisions was compared. We found that 21 of 22 (95.5%) shave biopsies were concordant with their respective excision specimens, and that 29 of 41 (70.7%) punch biopsies were concordant with their respective elliptical excision specimens. Of the shave biopsy specimens reviewed, 66% showed that the dysplastic naevi were completely excised with the initial biopsy, compared with 21.2% of the punch biopsy specimens. These findings confirm that shave biopsies provide accurate diagnostic information in the assessment of dysplastic naevi. Shave biopsies enable the entire lesion to be submitted for histopathological assessment, improving the chances of an accurate diagnosis.

Vesiculobullous Hailey-Hailey disease: Successful treatment with oral retinoids

A 56‐year‐old male presented with a pruritic, generalized vesiculobullous eruption. His past history revealed classical symptoms of limited Hailey‐Hailey disease for 34 years. Clinically, vesicles, bullae and occasional pustules were present and multiple biopsies confirmed this to be an unusual presentation of Hailey‐Hailey disease. Various therapeutic modalities including topical and oral antibiotics, oral prednisone and dapsone failed to achieve sustained remission. Treatment with lowdose oral etretinate (25 mg daily) produced marked clinical improvement with complete suppression of new vesicle formation after 6 weeks.

Acitretin for lichen amyloidosus

We present two cases of lichen amyloidosus treated with retinoids. A 57‐year‐old Vietnamese woman has had extensive generalized recalcitrant lichen amyloidosus for 23u2003years. Treatment with oral etretinate (25u2003mg/day) for 3u2003years, and later oral acitretin (10u2003mg/day) for the past 10u2003years, has controlled the pruritus and flattened the hyperkeratotic papules. Whenever the acitretin was ceased her symptoms flared within weeks. On each occasion reintroduction of acitretin was effective within 1–2u2003months. The second case is that of a 51‐year‐old Australian Aboriginal woman who had a 2‐year history of lichen amyloidosus affecting her lower legs. A 2‐month course of oral acitretin (25u2003mg b.d.) produced a marked improvement in both the pruritus and hyperkeratotic papules. She was then lost to follow up for 2u2003years, during which time her symptoms recurred.

Reactive perforating collagenosis: A condition that may be underdiagnosed

Reactive perforating collagenosis is a perforating disorder developing in adults, usually in association with diabetes mellitus or renal failure. We present three cases diagnosed at the Royal Prince Alfred Hospital in a 5 month period. All three patients had long‐standing diabetes mellitus, hypertension, hypercholesterolaemia and ischaemic heart disease. Each patient presented with generalized pruritus and a papular eruption across the trunk and limbs. More than one biopsy or multiple levels were needed before the diagnostic histological features were seen. The first patient responded to 0.5% phenol with 10% glycerine in sorbolene cream. The second patient did not respond to topical betamethasone diproprionate 0.5 mg/g cream and antihistamines (hydroxyzine 25 mg nocte) and required narrow‐band ultraviolet (UV) B. The third patient, having failed to respond to topical betamethasone diproprionate 0.5 mg/g cream and wet dressings, antihistamines (hydroxyzine 25 mg tds and doxepin 50 mg nocte) and UVB required acitretin 25 mg orally per day. Because reactive perforating collagenosis responds to treatment, we believe this condition should be considered in patients with diabetes mellitus or renal failure presenting with pruritus and that biopsy of intact lesions may need multiple levels to help establish the diagnosis.

Erythroplasia of Queyrat of the glans penis on a background of Zoon's plasma cell balanitis

Erythroplasia of Queyrat of the glans penis developed in a 79‐year‐old uncircumcised man on a background of biopsy proven Zoons plasma cell balanitis affecting the same site on the glans. The Zoons plasma cell balanitis had been treated with topical pimecrolimus for 1u2003month prior to the development of clinically evident erythroplasia of Queyrat. He was subsequently treated with topical 5‐fluorouracil 5% for 2u2003weeks, which resulted in clinical clearance. He has since been circumcised.