Scleromyxoedema-like eruption following haemodialysis or nephrogenic fibrosing dermopathy?
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Biomedical subjects
Publications and source records attributed to G Dawn.
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Solitary morphoea profunda (SMP) is an unusual form of scleroderma and is rarely mentioned in the literature. The back of the trunk is described as the commonest site of involvement by SMP. This disease has been recognized as a nonprogressive condition. We report three cases of SMP seen at our department within a 1-year period. Interestingly, all three patients were females and the lesions were situated on the right upper buttock. In one patient the lesion extended despite using topical tacrolimus but subsequently the lesion was kept under control with topical clobetasol propionate.
BACKGROUND: Erosive pustular dermatosis of the leg (EPDL) has been described in association with venous insufficiency and atrophy of the skin of the lower leg. Like erosive pustular dermatosis of the scalp, this disease has also been reported to be a non-infective condition. OBJECTIVES: To investigate the clinicopathological features and, where possible, the aetiology of clinical EPDL. METHODS: We identified a group of patients undergoing continuous compression bandaging for venous dermatitis of the legs and/or predominantly venous leg ulceration with clinical features described in patients with EPDL. They were investigated by skin biopsy, patch testing and microbiological tests for the presence of bacteria and fungi. RESULTS: Twenty-four of 400 (6%) patients were noted to have pustules on the leg(s). There was laboratory evidence of fungal infection in 13 of 24 patients (54%), with complete and sustained resolution of pustules after antifungal treatment. Pustulation in the other 11 patients (46%) was unresponsive to antibiotics for confirmed bacterial infection; some improvement was seen with potent topical corticosteroids but full clearance was achieved only after a switch from continuous four-layer compression bandaging to the use of intermittent long stretch compression. CONCLUSIONS: EPDL is a fairly common clinical picture seen in patients undergoing continuous compression bandaging. It may be produced by opportunistic, particularly fungal, infection. In almost half an infective aetiology cannot be demonstrated and a pyoderma gangrenosum-like process may be implicated.
BACKGROUND: Delayed pressure urticaria (DPU) is difficult to treat. High-dose intravenous immunoglobulin (IVIG) has been found to be effective in treating patients with autoimmune chronic urticaria. OBJECTIVES: To report the effect of IVIG on eight patients with severe unremitting DPU. METHODS: IVIG was administered at a dose of 2 g kg-1 over 2-3 days on an in-patient basis. The response to treatment was assessed subjectively and recorded as remission, improved or unchanged. An autologous serum skin test (ASST) was performed in seven patients. RESULTS: Three of eight patients achieved remission; two after one infusion and one after three infusions. Two patients improved. Three patients remained unchanged; of these, two declined further treatment after two infusions, and one failed to improve after six infusions at monthly intervals. Four of seven patients had positive ASST; three responded to IVIG. Two developed delayed positive ASST; both responded to IVIG. Of three patients with negative ASST, two responded. CONCLUSIONS: IVIG induced remission or improved symptoms in five of eight patients with DPU with severe unremitting disease who had failed to respond to other therapies or were controlled only with systemic corticosteroids. Those who responded did so with three or fewer infusions. ASST is not a reliable predictor of response to IVIG.
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BACKGROUND: The basopenia of chronic urticaria relates to histamine releasing autoantibodies in the serum of patients with autoimmune urticaria. This reduction in circulating basophils may be due to active recruitment into weals. If so, it might be expected that numbers in blood would be reduced when urticaria is active and increased after treatment. The primary aim of this study was to look at diurnal variation of basophil numbers in patients with chronic ordinary urticaria (not physical or vasculitic) in relation to disease activity and the effect of treatment with antihistamines and corticosteroids, and to compare the results with healthy controls. A secondary aim was to compare a standard manual counting method with automated basophil counts and to look at numbers of other circulating leucocytes that might be relevant to urticaria pathogenesis. METHODS: Manual basophil counts using a toluidine blue stain and automated 5-part differentials (Coulter Gen. S) were performed at 4-hourly intervals from 08.00 to 20.00 in 10 healthy controls (six women, age 24 to 63 years) and seven chronic urticaria patients (five women, 24 to 50 years). All chronic urticaria patients had severe daily or almost daily urticaria. Only one of six chronic urticaria sera showed in vitro basophil histamine releasing activity. Counts were performed without treatment, after a week of taking loratadine 10 mg daily and after 3 days of adding prednisolone at 0.6 mg/kg/day (maximum 40 mg). Daily urticarial activity scores (UAS) were derived from weal numbers and itch, maximum 7. RESULTS: There was no significant overall diurnal variation of basophil numbers in healthy controls or chronic urticaria patients. Mean (SE) manually counted basophil were higher in healthy controls than chronic urticaria (43.4/ microL (2.1) vs. 4.4 (0.8), P < 0.001). Basophil counts were reduced in healthy controls on steroids (19.2 (1.9), P < 0.001) but increased in chronic urticaria (8.9 (1.9), P < 0.001). Loratadine did not influence them. UAS fell on treatment (3.3 (0.4) baseline, 1.4 (0.5) on loratadine and 0.5 (0.2) on prednisolone with loratadine, P < 0.001). There was a negative linear correlation between basophil numbers and UAS in untreated chronic urticaria patients (P = 0.001, Spearman rank correlation). Manual and automated basophil counts showed poor agreement. Lymphocyte numbers were lower in chronic urticaria than healthy controls. Neutrophils increased whereas lymphocytes and eosinophils decreased in all subjects on prednisolone. They were unaffected by loratadine. CONCLUSION: The results are consistent with the hypothesis that circulating basophils may be recruited from blood into urticarial weals during disease activity. Automated counts are not suitable for assessing basophil numbers in chronic urticaria. The relevance of reduced lymphocyte numbers in chronic urticaria needs to be explored.
Post-menopausal frontal fibrosing alopecia (PFFA) has become an increasingly recognized distinct clinical entity in recent years. Most cases have been reported from Australia; however, it seems likely that the condition is under-recognized. PFFA has similarities to lichen planopilaris but is differentiated by a distinctive symmetrical fronto-temporal distribution and progressive course. We report two cases from the UK and review the literature.
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Patients with vascular spiders and angiomas, especially on exposed sites, demonstrate considerable psychological morbidity and therefore request treatment. Traditionally, electrosurgical modalities have been used to treat such lesions, but more recently lasers have been introduced. This open study assesses the efficacy of these two treatment modalities, scoring patients' preference and psychological morbidity before and after treatment. All patients had two vascular spiders or angiomas. One lesion was randomized for treatment with the hyfrecator, whilst the other lesion was treated with the potassium titanyl phosphate (KTP) vascular laser. The end-point of the study was either clearance of lesions or a maximum of three treatments at 2-month intervals. In this first comparative study, we demonstrate that both therapeutic modalities were effective in treating these lesions and in significantly reducing the post-treatment psychological morbidity score. However, on average, only one treatment with the KTP laser was required to achieve clearance compared with two treatments with the hyfrecator. This would result in less clinic visits. In addition, patients favoured the KTP laser because of the lack of side-effects. Both the KTP vascular laser and the hyfrecator were able to clear vascular spiders and angiomas, but the KTP laser was superior as fewer treatment episodes were required and patients preferred this treatment modality because of the lack of side-effects.
We describe the case of a 37-year-old female with a history of psoriasiform dermatitis who presented with multicentric primary cutaneous CD30-positive anaplastic large T cell lymphoma (ALCL). Despite aggressive systemic therapy, the patient suffered multiple relapses and the lymphoma spread to cervical and inguinal lymph nodes. Later in her clinical course it was appreciated that she was also suffering from lymphomatoid papulosis (LyP). The case illustrates the overlapping clinical, histological and immunophenotypic features of ALCL and LyP, conditions which represent a spectrum of CD30-positive lymphoproliferative disease. A multidisciplinary approach between dermatologist, oncologist and pathologist is essential for the optimal management of these complex conditions.
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Angiogenesis plays an important role in progression of various tumours including malignant melanoma. It is possible that the immunostaining of angiogenic markers could differentiate benign and malignant melanocytic lesions or that the immunostaining pattern with angiogenic markers could vary with tumour thickness and thus be a prognostic marker. We were interested to see whether there was any correlation between endoglin (CD 105; EDG) expression with tumour thickness in primary cutaneous malignant melanomas (MM), any correlation between EDG expression and clinical outcome in patients with primary cutaneous MMs and any difference in staining pattern between cutaneous MMs and Spitz naevi which could be of diagnostic value. Tissue sections from 14 primary cutaneous MM lesions with a Breslow thickness of > 2 mm, 10 primary cutaneous MM lesions with a Breslow thickness of 1-2 mm, and six Spitz and 10 compound naevi were stained for EDG. EDG expression was compared with survival in patients with primary cutaneous MMs. Overall, EDG staining was positive in 96% of MMs and 94% of benign melanocytic naevi. Very strong (++++) and strong (+++) EDG staining was found in 58% of MMs and 56% of benign melanocytic lesions. EDG expression did not vary significantly with the thickness of the lesion in primary cutaneous melanoma. Survival of melanoma patients did not correlate with the degree of EDG expression. Therefore, expression of this marker alone is not sufficient to differentiate benign and malignant melanocytic lesions.
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The aims of this study were to analyze the change in trend of allergic contact dermatitis (ACD) from nickel in a single tertiary referral centre. In 1982 and 1997, 800 and 860 patients were patch tested, respectively. The frequency of positive reactions to nickel from our centre was 16% in 1982 and 22% in 1997. In both years, the vast majority of patients developed the eruption below 30 years of age. However, the commonest age of onset in 1982 was in the 11-20 year age group, while in 1997, this was 1 decade later, in the 21-30 year age group. In 1997, we noted a much higher female preponderance (F:M=13:1) than in 1982 (F:M=6:1). There was no change in male prevalence but a slight increase in female prevalence in 1997. The rate of atopy in patients with nickel ACD showed an increase from 23% in 1982 to 33% in 1997. Nickel was thought to be a contributory factor in causing occupational dermatitis in 27% of patients in 1982 and 24% patients in 1997. Of these, hairdressing in 1982 and nursing in 1997 were the commonest occupations. In 1982 and 1997, respectively, 40 (5%) and 37 (4%) patients were positive to nickel alone, while 89 (11%) and 150 (18%) patients showed positive reactions to other allergens in addition to nickel. In both the years, the hands were the main sites of involvement. However, in 1997 there was an increase in the number of patients presenting with face and neck involvement.