Progressive pigmented purpura (Schamberg's disease) responding to TL01 ultraviolet B therapy.
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We describe four patients in whom porokeratosis coexisted with lympoedema of the legs. A possible pathogenetic link between the two disorders is discussed, as well as the therapeutic implications and the novel physical sign of lymphoedema bulging through the porokeratotic lesions.
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Transepidermal water loss (TEWL) in psoriatic skin lesions seems to be related to the severity of the psoriasis, and the electrical capacitance and conductance of the skin are indicators of the hydration level of the stratum corneum. We compared the characteristics of these electrical measurements, in assessing the persistent effect of a moisturizing cream on skin hydration and barrier function in psoriasis patients. Seventeen Korean psoriasis patients were recruited. Their right leg was treated with the moisturizer twice daily for 6 weeks, while their left leg was used as the control site. For each patient, one psoriatic plaque on each leg was selected as the involved psoriatic lesion. Uninvolved psoriatic skin was regarded as the apparently healthy looking skin 4-5 cm away from the periphery of the psoriatic lesion. The TEWL, electrical capacitance and conductance were measured, in order to evaluate the barrier function and hydration level of the stratum corneum. The clinical and biophysical data for each patient were recorded at the start of the study and after 2, 4 and 6 weeks. The degree of skin dryness at the applied area improved progressively. The electrical capacitance at the treated psoriatic lesion increased significantly after 2 weeks, and this improvement was maintained during the entire study period. However, no noticeable change was observed in the electrical conductance. The TEWL showed an inverse pattern to that of the skin capacitance, decreasing during the study period. The skin capacitance and TEWL exhibited good correlation with the visual assessment of skin dryness, but the skin conductance did not. Our data suggest that electrical capacitance and TEWL may be useful in the evaluation of the effect of a moisturizer on the hydration status and barrier function of psoriatic skin.
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The primary cause of collagen degeneration in necrobiosis lipoidica (NL) is proposed to be immunologically mediated vascular disease. Ultraviolet (UV)A1 has been used successfully to treat scleroderma in which both vascular damage and collagen dysregulation also occur. We treated six patients with NL [(five women; mean age of 32 years (range 22-70) and mean disease duration of 2.9 years (range 6 months to 5 years)] with a high-output ultraviolet (UV)A1 2-kW filtered metal halide source (Dr Hönle; Dermalight ultrA 1) having an emission spectrum of 340-440 nm. All patients had NL on the shins, which had been unresponsive to potent topical corticosteroid therapy (n = 6) and had responded minimally or not at all to TL-01 UVB (n = 2), topical psoralen plus UVA (PUVA) soaking (n = 2) or oral PUVA (n = 1) therapy. Patients received a variable number of total exposures (15-51), given 3-5 times weekly. NL resolved completely in one patient; this patient had minimal improvement after the first course of 16 exposures, but after a further 13 exposures, resolution occurred 6 months later. Two subjects obtained moderate improvement in their overall disease severity after 15 and 24 exposures, while two had only minimal improvement after 15 and 51 exposures. The remaining patient had no improvement after 16 treatments. Patients with the shortest disease duration had the greatest response. UVA1 therapy may be of benefit for the treatment of NL as an adjuvant therapy to topical corticosteroids or as a second-line alternative to other phototherapies, and may have a superior outcome in a proportion of patients.
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Lichen amyloidosis (LA) is a chronic, pruritic skin disorder characterized by brownish-grey papules on extensor surfaces of legs and rarely on the trunk. Thioflavin T-positive amyloid deposits are found in the papillary dermis of affected skin, which is the only organ involved. A variety of therapeutic regimens for lichen amyloidosis have been described; however, in many cases with only limited effect. We report on two patients with lichen amyloidosis with typical clinical symptoms not responding to local treatment. A combined regimen with bath psoralen ultraviolet A (PUVA) and oral acitretin was initiated, resulting in nearly complete resolution of the papules and impressive relief from the severe pruritus. The beneficial response has persisted for 8 months. The suggested combined therapy with bath PUVA photochemotherapy and oral acitretin represents an efficacious and practical treatment modality for lichen amyloidosis with long-lasting effects.
A 54-year-old woman was treated with an oral dose of 555MBq of 131I radioiodine for thyrotoxicosis. She had no clinically detectable extrathyroidal manifestations of Graves' disease at the time, but within two months developed moderately severe ophthalmopathy and very extensive thyroid dermopathy affecting her face, arms, hands and feet, in addition to the classic pre-tibial area. Although she developed mild post radioiodine hypothyroidism, this was detected at an early stage and its treatment had no effect on the extrathyroidal signs. Thyrotrophin receptor antibodies (TRAb) were positive before treatment (22% inhibition of TSH binding in neat serum), rose to very high levels following radioiodine (97.6% inhibition), and fell progressively over the following year during treatment with prednisolone. Thyroglobulin autoantibodies became detectable following radioiodine but thyroid peroxidase antibodies were undetectable throughout. Serum and purified IgG from blood samples obtained prior to steroid therapy and over the subsequent year were tested on a dermal fibroblast cell line in vitro for the stimulation of synthesis of glycosaminoglycans, protein and DNA, but no increase in radiolabel incorporation was apparent for any sample when compared to controls. The temporal relation between the radioiodine and the acute onset of dermopathy and ophthalmopathy, together with the abrupt rise in TRAbs, indicates a probable causal association. However, the absence of in-vitro fibroblast stimulation would suggest that the pathogenesis of Graves' dermopathy is not dependent solely on any simple humoral factor.
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