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We read with interest the guidelines recently published on sclerosing diseases of the skin (Part 2: Scleromyxedema, scleredema and nephrogenic systemic fibrosis)[1, 2]. However, we are concerned that the guideline recommendations proposed for prevention of nephrogenic systemic fibrosis (NSF) are potentially dangerous. Although we recognise the challenges in constructing comprehensive guidelines, we are concerned that this may be because the guidelines have not involved a multidisciplinary team.

Background: The efficacy and safety of UVA1 (340–400 nm) phototherapy were established by studies from European countries. Purpose: Evaluate experience with UVA1 phototherapy for patients with cutaneous diseases in the United States. Methods: A retrospective analysis of 92 cases of UVA1-treated cutaneous conditions from four medical centers in the United States was performed. Results: Two-third of the patients showed a fair to good response (26–100% improvement) and one-third of the patients showed a poor response (0–25% improvement). Diseases with a moderate to good response (51–100% improvement) included scleredema adultorum, hand or foot dermatitis, atopic dermatitis, morphea (medium or medium- to high-dose UVA1), systemic sclerosis, and urticaria pigmentosa. Besides tanning, other adverse effects were found in 15% of patients, which include pruritus, erythema, tenderness, and burning sensation. Patients with skin types I–III responded better that those with a darker skin type. Conclusion: UVA1 phototherapy is a useful and well-tolerated treatment option for a variety of skin conditions.

Two children with chronic and asymmetric skin indurations are presented. The clinical and pathologic features are suggestive of asymmetric childhood scleredema and stiff skin syndrome. The key features of scleredema and stiff skin syndrome are discussed.

We report a patient with the typical lesions of diabetic scleredema. Histological findings of the involved skin were thickening of the dermis, depositions of mucins, and fibrosis. Biochemical analysis revealed an increase in glycosaminoglycans in the involved skin as well as in the cutaneous lupus mucinosis. Mucinous materials were composed of hyaluronic acid.

Acanthosis nigricans (AN) and scleredema are two skin conditions that have been reported in association with diabetes mellitus. Few cases associating scleredema and AN have been reported. A literature search did not reveal any reports of diabetic patients developing AN on top of a scleredema plaque. Here we report a patient with diabetes mellitus who developed AN within the same indurated scleredema plaque.

Scleredema adultorum is a rare connective tissue disorder of unknown cause. Both bath-PUVA and cream-PUVA therapy were reported to be effective. We describe a patient with scleredema adultorum who showed a striking clinical improvement with a medium-dose UVA1 phototherapy (single dose, 50 J/cm(2); 35 treatments).

The association of specific dermatological complications with diabetes mellitus is well recognized. Of 100 hospital-based patients with diabetes mellitus (age 48 years +/- 2SE), 14% had scleroedema diabeticorum. The affected subjects had a higher prevalence of retinopathy (p less than 0.001) and albuminuria (p less than 0.025). The duration of scleroedema correlated with the duration of diabetes (p less than 0.005). These findings highlight the relatively common occurrence of this skin condition which often goes unrecognized in people with diabetes.

The skin thickness in a group of patients with scleredema was measured using ultrasonic techniques and then compared with a group of normal individuals. The mean dermal thickness in scleredema was 7.7 mm, compared to 3.3 mm in the normal control group. Ultrasonic evaluation of skin thickness may be of value in following the natural history or response to therapy of certain diseases that affect dermal thickness.

A case of the uncommon association of scleredema and diabetes mellitus is presented. Such patients usually are obese, and the scleredema quite extensive and longlasting. The difference between this entity and scleredema adultorum is discussed. Attempts at treatment during a two-week period were unsuccessful.