Scleredema of Buschke associated with lichen sclerosus: Three cases.

Scleredema adultorum of Buschke is a rare fibromucinous, scleroderma-like connective tissue disease most commonly found in a post-infectious setting or linked to hematological disorders or diabetes. Lichen sclerosus et atrophicus is an autoimmune condition only in 2.5% of cases localized exclusively at an extragenital site, occurring in up to 34% of patients in association with other autoimmune diseases such as vitiligo, thyroid disorders, alopecia areata, lichen planus, morphea, pernicious anemia and systemic lupus erythematosus. In particular, a stronger link with an autoimmune background in lichen sclerosus et atrophicus has been observed in women who showed higher prevalence for autoimmune conditions and circulating autoantibodies. Literature reveals a genetic susceptibility linked to specific HLA types. We report three patients who developed lichen sclerosus et atrophicus superimposed on skin involved by scleredema adultorum of Buschke. Although the association of lichen sclerosus et atrophicus with scleredema adultorum of Buschke could be coincidental, both diseases could be considered part of the spectrum of sclerodermoid disorders with common underlying pathogenetic mechanisms; which could explain the sequential or simultaneous occurrence of both lesions in our patients.

Onychomadesis Following Cutaneous Vasculitis.

Beau lines are transverse, band-like depressions extending from one lateral edge of the nail to the other and affecting all nails at corresponding levels (1). Onychomadesis is considered an extreme form of Beau line with subsequent separation of the proximal nail plate from the nail bed. Both fall along a spectrum of nail plate abnormalities that occur secondary to temporary nail matrix arrest (NMA). Various systemic and dermatologic conditions have been reported in association with onychomadesis (2-7) (Table 1). Nail changes can affect all or some of the nails and both the fingernails and toenails; however, fingernails are more frequently affected. The severity of the nail changes varies depending on the underlying cause, its duration, and environmental factors (8). We present a case of onychomadesis following cutaneous leukocytoclastic vasculitis (CLCV). A 61-year-old woman presented to the Dermatology Clinic complaining of a purpuric rash that began on her lower extremities and rapidly progressed to her abdomen and upper extremities over the previous five days. Her medical history was remarkable for hypertension and diet-controlled diabetes mellitus. Her medications included enalapril, which she had been taking for the past four years. On three consecutive days before the skin eruption, the patient took oral diclofenac sodium for hip pain. A clinical examination revealed non-blanching petechial rash on the legs, abdomen, and upper limbs up to the elbow (Figure 1, A) with leukocytoclastic vasculitis on biopsy (Figure 1, B). Direct immunofluorescence was negative. Laboratory investigations revealed a white blood cell count of 14.5 × 109/L with a normal differential count, and a platelet count of 380 × 109/L. Westergren erythrocyte sedimentation rate was 65 mm/1st h, and C reactive protein was at 8.5 mg/dL. Antinuclear antibodies, rheumatoid factor, immune complexes, and cryoglobulinemia were negative, as were B and C hepatitis virus serological tests. Her renal, cardiac, pulmonary, and abdominal exams were normal. Diclofenac was discontinued due to a clinical suspicion of drug-induced cutaneous vasculitis. The rash resolved in 2 weeks without treatment, leaving post-inflammatory hyperpigmentation. Four weeks later, she presented with painless, palpable grooves on all 10 fingernails (Figure 2). The grooves were 3 to 4 mm in width, at a similar distance from the proximal nail fold. There were no signs of periungual inflammation. The patient denied any recent history of trauma, unusual activities, or chemical exposure. Routine serum biochemistry and hematology results were normal. Repeated potassium hydroxide preparations and fungal cultures of the nail clippings were negative. A diagnosis of Beau lines and onychomadesis was made. Nail changes were tolerable and did not require any specific treatment. During the follow up, the Beau lines advanced with the linear growth of the nails and disappeared (Figure 3 and 4). Four fingernails developed complete nail shedding (onychomadesis). No toenail alterations were observed in this period. A complete recovery of the nail plate surface was observed after 4 months. The nail matrix epithelium is formed by highly proliferating cells that differentiate and keratinize to produce the nail plate. The nail matrix epithelium is very susceptible to toxic noxae, and acute damage results in a defective nail plate formation. Nail matrix arrest is a term used to describe a temporary inhibition of the nail matrix proliferation that can present as Beau lines and onychomadesis (8). The width of Beau lines relates to the duration of the etiological agent. As the nail adheres firmly to the nail bed, the onychomadesis remains latent for several weeks before leading to temporary shedding (8,9). There are several proposed etiological mechanisms for NMA. NMA associated with fever, severe infection, and major medical illnesses can be explained by an inflammation of the matrix, periungual tissues, or digital blood vessels (8); chemotherapy agents temporary inhibit the mitotic activity in nail matrix (10); the detection of Coxackie virus in the shedding nail particle, following hand, foot, and mouth disease, suggests that the viral replication itself may directly damage the nail matrix (11). However, as nail changes are not unique, it may be difficult to incriminate a single etiological agent. Our patient presented with an onset of Beau lines seven weeks after the initial CLCV lesions, which suggests that vasculitis might have acted as a trigger for NMA. As the fingers were not affected by CLCV, an indirect effect of vasculitis is more plausible. Leukocytoclastic vasculitis is a small-vessel inflammatory disease mediated by a deposition of immune complexes. Thus, the circulating immune complexes may be involved in the damage of nail bed microvasculature. Considering that the patient had been receiving enalapril and diclofenac, it is less likely that those drugs were involved in the pathogenesis of NMA. Enalapril was continued, and the nail changes were resolved while patient was still on enalapril. Furthermore, diclofenac is a widely prescribed drug and its association with NMA is yet to be described in literature. We described a patient who developed Beau lines and onychomadesis following cutaneous leukocytoclastic vasculitis. This clinical observation can expand the spectrum of possible causes of nail matrix arrest.

Complementary and alternative medicine use among patients with psoriasis.

Despite the growing attention on safety and efficacy of conventional treatments, there is little information available on complementary and alternative medicine (CAM) used in psoriasis. In order to collect comprehensive information on CAM use, we conducted a face-to-face interview with 122 patients with psoriasis. All unconventional treatments for psoriasis used in the last 12 months were recorded. Fifty-seven patients (46.7%) used one of the CAM methods in the previous year, including topical and systemic antipsoriatics, dietary supplements, and diet. Forty-one different nonconventional topical treatments were used. Seven patients (5.7%) took nonconventional systemic medication, and 15.5% used dietary supplements. There were three patients who reported current adherence to a diet as treatment of psoriasis. Clinicians are often not informed that their patients are using complementary therapies. CAM may offer benefits as well as risks to patients with psoriasis. It is important to remind patient to report all ongoing and past topical and systemic treatments. The use of medications with unknown composition, efficiency, and safety should be discouraged.

Eosinophilic ulcer of the oral mucosa: report of a case with multiple synchronous lesions.

Eosinophilic ulcer of the oral mucosa is considered to be a benign, reactive, and self-limiting lesion, with unclear pathogenesis, manifesting as a rapidly developing solitary ulcer. We report the case of a 52-year-old man who presented with 4 synchronous ulcerations of the tongue. Histopathological examination showed polymorphic inflammatory infiltrate, rich in eosinophils, involving the superficial mucosa and the deeper muscle layer. Immunohistochemical analysis revealed single CD30 cells scattered within an inflammatory infiltrate. All the lesions began to regress spontaneously within 1 week after biopsy. A 4-year follow-up showed no recurrence.

Metabolic syndrome in untreated patients with psoriasis: case-control study.

BACKGROUND AND OBJECTIVES: Previous studies have shown a higher prevalence of metabolic syndrome in patients with psoriasis compared to controls. However, little attention has been paid to the effect of systemic anti-psoriatic drugs on the metabolic syndrome. The aim of this study was to investigate the association between psoriasis and the metabolic syndrome, by comparing untreated patients with psoriasis and population based control. PATIENTS AND METHODS: We conducted a hospital-based case-control study that included 122 untreated patients with plaque psoriasis and 122 age- and gender-matched controls. RESULTS: There was no significant difference in the prevalence of the metabolic syndrome between the patients with psoriasis (24.6 %) and the controls (22.9 %) (OR 1.095, 95 % CI 0.607-1.974). Among the components of the metabolic syndrome only hypertriglyceridemia and abdominal obesity were associated with psoriasis. The psoriatic patients with metabolic syndrome had a higher mean age (p =  0.001), and higher mean BMI (p =  0.001) compared with the psoriatic patients without metabolic syndrome. The metabolic syndrome was not associated with the severity of psoriasis. CONCLUSIONS: Untreated patients with psoriasis have no significantly higher prevalence of the metabolic syndrome than healthy controls. Our data suggest that systemic antipsoriatic drugs may play an important role in the pathogenesis of the metabolic syndrome.

Evaluation of two different intermittent pneumatic compression cycle settings in the healing of venous ulcers: a randomized trial.

BACKGROUND: Intermittent pneumatic compression (IPC) has been successfully used in the treatment of venous ulcers, although the optimal setting of pressure, inflation and deflation times has not yet been established. The aim of this study was to compare the effect of two different combinations of IPC pump settings (rapid vs slow) in the healing of venous ulcers. MATERIAL/METHODS: 104 patients with pure venous ulcers were randomized to receive either rapid IPC or slow IPC for one hour daily. The primary and secondary end points were the complete healing of the reference ulcer and the change in the area of the ulcer over the six months observational period, respectively. RESULTS: Complete healing of the reference ulcer occurred in 45 of the 52 patients treated with rapid IPC, and in 32 of the 52 patients treated with slow IPC. Life table analysis showed that the proportion of ulcers healed at six months was 86% in the group treated with the fast IPC regimen, compared with 61% in the group treated with slow IPC (p=0.003, log-rank test). The mean rate of healing per day in the rapid IPC group was found to be significantly faster compared to the slow IPC group (0.09 cm2 vs 0.04 cm2, p=0.0002). CONCLUSIONS: Treatment with rapid IPC healed venous ulcers more rapidly and in more patients than slow IPC. Both IPC treatments were well tolerated and accepted by the patients. These data suggest that the rapid IPC used in this study is more effective than slow IPC in venous ulcer healing.

Pentoxifylline--efficient in the treatment of venous ulcers in the absence of compression?

The aim of the study was to test the efficacy and tolerability of pentoxifylline on the healing of venous ulcers in the absence of standard limb compression. The study used a prospective randomized, open, controlled, comparative, parallel group design. The study included 80 eligible patients with confirmed venous ulcers (with clinical and photoplethysmography findings). The patients received either pentoxifylline 1200 mg per day (3 x 400 mg) orally in addition of local therapy, or the same local therapy alone. The main outcome measures were complete healing of ulcers, change in the ulcer area over the six-month observation period, and tolerability of the drug. The results showed that complete healing occurred in 23 (57.5%) patients receiving pentoxifylline and 11 (27.5%) patients without pentoxifylline (log rank test =2.49, p=0.013). Unwanted effects of pentoxifylline occurred in 11/40 (27.5%) patients but were mild. Pentoxifylline is concluded to be efficacious in healing of venous ulcers in patients unable to tolerate compression therapy.