Ichthyosiform sarcoidosis: report of three cases from Japan and literature review.

Cutaneous lesions of sarcoidosis present with various manifestations including specific and non-specific cutaneous lesions. Ichthyosiform sarcoidosis is a rare form of cutaneous sarcoidosis, presenting with asymptomatic, adherent, polygonal scales, mainly appearing on the lower limbs.  Ichthyosiform sarcoidosis has a predilection for dark-skinned races, and cases affecting Japanese patients have rarely been reported in English literature.  We herein describe three Japanese cases of ichthyosiform sarcoidosis on the lower limbs. All of the patients were female, with an age range of 57-69 years old.  Histologically, sarcoidal granulomas were located in the mid- to lower dermis.  All cases had scar sarcoidosis on the knees.  Furthermore, Case 1 presented with papular sarcoidosis on the back, and Case 3 presented with subcutaneous nodules on the buttock as well as erythema nodosum-like lesions on the lower legs.  All patients had lung sarcoidosis, but ocular sarcoidosis was seen in only Case 2. Case 3 showed Heerfordt syndrome with facial nerve paralysis. Histological features showed that the granular layers were scarcely detected in the overlying epidermis; however, filaggrin expression was not decreased.  Sarcoidal granulomas accumulated around the sweat glands in one case, whereas those features were not detected in the other two cases. In conclusion, ichthyosiform cutaneous sarcoidosis may be overlooked or misdiagnosed as xerotic dry skin which is frequently found in elderly people, and ichthyosiform cutaneous lesions may be more prevalent than previously estimated.

Clinicopathological analysis of mechanic's hand associated with dermatomyositis.

Mechanic's hand is often seen in the fingers of patients with dermatomyositis and is frequently associated with anti-aminoacyl-transfer RNA synthetase autoantibodies and interstitial lung disease. We analysed the clinical symptoms of 50 patients with dermatomyositis who had visited our department, 26 of whom also had mechanic's hand. A histological examination was carried out in 16 of the 26 cases, which revealed hyperkeratosis in all cases and colloid bodies in the epidermis in 15 cases. The number of cases of interstitial lung disease in patients with mechanic's hand (22/26, 85%) was significantly higher than that in those without mechanic's hand (12/24, 50%) (P < 0.05). Mechanic's hand is an important skin lesion of dermatomyositis, and increases the likelihood of interstitial lung disease.

Serum levels of GRO-α are elevated in association with disease activity in patients with Behçet's disease.

BACKGROUND: Although the etiology of Behçet's disease (BD) is still unknown, neutrophils are implicated in its pathogenesis. Growth-related oncogene-α (GRO-α) is a potent chemoattractant and activator for neutrophils. OBJECTIVE: To determine the role of GRO-α in the pathogenesis of BD, we investigated serum GRO-α levels in patients with BD. MATERIALS AND METHODS: Sera from patients with BD (n=57) and control subjects (n=26) were measured by enzyme-linked immunosorbent assay. Serum levels of GRO-α were compared with clinical symptoms. RESULTS: Patients with BD had significantly elevated serum GRO-α levels compared with healthy controls (121.7±79.2pg/ml vs. 75.9±20.6pg/ml, P<0.01). Concerning the subgroups of BD, serum GRO-α levels in active patients with BD (n=35) were significantly higher than in inactive patients with BD (n=22; 139.0±92.8 pg/ml vs. 94.3±36.2pg/ml, P<0.05). Also, as seen in previous studies, serum interleukin-8 levels in patients with BD (52.4±81.8pg/ml) were significantly higher than in controls (13.9±19.7pg/ml, P<0.01). Enhanced GRO-α levels correlated with clinical symptoms such as erythema nodosum. CONCLUSION: Our results indicate that serum levels of GRO-α are elevated in patients with active stage BD, suggesting that GRO-α may serve as a reliable marker for disease activity of BD.