Subclinical synovitis and enthesitis in psoriasis patients and controls by ultrasonography in Saudi Arabia; incidence of psoriatic arthritis during two years.

OBJECTIVE: To evaluate ultrasonographic subclinical inflammatory synovitis and enthesitis in psoriasis patients, without clinical arthritis or enthesitis compared with healthy controls, with a 2-year follow-up to study the associated incidence of psoriatic arthritis (PsA). METHODS: A total of 109 consecutive psoriasis vulgaris patients without clinical signs of PsA and 90 healthy controls were included from two tertiary medical centers. Subjects underwent dermatological examination, PASI score evaluation for severity of psoriasis, musculoskeletal examination using 68/66 joints count for tenderness and swollen joints. Patients were assessed for CRP, musculoskeletal ultrasound (MSUS) in the form of grayscale ultrasound (GSUS), and power Doppler ultrasound (PDUS) for eight entheses and 34 joints to detect MSUS subclinical enthesitis and synovitis. All patients were followed-up for 2 years to detect evolving PsA. RESULTS: Subclinical enthesitis and synovitis were detected in 39.5% of psoriasis patients and 10% of controls (P < 0.001). CRP was significantly higher in psoriasis patients with MSUS manifestations (P < 0.01). PDUS and GSUS subclinical synovitis and/or enthesitis were detected at least in one site in psoriatic patients more than in controls (P < 0.05). During a 2-year follow-up of patients, the annual PsA incidence was 4.3%. Psoriasis patients who developed PsA showed a higher prevalence of baseline enthesitis, higher PDUS and GSUS synovitis scores, and higher baseline CRP level than those who did not develop PsA. CONCLUSIONS: MSUS subclinical synovitis and enthesitis are quite common in psoriasis patients. The incidence of PsA in Saudi's psoriasis patients was slightly higher than worldwide reports. Subclinical enthesitis, PDUS, and GSUS synovitis could predict PsA development.

Homozygous Familial Hypercholesterolemia Associated with Symmetric Subcutaneous Lipomatosis.

Homozygous familial hypercholesterolemia is an autosomal dominant disorder of lipid metabolism, characterized by reduced clearance of low-density lipoprotein-cholesterol and a high risk of rapid development of cardiovascular diseases. Its incidence is relatively rare and estimated to be one in one million in general populations. Here, we report homozygous familial hypercholesterolemia in two Egyptian young siblings, presented with cutaneous, tendinous xanthomas, and corneal arcus. One of them has symmetric subcutaneous lipomatosis, which has not been reported before in association with familial hypercholesterolemia.

Immunohistochemical expression of aberrant Notch-1 signaling in vitiligo: an implication for pathogenesis.

The etiopathogenetic mechanisms leading to pigment loss in vitiligo are not fully understood. Notch signaling is required for development and maintenance of melanocyte lineage and acts as a key component among keratinocyte-melanocyte interactions. The current study aimed to investigate the possible role of Notch signaling and its effect on the whole melanocyte lineage in vitiligo and correlating it with the different clinicopathologic parameters. Using immunohistochemical technique, Notch-1 expression was evaluated in 50 lesional and 20 perilesional biopsies of patients with vitiligo in comparison with 20 normal skin biopsies as a control group. Lesional biopsies were stained with human melanoma black-45 and tyrosinase-related protein-2 to demonstrate the melanocyte lineage. Membranous and/or nuclear expression of Notch-1 was in favor of control and perilesional skin, whereas cytoplasmic expression appeared only in vitiliginous lesions (P < .05). Membranous and/or nuclear expression of Notch-1 was significantly associated with epidermal human melanoma black-45 positivity (P = .01) and percentage of expression in both epidermis (P = .02) and hair follicles (P = .03) of lesional skin. Cytoplasmic pattern of Notch-1 expression in epidermis was significantly found in lesions with white hair (P = .04) and in cases with marked keratinocyte vacuolization (P = .03). Segmental and acrofacial vitiligo were associated with mild to moderate Notch-1 intensity, whereas generalized vitiligo was associated with strong intensity of expression (P = .02). In conclusion, Notch-1 signaling is inactivated in vitiligo with consequent loss of epidermal and/or follicular active melanocytes. Aberrant Notch signaling in vitiliginous white hair and acral and segmental vitiligo may be the cause of their treatment resistance.

Immunohistochemical study of melanocyte-melanocyte stem cell lineage in vitiligo; a clue to interfollicular melanocyte stem cell reservoir.

There has been a long lasting controversy over whether melanocytes (MCs) in vitiligo are actually lost or still present but functionally inactive. We aimed to evaluate the MC cell lineage in follicular and interfollicular vitiliginous epidermis through immunohistochemical localization of Human Melanoma Black-45 (HMB-45) and Tyrosinase Related Protein 2 (TRP2) and to correlate it with clinicopathologic parameters. Using immunohistochemical techniques, skin biopsies from 50 vitiligo patients and 20 age- and gender-matched healthy subjects were examined. Differentiated active MCs were detected in 44% of interfollicular epidermis (IFE) and 46.7% of follicular epidermis (FE) in lesional skin. Melanocyte precursors/stem cells were detected in 54% of IFE and 63.3% of FE in lesional skin. Melanocyte precursors/stem cells of IFE were significantly associated with residual melanin pigment (p = 0.007) and with absence of angiogenesis (p = 0.05). HMB-45 percentage of expression in IFE was positively correlated with MC precursors/stem cells percentage in FE (r = +0.65, p < 0.001) and IFE (r = +0.33, p = 0.01). Melanocyte precursors/stem cells positivity (p < 0.001) was progressively decreasing with advanced histopathologic grading. There was no significant association between interfollicular or follicular expression of HMB-45, TRP2 or MC precursors/stem cells and the clinical type of vitiligo or its duration. In conclusion, functioning MCs may exist in vitiligo. The presence of MC precursors/stem cells in IFE may provide an additional reservoir needed for repigmentation.