Merkel cell number and distribution, and CD200 expression in patients with lichen planopilaris and discoid lupus erythematosus.

BACKGROUND: Immune mechanisms are considered to be responsible for the pathogenesis of cicatricial alopecia in lichen planopilaris (LPP) and discoid lupus erythematosus (DLE) diseases. CD200 has an immunomodulatory function in hair follicles. The functions of Merkel cells (MCs) in hair follicles remain to be fully understood. OBJECTIVE: This study aimed to determine the number and distribution of MCs as well as CD200 expression in patients with DLE and LPP. METHODS: Using immunohistochemistry, the number and distribution of MCs (staining with CK20) and CD200 expression in biopsy specimens of LPP and DLE patients were compared with control group patients. RESULTS: The number of follicular MCs, total MCs, mean follicular MCs, and CD200 expression were significantly lower in the case groups compared to the control group. In CD200- cases, the number of follicular MCs and mean follicular MCs were significantly lower than in CD200+ cases. Retrospective design, lack of data regarding the history of alopecia in the control group, and unknown stage of disease in patients were the limitations. CONCLUSION: MC loss might play a role in immune privilege collapse in hair follicles. This study is novel in terms of investigating MCs in DLE and LPP patients.

Age and psoriatic arthritis are important predictors of biologic agent switch in psoriasis.

BACKGROUND: The demand of biologic switching is increasing for different reasons.We aimed to define reasons for switching biologics and possible predictors for switching risk,and to estimate data on drug survival. METHODS: 115 patients treated with biologics who switched to a second, third,and/or fourth biologic were eligible for this retrospective study.Patients were divided into 2 groups as switched once,and switched twice or more.Drug survival rates were calculated using the Kaplan-Meier method. RESULTS: All patients switched at least one, 36 patients switched twice, and 9 switched thrice. First-, second-, and third-line biologics were mostly switched due to secondary lack of efficacy for skin disease.Each unit increase in age decreased the risk of having ≥2 switches 4% (p=0.038,OR:0.964,95%CI:0.93-0.998),whereas PsA increased the risk of having ≥2 switches 2.69-fold (p=0.026,OR:2.69,95%CI:1.12-6.44).There was significant difference between biologics in terms of drug survival(p=0.001).Adalimumab had a lower drug survival compared to ustekinumab(p<0.001) and secukinumab(p=0.003) in transition from second-line biologic to third-line biologic. CONCLUSION: Switching biologics was most commonly due to secondary lack of efficacy for skin disease.Lower ages and the presence of PsA were associated with a higher need for switching in long-term.Ustekinumab and secukinumab are superior to adalimumab in clinical practice in terms of drug survival of second-line biologics.

The effect of warts on quality of life in Turkish pediatric patients.

BACKGROUND: The negative effect of genital and extragenital warts on adult patient quality of life (QoL) is well known; however, the literature lacks data on the effect of extragenital warts on Turkish pediatric patient QoL. The aim of this study was to determine the effect of extragenital warts that persist for ≥6 months on Turkish pediatric patient QoL, as well as to determine the relationship between patient demographic and clinical characteristics, and QoL. METHODS: The Pediatric Quality of Life Inventory Version 4.0 (PedsQLTM 4.0) was administered to 85 children and their parents (patient group), and 85 age- and gender-matched children without any skin disease and their parents (control group). Children`s Dermatology Life Quality Index (CDLQI) was administered to the patients. Higher CDLQI and PedsQLTM are indicative of lower QoL. RESULTS: Median wart duration and median age at the time of wart onset was 12 months (range 6-84) and 10 years (range 1-16), respectively. In the patient group mean (±Standard deviation [SD]) CDLQI score was 5.20 ± 5.97, and warts had the greatest negative effect on CDLQI symptoms and feelings scores. Mean (±SD) PedsQLTM total score was higher in the affected patients than that for the controls (23.42 ± 12.33 versus 15.81 ± 7.37, P < 0.001), and school, social and emotional functionality subscales exhibited the greatest differences between these groups. Mean (±SD) PedsQLTM total score for the patients` parents was higher than that for the controls` parents (25.94 ± 12.49 versus 17.81 ± 6.87, P < 0.001), and social and emotional functionality subscales exhibited the greatest difference between these groups. CONCLUSIONS: The findings show that Turkish children with warts that persist for ≥6 months had lower QoL than the controls.

Acquired reactive perforating collagenosis in association with prostate adenocarcinoma, chronic lymphocytic leukemia, and Graves disease

Abstract Acquired reactive perforating collagenosis is a rare skin disorder characterized by the presence of umbilicated pruritic papules and nodules. Transepidermal elimination of altered and perforating bundles of basophilic collagen from the epidermis is a characteristic histologic feature of acquired reactive perforating collagenosis. Along with its well-known association with systemic diseases such as diabetes mellitus, chronic renal failure, and dermatomyositis, there are reports of acquired reactive perforating collagenosis being associated with malignancies. Herein, we present a case of acquired reactive perforating collagenosis associated with chronic lymphocytic leukemia, prostate adenocarcinoma, and Graves's disease. Clinicians are required to be more vigilant in evaluating patients with acquired reactive perforating collagenosis due to its unique association with malignancies and other systemic diseases.

Acquired reactive perforating collagenosis in association with prostate adenocarcinoma, chronic lymphocytic leukemia, and Graves' disease.

Acquired reactive perforating collagenosis is a rare skin disorder characterized by the presence of umbilicated pruritic papules and nodules. Transepidermal elimination of altered and perforating bundles of basophilic collagen from the epidermis is a characteristic histologic feature of acquired reactive perforating collagenosis. Along with its well-known association with systemic diseases such as diabetes mellitus, chronic renal failure, and dermatomyositis, there are reports of acquired reactive perforating collagenosis being associated with malignancies. Herein, we present a case of acquired reactive perforating collagenosis associated with chronic lymphocytic leukemia, prostate adenocarcinoma, and Graves's disease. Clinicians are required to be more vigilant in evaluating patients with acquired reactive perforating collagenosis due to its unique association with malignancies and other systemic diseases.

Childhood-Onset Keratosis Lichenoides Chronica Accompanied by Severe Hair Loss.

A 37-year-old woman attended the dermatology outpatient clinic because of recent hair loss from the eyebrows and axillae. Her past medical history revealed mild generalized erythema and hyperpigmented papules and plaques since childhood. On dermatologic examination, there were flat-topped, purple to brown hyperkeratotic lichenoid papules and linear plaques on the elbows, trunk, and buttocks, some of which coalesced into hyperpigmented reticular plaques on the axillae, neck, and groin. Mild erythema was noted. There was thinning and loss of hair of the eyebrows; severe loss of hair was noted in the axillae and genital regions (Figure 1). One of the lichenoid papules was biopsied. The specimen showed histopathologic findings of focal parakeratosis, irregular acanthosis, an increased granular layer, and focal vacuolar degeneration of the basal layer. Necrotic keratinocytes were also observed. Hyalinization and abundant melanin in the papillary dermis and marked congestion of blood vessels were noted (Figure 2). Clinicopathologic correlation of the case was consistent with keratosis lichenoides chronica (KLC).

Clinical and Laboratory Characteristics of Patients With Erythema Nodosum.

Erythema nodosum (EN) represents an acute, erythematous nodular eruption that is generally found on the lower aspects of the legs. Despite the variety of triggering factors, the clinical findings of EN are classic. It is often hard to determine which patients have an underlying systemic disorder. The aim of this study was to investigate clinical and laboratory parameters in patients with EN, especially those with an underlying systemic disorder. A total of 43 patients diagnosed with EN at an adult and children's hospital were retrospectively reviewed for triggering factors, any underlying systemic diseases, clinical features, laboratory parameters, treatment modalities, and disease outcome. The mean age of the patients was 40.91±15.57 years (minimum 7 years, maximum 71 years). Patients with an underlying systemic disorder were grouped as complicated EN (CEN), patients without an underlying disorder were grouped as non-complicated EN (NCEN). Patients with EN more frequently presented with more nonpretibial localizations than patients with NCEN (P=.023). Platelet levels in patients with CEN were significantly higher than in patients with NCEN (P=.036). Erythrocyte sedimentation rate, C-reactive protein, and procalcitonin levels did not differ among the two groups (P>.05). Hospitalization shortened the active disease duration (P=.046). EN lesions present on the nonpretibial area, which may be a clue for systemic associations of the disease. The presence of elevated platelet levels may indicate systemic inflammatory and infectious diseases in patients with EN. Procalcitonin, which is a marker for systemic infection, was not helpful in detecting chronic infections such as tuberculosis or systemic fungal infections in patients.

Molecular analysis of Chanarin-Dorfman syndrome (CDS) patients: Identification of novel mutations in the ABHD5 gene.

Chanarin-Dorfman syndrome (CDS) is an autosomal recessive metabolic disorder associated with congenital ichthyosis and a multisystemic accumulation of neutral lipids in various types of cells. Recently, mutations of the ABHD5 gene were identified as the cause of CDS. In this work, we carried out molecular analysis of the ABHD5 gene in 6 unrelated patients. We identified one previously reported mutation, N209X and two novel genetic alterations; a nonsense mutation (p.Y50X) and missense mutation (p.S73A).

Extranodal type T/NK-cell lymphoma with an atypical clinical presentation.

Peripheral-type natural killer (NK)- or T-cell lymphomas are rare disorders characterized with clonal proliferation of mature lymphocytes. They have been linked to chronic and active Epstein-Barr virus infection (CAEBV), which itself is not defined as a malignant hematological disorder. The authors present a patient with T/NK-cell lymphoma involving skin, kidneys, spleen, pancreas, and meninges. She was remarkable for having the mosaic feature of more than one type of extranodal T/NK-cell lymphoma. She also had mixed findings of CAEBV that might have been attributed both to hypersensitivity to mosquito bites and to hemophagocytic lymphohistiocytosis.

In search of an optimum dose escalation for narrowband UVB phototherapy: is it time to quit 20% increments?

OBJECTIVES: Our aim was to compare 20% with 10% to 5% incremental regimens in narrowband UVB phototherapy. STUDY PATIENTS: The study included patients with psoriasis (N = 191) with Fitzpatrick skin phototypes II and III. RESULTS: Occurrence of erythema as well as maximum and cumulative doses were higher with 20% escalations, whereas response rates and time to response did not significantly differ. LIMITATIONS: The study was limited by its retrospective nature. CONCLUSION: A 5% or 10% incremental regimen may have similar therapeutic efficacy.