Is Immune Thrombocytopenia and its Treatment Associated with Sarcopenia?

BACKGROUND: Immune thrombocytopenia (ITP) is defined as an isolated platelet count less than 100 × 109/L in the absence of other causes of thrombocytopenia. Sarcopenia is a body-wide muscular disorder with a progressive nature that leads to reduced mobility, physical disability, falls, and poor quality of life. We aimed to evaluate the frequency of objectively diagnosed sarcopenia in patients with ITP and to determine whether ITP therapies have sarcopenic effects. METHODS: This prospective study included patients who were followed up with ITP in the hematology outpatient clinic. Patients who had received corticosteroids within 3 months were excluded. The handgrip strength test, appendicular skeletal muscle mass (ASMM), ASMM/height2 value, soft lean mass (SLM), trunk soft lean mass (SLMT), and the 6-min walking speed test were applied for muscular evaluations and physical performance assessment. RESULTS: We included 53 patients (female/male: 73.58%/26.42%). While sarcopenia was not observed in 77.36% of ITP patients, possible sarcopenia was diagnosed in 9.43% and confirmed sarcopenia in 13.21%. Severe sarcopenia was not seen in any of the patients. Loss of muscle strength was observed in 22.64% of patients. SLM was found to be low in 92.45%. CONCLUSION: Sarcopenia may be more frequent among patients with ITP compared to the population, and it is important to note that 92.45% of patients had low SLM and 54.72% had low SLMT. Eltrombopag therapy might be beneficial as demonstrated by higher SLM, ASMM, and ASMM/height2 values.

Tyrosine Kinase Inhibitor Therapies in Chronic Myeloid Leukemia: Effects on Clinical Characteristics and Triglyceride-to-High Density Lipoprotein Cholesterol Ratio.

Background and Aim: Tyrosine kinase inhibitors (TKIs) have dramatically improved chronic myeloid leukemia (CML) prognosis. However, TKIs are associated with dyslipidemia and impaired glucosehomeostasis. Triglyceride-to-high-density lipoprotein cholesterol ratio (TG/HDL-C) is proposed to be an indicator of insulin resistance and atherogenic index, but there is no research on TG/HDL-C alterations in patients receiving TKIs for CML. We aimed to evaluate relationships between TKI type/count, clinical characteristics, and laboratory results (particularly TG/HDL-C) in CML patients. Patients and Methods: A total of 104 patients with chronic phase CML were enrolled in the study. All patients received initial imatinib therapy at 400 mg daily, the type or dose of TKI was then changed according to treatment response and clinical outcomes. Patients were compared with respect to TG/HDL-C categorization (>2.5 versus <2.5), number of TKIs used, and use of imatinib as the only TKI. Results: The median TG/HDL-C was 2.82 (1.03-17.33) and this ratio was higher than 2.5 in 59 (56.7%) patients. Patients with high TG/HDL-C had a significantly higher age than patients with low values (P < 0.001). Recipients of more than one TKI had higher EUTOS risk score and white blood cell (WBC) count (P < 0.05). Recipients of imatinib as the only TKI had higher age, low EOTUS risk score, low WBC, and low neutrophil count (all, P < 0.05). Conclusion: TG/HDL-C values were not associated with the number of different TKIs used or the use of imatinib only in chronic-phase patients with CML. Further large-scale prospective studies are needed to determine whether TG/HDL-C can be used for diagnostic or prognostic purposes in TKI recipients.

Unexpected clinical involvement of hereditary total leuconychia with congenital fibrosis of the extraocular muscles in three generations.

We report familial segregation of hereditary total leuconychia (HTL) with ptosis and restriction of ocular motility due to congenital fibrosis of the extraocular muscles type 1 (CFEOM1) in three generations. In this family, 4 people have HTL and ptosis, and there is restriction of ocular motility due to CFEOM1 in 10 members of the family. To our knowledge, this is the first description of familial segregation of CFEOM1 and HTL, and the second report of unexpected clinical involvement of CFEOM1. We suggest that CFEOM1 is not an isolated phenomenon in these cases. These phenotypes provide valuable insight into the function of the gene(s) localized to 12q13, giving a new perspective on the clinical component of molecular dysmorphology, but this requires further clarification.

Giant condyloma acuminata of Buschke-Löwenstein: successful treatment with a combination of surgical excision, oral acitretin and topical imiquimod.

Human papillomavirus (HPV) is the most common sexually transmitted disorder in young, sexually active populations in the USA and Europe. Giant condyloma acuminatum (GCA) is a unique variant of condyloma acuminata, characterized by local aggressive behaviour despite benign histology. It carries a substantial risk of squamous cell carcinoma. Various treatments have been used, but response is often poor and recurrence rates high. We present a case of GCA successfully treated with a combination of surgical excision, oral acitretin and topical imiquimod. The diagnosis was based on histological examination, immunohistochemical analysis and in situ hybridization for HPV 6 and 11. We recommend a trial of oral retinoid and topical imiquimod in selected cases of GCA.

Doxycycline-induced staining of permanent adult dentition.

BACKGROUND: Doxycycline is the most effective antibiotic for managing brucellosis. Although it is relatively free from side effects, complications involving the skin, nails and teeth may rarely be encountered. METHODS: Four patients with brucellosis developed yellow-brown discolouration of teeth following a 30-45 day course of doxycycline therapy during summer at a dose of 200mg/day. RESULTS: All four patients were diagnosed as having doxycycline-induced staining of the permanent dentition. In all cases, the staining completely resolved and the teeth recovered their original colour following abrasive dental cleaning. CONCLUSIONS: These observations indicate that the incidence of staining of the permanent dentition, as a complication of doxycycline, may be much higher than the literature indicates, especially if treatment is administered during summer months. Fortunately, this complication is reversible and does not require termination of doxycycline therapy. Complete resolution following abrasive cleaning may suggest that an extrinsic mechanism within the dental milieu may be involved in its pathogenesis. Strict avoidance of sunlight exposure during high-dose, long-term doxycycline therapy might prevent the development of this complication.

Cutaneous fixed drug eruption to paclitaxel; a case report.

The aim of this report was to investigate an adverse effect of paclitaxel. Cutaneous fixed drug eruption against paclitaxel--which is rarely seen--is explained. A 70-year-old caucasian woman experienced three operations for ovarian carcinoma and finally a paclitaxel-based chemotherapy regimen was initiated as adjuvant chemotherapy. Following the administration of the first dose of paclitaxel, the patient had a cutaneous reaction that was clinically consistent with "fixed drug eruption". Lesions regressed with a topical steroid dressing. Histopathological examination of the lesion confirmed clinical diagnosis. It is interesting that such a reaction could occur despite the use of premedication with systemic corticosteroid antihistaminic prophylaxis yet heal rapidly with topical corticosteroid therapy. Thus we recommend careful observation for cutaneous side-effects in patients who receive paclitaxel or other taxanes, even with premedication prophylaxis.

An unusually extensive case of cutaneous anthrax in a patient with type II diabetes mellitus.

Naturally acquired anthrax infection remains an important public-health problem in developing countries. Turkey is one of the countries in which the zoonotic form of anthrax may still be encountered. The most frequent portal of entry for anthrax spores is the skin. Although cutaneous anthrax is usually self-limiting, complications may arise in untreated cases. Underlying systemic disorders such as diabetes mellitus may confound the clinical picture and lead to atypical presentations. We present an unusually extensive case of cutaneous anthrax in a patient with newly diagnosed diabetes mellitus.

Pigmented terminal hair cysts within an intradermal melanocytic naevus.

The combination of melanocytic naevi and cysts of adnexal or epithelial origin has been described previously. Herein we describe a case of intradermal melanocytic naevus containing pigmented terminal hair cysts, a rare variant of epithelial cysts. Our observation suggests that pigmented follicular cysts and combined cystic-melanocytic lesions may be attributed to follicular occlusion.

Annular verrucous psoriasis with exaggerated papillomatosis.

Psoriasis is a disease of substantial clinical and microscopic diversity. We report a case of annular verrucous psoriasis associated with abnormal papillomatosis, resulting in finger-like projections. We believe that this finding may represent another odd histopathologic feature in psoriasis with verrucous and rupial clinical morphology.

Gianotti-Crosti syndrome preceded by oral polio vaccine and followed by varicella infection.

Gianotti-Crosti syndrome (papular acrodermatitis of childhood) is considered a nonspecific cutaneous host response to a variety of infectious agents, particularly viruses. Herein we report a case of Gianotti- Crosti syndrome preceded by oral polio vaccine and followed by varicella infection. The present case may support the hypothesis of initial immunization and following viral infection as a possible mechanism for Gianotti-Crosti syndrome.

Hepatitis C virus infection prevalence in lichen planus: examination of lesional and normal skin of hepatitis C virus-infected patients with lichen planus for the presence of hepatitis C virus RNA.

Hepatitis C virus (HCV) is the main cause of parenterally transmitted non-A, non-B viral hepatitis. In recent years, a significant association between lichen planus and chronic HCV infection has been reported. Anti-HCV antibody status was evaluated by ELISA in 54 patients with lichen planus and 54 patients with minor dermatological disorders. PCR was used to examine HCV RNA from serum and lesional and nonlesional cutaneous biopsy samples of HCV-infected patients. Seven patients with lichen planus (12.9%) and two patients in the control group (3.7%) were anti-HCV antibody positive. Five out of seven patients with anti-HCV antibodies had demonstrable HCV RNA in lesional skin biopsies. The viral RNA was absent in three out of four patients with lichen planus whose serum samples were positive for HCV RNA and agreed to biopsy of nonlesional skin. The prevalence of HCV infection is not increased in Turkish patients with lichen planus. However our findings suggest that the virus may play a potential pathogenic role by replicating in cutaneous tissue and triggering lichen planus in genetically susceptible HCV-infected patients.

Kindler syndrome: absence of definite ultrastructural feature.

Kindler syndrome is characterized by congenital blister formation, photosensitivity, poikiloderma, and cutaneous atrophy in later life. There are few reports about the ultrastructural features of this syndrome, but still there is no consensus about the basic disease. Here we report a case of Kindler syndrome with ultrastructural findings.

Examination of mycosis fungoides for the presence of Epstein-Barr virus and human herpesvirus-6 by polymerase chain reaction.

BACKGROUND: The aetiology of cutaneous T-cell lymphoma (CTCL) remains unknown despite numerous investigations. In recent years, retroviruses and human herpesviruses have been implicated to play a causal part in CTCL. OBJECTIVE: The aim of this study was to elucidate the possible aetiopathogenetic role of human herpesviruses (HHV) in mycosis fungoides (MF). METHODS: Polymerase chain reaction was used to study formalin-fixed, paraffin-embedded lesional skin biopsies from 92 subjects with MF to evidence possible presence of Epstein-Barr virus (EBV) and HHV-6. RESULTS: Biopsy specimens from nine subjects (9.8%) evidenced EBV DNA, whereas all except one of the subjects (1.1%) lacked HHV-6 DNA. CONCLUSIONS: Although these findings do not support a primary aetiological role for EBV and HHV-6 in classical CTCL, the possibility remains that both viruses, particularly EBV, may act as potential cofactors in the development of CTCL.

A case of porokeratotic eccrine ostial and dermal duct naevus of late onset.

We describe the case of a 21-year-old man with an 8-year history of porokeratotic eccrine ostial and dermal duct naevus (PEODDN) of late onset. The patient had pruritic, keratotic papules on the dorsal surface of his left hand, bilaterally on his palms and multiple yellowish lesions on the plantar surface of his feet. On histopathological examination cornoid lamella-like parakeratotic columns above eccrine sweat ducts were observed. The acrosyringium was also dilated. The lesions were refractory to treatment with salicylic acid in petrolatum and acitretin for 2 months. This entity should be taken into consideration in the differential diagnosis of linear keratotic cutaneous eruptions.

An unusual form of generalized granuloma annulare in a patient with insulin-dependent diabetes mellitus.

The generalized form of granuloma annulare may be associated with systemic disorders, including diabetes mellitus. We describe here an unusual form of generalized granuloma annulare in a patient with complicated insulin-dependent diabetes mellitus. The cutaneous eruption had been present for decades as non-pruritic, persistent, violet-brown patches with raised edges. There were flexion deformities of the small joints of the hands and feet associated with thickening of the skin over dorsa of the fingers. The patient is currently on isotretinoin therapy, with partial resolution of lesions at 3 months follow-up.