Giant Plexiform Neurofibroma of the Perineum and Pelvic Cavity Manifesting as Segmental Neurofibromatosis

Segmental neurofibromatosis (SN) is rare form of neurofibromatosis characterized that cutaneous or neural changes are limited to one region of the body. SN present neurofibroma and less frequently, café au lait macules (CALMs) on usually unilateral or rarely bilateral of the body region. SN seems to have fewer systemic complications than neurofibromatosis type I or II, except patients with plexiform neurofibromas (PNFs). PNFs are rare benign peripheral nerve sheath tumors which arise from single or multiple nerves. PNFs can easily become aggressive growth particularly during puberty or pregnancy and leading to disfigurement and functional impairment. Also, PNFs can transform to malignant peripheral nerve sheath tumor, higher rate than classic neurofibroma. So, it is important to decide appropriate treatment modalities and time to intervention.     

A Case of Isolated Plexiform Neurofibroma in a Patient with Myasthenia Gravis

We report a case of an isolated plexiform neurofibroma occurring in a patient with myasthenia gravis. A 48-year-old man presented with asymptomatic skin-colored nodules on the tip of his 4th finger. Microscopically, a plexiform neurofibroma was identified located in the dermis that appeared to originate from small superficial nerves. He had a 20-year history of treated myasthenia gravis; otherwise, his personal and family histories were unremarkable. Given that myasthenia gravis is a disorder of the peripheral nerves, plexiform neurofibromas could be associated with myasthenia gravis. However, the development of an isolated plexiform neurofibroma in a case of myasthenia gravis has not yet been reported. The occurrence of a neurofibromas in a patient with myasthenia gravis suggests a link in the pathogenesis of these two diseases.     

Plexiform melanocytic schwannoma: a mimic of melanoma

We present a unique dermal tumor for which we propose the term plexiform melanocytic schwanomma. The proliferation consisted of lobules of epithelioid and spindled cells with S100, Melan-A and HMB-45 positivity but without obvious melanin pigmentation. The nuclei were moderately pleomorphic in some areas, and in a few areas the mitotic index was elevated. Schwannian differentiation was inferred from the presence of areas with nuclear palisading resembling Verocay bodies, from plexiform architecture and from the presence of a thin rim of EMA positivity around the tumor. Array-based comparative genomic hybridization showed genomic losses that overlap with those seen in sporadic schwanomma. The differential diagnosis included melanoma, melanotic schwannoma and cutaneous melanocytoneuroma, and we compare and contrast our case with these entities.     

Cutaneous Angiomyolipoma Masquerading as a Neurofibroma in a Child with Neurofibromatosis Type 1: A Case Report

Angiomyolipomas are benign tumors composed of blood vessels, smooth muscle, and adipose tissue that are frequently associated with tuberous sclerosis. Cutaneous angiomyolipomas are rare and typically present in men in the fifth or sixth decade of life. We present a case of a cutaneous angiomyolipoma masquerading as a neurofibroma in a 13‐year‐old boy with a history of neurofibromatosis type 1.     

Hair cycle control by leptin as a new anagen inducer

Our purpose is to clarify the physiological role of leptin in hair cycle as leptin reportedly causes activation of Stat3, which is indispensable for hair cycling. While hair follicles in dorsal skin of 5‐week‐old C57/BL6 mice had progressed to late anagen phase, those in dorsal skin of 5‐week‐old leptin receptor deficient db/db mice remained in the first telogen and later entered the anagen at postnatal day 40, indicating that deficiency in leptin receptor signalling delayed the second hair cycle progression. Next, we shaved dorsal hairs on wild‐type mice at postnatal 7 weeks and injected skin with mouse leptin or a mock. After 20 days, although mock injection showed no effect, hair growth occurred around leptin injection area. Human leptin fragment (aa22–56) had similar effects. Although the hair cycle of ob/ob mice was similar to that of wild‐type mice, injection of mouse leptin on ob/ob mice at postnatal 7 weeks induced anagen transition. Immunohistochemically, leptin is expressed in hair follicles from catagen to early anagen in wild‐type mice, suggesting that leptin is an anagen inducer in vivo. Phosphorylation of Erk, Jak2 and Stat3 in human keratinocytes was stimulated by leptin and leptin fragment. In addition, RT‐PCR and ELISA showed that the production of leptin by human dermal papilla cells increased under hypoxic condition, suggesting that hypoxia in catagen/telogen phase promotes leptin production, preparing for entry into the next anagen. In conclusion, leptin, a well‐known adipokine, acts as an anagen inducer and represents a new player in hair biology.     

Acral Plexiform Palisaded Encapsulated Neuromas as the Initial Cutaneous Manifestation of Cowden Syndrome

Cowden syndrome (CS) is an autosomal dominant genodermatosis associated with characteristic mucocutaneous findings of facial trichilemmomas, palmoplantar keratoses, sclerotic fibromas, and oral papillomas. Mucocutaneous neuromas have also been reported in association with CS. We describe a patient with CS whose sole cutaneous finding was palisaded encapsulated neuromas (PENs) with a plexiform growth pattern in an acral location. Along with previous reports, this case suggests that acral plexiform PENs may be an early, highly specific finding in CS and highlights the importance of screening these patients for PTEN mutation.     

头顶部皮下神经纤维瘤一例

患者，女，43岁。无症状头部肿物2年余。皮肤科检查：头顶部可触及一个约花生米大类圆形皮下肿物，皮肤颜色正常，表面光滑，质中，活动性差，有轻压痛。全身浅表淋巴结未扪及增大。组织病理诊断：神经纤维瘤。该肿物已手术完整切除。     

Tissue Eosinophilia as an Indicator of Drug‐Induced Leukocytoclastic Vasculitis: A Clinicopathologic Investigation

Cutaneous leukocytoclastic vasculitis (LCV) may be idiopathic, drug‐induced, related to infection, or may occur in patients with autoimmune disorders. Our objective was to determine whether tissue eosinophilia is a reliable indicator of a drug‐induced etiology in the setting of LCV. Thorough chart investigation of 63 patients with LCV was performed with division of patients into drug‐induced and non‐drug‐induced groups. Corresponding histopathologic material was reviewed by a dermatopathologist blinded to the etiologic associations. An eosinophil score was tabulated by averaging the number of eosinophils in 10 high‐power fields (400x) and dividing by the density of inflammation. Statistical analysis was performed using the Wilcoxin rank sums test. Mean eosinophil scores in the drug‐induced (n = 16) and non‐drug‐induced (n = 47) groups were 5.20 and 1.05 respectively (p = 0.0126). Vascular fibrin deposition and epidermal changes were present in both groups. Clinical evidence of systemic vasculitis was present in 6%(1/16) of the drug‐induced group vs. 38%(18/47) of the non‐drug‐induced cases. This study establishes tissue eosinophilia as a reliable indicator of drug induction in the setting of LCV. Furthermore, this information may be useful for guiding management decisions, especially in settings of unclear etiology.     

Hair growth inhibition as a method of screening drugs for local antimitotic activity.

The intradermal injection of certain drugs with antimitotic properties in the guinea pig resulted in localized areas of reversible hair loss or hair growth inhibition. The size of the affected field was related to the dose. This may provide a useful and simple method for the rapid screening of potentially useful agents for local cytostatic activity.     

Hair shaft miRNA‐221 levels as a new tumor marker of malignant melanoma

miRNA‐221 (miR‐221) is known to be abnormally expressed in many human cancers. The serum levels of miR‐221 have been reported as a tumor marker for malignant melanoma (MM). We hypothesized that the hair shaft miR‐221 levels may be increased in patients with MM. We therefore assessed the possibility that hair shaft miR‐221 levels could be a marker for MM. The hair shaft miR‐221 levels were significantly higher in patients with MM than controls. The rates of increased hair shaft miR‐221 levels above the cut‐off value were comparable to those of serum 5‐S‐CD, which is a tumor marker commonly used for MM. Measurements of the hair shaft miR‐221 levels could have potential clinical value in the detection of MM. This is the first report investigating the hair shaft levels of an miRNA in patients with MM. Our investigations offer new insight into the relationship between miR‐221 and MM, and may provide a new, non‐invasive way to screen for melanoma.     

Hair bundles: a presentation of folliculitis

Case reports of three patients with folliculitis of the posterior scalp are presented. In each an abnormality of hair growth was observed in the affected area in which bundles of hairs emerged from each follicle. The individual hair shafts came from independent papillae but converged into a single infundibulum in the upper dermis, and there was an associated granulomatous inflammation. This type of compound follicle with staphylococcal infection has rarely been reported in man before. Some of the mechanisms by which compound follicles can be formed are discussed.     

The Medical and Psychosocial Associations of Alopecia: Recognizing Hair Loss as More Than a Cosmetic Concern

Alopecia encompasses a broad range of hair loss disorders, generally categorized into scarring and non-scarring forms. Depending on the specific pathogenesis of hair loss and geographic location, a number of psychiatric and medical comorbidities, including but not limited to thyroid disease, lupus erythematosus, diabetes mellitus, atopic dermatitis, sinusitis, coronary artery disease, anxiety, depression, and suicidality, have been identified in association with alopecia. In addition to the numerous associated comorbid conditions, patients with alopecia report decreased quality-of-life measures across symptomatic, functional, and global domains. While alopecia can affect patients of all ages, genders, and ethnicities, hair loss may more significantly impact women as hair represents an essential element of femininity, fertility, and female attractiveness in society. Individuals of lower socioeconomic status may also face health disparities in the context of alopecia as a majority of hair loss treatments are considered cosmetic in nature and accordingly are not covered by third-party insurance providers. Although traditionally thought of as a merely aesthetic concern, alopecia encompasses a significant burden of disease with well-defined comorbid associations and genuine psychosocial implications, and thus should be assessed and managed within a proper medical paradigm.

     

Woolly Hair Nevus with an Ipsilateral Associated Epidermal Nevus and Additional Findings of a White Sponge Nevus

Abstract:  We report a case of a 16-year-old male with a woolly hair nevus, an associated ipsilateral epidermal nevus who also had a white plaque on his tongue, clinically diagnosed as a white sponge nevus. The concurrent findings of a white sponge nevus, a woolly hair nevus, and an ipsilateral epidermal nevus, to our knowledge, have not been previously reported.