Saurian papulosis: a new clinicopathological entity.

BACKGROUND: Epidermal keratinization disorders comprise a heterogeneous group of skin diseases that share the common feature of abnormal epidermal maturation, often leading to a disturbed stratum corneum. OBJECTIVE: To describe two cases of an unusual disorder of epidermal keratinization. METHODS: The clinical features of two unrelated patients with a long-standing widespread cutaneous eruption are described. Histopathologic examination and immunohistochemical studies were performed on skin biopsy specimens. RESULTS: The eruption was characterized by symmetric erythematous, flat, discrete papules with a polygonal shape and fine scaling. The papules covered most of the skin surface and, in some areas of the trunk, they were arranged along the lines of cleavage, parallel to the ribs. There was no facial, mucosal, nail, or palmoplantar involvement; the teeth and hair were normal. The first patient had a sister with an identical eruption, and a brother of the second patient was said to have similar skin lesions. Histopathology revealed well-demarcated areas of compact eosinophilic orthokeratotic hyperkeratosis overlying a slightly acanthotic epidermis. Lesional skin showed weaker immunoexpression for connexin 43 compared with normal skin. LIMITATIONS: Only two patients and one sibling were investigated. CONCLUSION: We propose the name "saurian papulosis" to describe this newly described clinicopathologic entity.

Familial acral localized late-onset focal dermal elastosis.

Late-onset focal dermal elastosis is a rare disorder of elastic tissue, characterized by a local accumulation of elastic fibers in the mid and deep reticular dermis. This disorder occurs mainly in elderly people and consists of multiple yellow papules that are typically distributed on the sides of the neck and flexural areas, closely resembling pseudoxanthoma elasticum from the clinical point of view. We report 2 sisters who have presented with a 20-year history of multiple yellow papules, mainly located on the dorsum of their hands. They also had few lesions of similar morphology scattered on the anterior aspects of their wrists, thighs, and lower abdomen. Histopathologic study demonstrated a focal increase in thick, interlacing elastic fibers in the mid and deep reticular dermis. The most striking features of our cases were the early age of clinical presentation, the familial involvement, and the predominantly acral distribution of the lesions.

Correction: IKKα regulates the stratification and differentiation of the epidermis: implications for skin cancer development

[This corrects the article DOI: 10.18632/oncotarget.12527.]

IKKα regulates the stratification and differentiation of the epidermis: implications for skin cancer development.

IKKα plays a mandatory role in keratinocyte differentiation and exerts an important task in non-melanoma skin cancer development. However, it is not fully understood how IKKα exerts these functions. To analyze in detail the role of IKKα in epidermal stratification and differentiation, we have generated tridimensional (3D) cultures of human HaCaT keratinocytes and fibroblasts in fibrin gels, obtaining human skin equivalents that comprise an epidermal and a dermal compartments that resembles both the structure and differentiation of normal human skin. We have found that IKKα expression must be strictly regulated in epidermis, as alterations in its levels lead to histological defects and promote the development of malignant features. Specifically, we have found that the augmented expression of IKKα results in increased proliferation and clonogenicity of human keratinocytes, and leads to an accelerated and altered differentiation, augmented ability of invasive growth, induction of the expression of oncogenic proteins (Podoplanin, Snail, Cyclin D1) and increased extracellular matrix proteolytic activity. All these characteristics make keratinocytes overexpressing IKKα to be at a higher risk of developing skin cancer. Comparison of genetic profile obtained by analysis of microarrays of RNA of skin equivalents from both genotypes supports the above described findings.

Lipedematous alopecia: an uncommon clinicopathologic variant of nonscarring but permanent alopecia.

A 52-year-old black woman presented with a 5-year history of gradual swelling and slowed hair growth involving the vertex and both parietal regions of the scalp. Gradually, the swelling progressed to involve the entire scalp, only sparing a peripheral crown. She reported no history of trauma or medications. Slight pruritus of the involved area was the only accompanying symptom. There was no family history of a similar condition. Her past medical history included surgery for ovarian cysts, 10 years previously, and cholelithiasis. Physical examination revealed diffuse hair thinning and alopecia, more prominent along the vertex and parietal regions (Fig. 1a). There was no evidence of scalp inflammation, scaling, or increased hair fragility. The scalp was mildly tender on palpation and had a boggy, spongy consistency. The hairs which still remained in the involved areas were thin, short, and soft (Fig. 1b). The involved area was slightly hypopigmented when compared with adjacent noninvolved scalp. The rest of the physical examination was within normal limits. No abnormalities of the hair shaft were observed on microscopic examination of several plucked hairs. Laboratory investigations, including a complete blood cell count, blood chemistry, urinalysis, sedimentation rate, antinuclear antibodies, and serologic tests for syphilis, hepatitis B and C virus, and human immunodeficiency virus, were negative. A computed tomography scan of the skull demonstrated diffuse and regular thickening of subcutaneous fatty tissue of the scalp, disclosing a maximum scalp thickness of 15 mm at the vertex (Fig. 2). The biopsy from the vertex revealed a normal epidermis and dermis, with diffuse loss of hair follicles. The most striking feature consisted of a large increase in thickness of the subcutaneous fatty tissue (Fig. 3a). Pre-existing hair follicles were replaced by vertical fibrous tracts of lamellar fibroplasia with no inflammatory infiltrate (Fig. 3b). Adipocytes showed a normal size and shape, but the connective tissue septa, which are normally present separating the subcutaneous tissue into fat lobules, were lacking, and subcutaneous fatty tissue consisted of a continuous and diffuse sheet of mature adipocytes (Fig. 3c). Orcein stain revealed normal contents of elastic fibers with foci of condensation at the sites of disappeared pre-existing hair follicles (Fig. 3d). Colloidal iron and Alcian blue (pH 2.5) stains revealed no mucin deposits.

Facial granulomas secondary to Dermalive microimplants: Report of a case with histopathologic differential diagnosis among the granulomas secondary to different injectable permanent filler materials.

Wrinkle reduction and the correction of skin defects using injectable aesthetic microimplants are now widely performed by dermatologists and plastic surgeons. In recent years, dermal filler substances containing polymer particle suspensions such as Bioplastique, Artecoll, and Dermalive are the most commonly used materials. These microimplants are permanent, non-biodegradable, and generally well tolerated, although various adverse reactions are still possible. We describe here a patient with facial granulomas secondary to Dermalive injections for correction of naso-labial folds and wrinkles. The particular shape of the injected particles allows for correct identification of the implanted material. Therefore, histopathologic examination is the best means to obtain the correct diagnosis of foreign body granuloma and to identify the type of filler particles. We discuss the histopathologic differential diagnosis among the granulomas secondary to the most commonly used aesthetic permanent filler materials.