Generalized epidermolytic ichthyosis with palmoplantar hyperkeratosis.

Epidermolytic ichthyosis (EI, OMIM 113800) is a rare autosomal dominant keratinization disorder that is caused by keratin 1 or 10 gene mutation. It can be classified clinically based on the presence of palmoplantar hyperkeratosis involvement and extent of skin involvement. The diagnosis is made by clinical and histopathological examinations that can be confirmed by genetic testing.

Acquired Human Papilloma Virus Type 6-Associated Epidermodysplasia Verruciformis in a Patient With Systemic Lupus Erythematosus.

Although historically known as a genetic disorder, epidermodysplasia verruciformis (EV) might be acquired in patients with a noninherited defective cell-mediated immunity. This article reports a case of EV in a patient with systemic lupus erythematosus and a history of 3 years immunosuppressive methylprednisolone treatment. The microscopic features of the skin biopsy showed morphologic changes of the keratinocytes characteristic of human papilloma virus (HPV) infections and immunoreactivity to p16.

Concurrence of Acrodermatitis Enteropathica and Eczema Herpeticum in a Child with Atopic Dermatitis.

Acrodermatitis enteropathica (AcE) is a rare, autosomal recessive inherited disorder caused by mutation of the gene coding for zinc transport protein (ZIP 4). The disease appears during childhood especially in breastfeeding or post-breastfeeding infant. Eczema herpeticum refers to a disseminated skin infection of herpes simplex virus that usually leads to vesicular eruptions commonly seen on a background of atopic dermatitis (AD).

Identification of novel homozygous SLURP1 mutation in a Javanese family with Mal de Meleda.

Background: Mal de Meleda (OMIM# 248300; keratosis palmoplantaris transgrediens) is an autosomal recessive form of palmoplantar keratoderma, clinically characterized by sharp demarcated erythema and hyperkeratosis of the palms and soles that progress with age and extend to the dorsal aspects of the hands and feet. The mal de Meleda is caused by mutations in the SLURP1 gene that encodes secreted lymphocyte antigen 6/urokinase-type plasminogen receptor-related protein 1 (SLURP1). To date no reported cases from Indonesia.

Topical Timolol Maleate 0.5% for Infantile Hemangioma: Its Effectiveness Compared to Ultrapotent Topical Corticosteroids - A Single-Center Experience of 278 Cases.

Background: Infantile hemangioma (IH) may have implications on parental distress and cosmetic disfigurement. To date, ultrapotent corticosteroids are used as a treatment of choice for superficial IH. However, due to their side effects and sometimes lack of IH regression, it is necessary to find alternative topical therapies.

Cellular viability, collagen deposition, and transforming growth factor β1 production among ultraviolet B-irradiated keloid fibroblasts.

Background: A keloid is a fibrous tumor produced by fibroblast hyperproliferation and excessive collagen accumulation due to overproduction of transforming growth factor β1 (TGF-β1). High keloid incidence is found among individuals with unexposed skin, especially in Negroid and Mongoloid people with high melanin contents in their skin. Because melanin serves as an ultraviolet B (UVB) light absorber, it is assumed that lack of UVB light penetration may play a role in the keloid pathomechanism.

The effect of 50% glycolic acid on the percutaneous absorption of eutectic mixture of local anesthetics (EMLA): a study of the electrofulguration-induced pain.

Background: EMLA has a slow onset due to its limited percutaneous absorption into an intact skin, while Glycolic acid (GA) has been known to have the capability of disrupting the skin barrier function. To the best of our knowledge, the effect of 50% GA on the percutaneous absorption of EMLA has not been studied previously.

Methods: The study used a two-step randomized double blind controlled trial involving 20 healthy subjects each.