Unilateral segmental Darier disease following Blaschko lines: A case report
Journal Title: Nasza Dermatologia Online - Year 2017, Vol 8, Issue 3
Abstract
Darier disease is an autosomal-dominant disorder of keratin production which leads to a loss in epithelial adhesion and abnormal keratinization. The clinical correspondence is keratotic papules grouped in sebaceous areas of trunk, scalp, forehead and flexures. It is a rare disease and the variant focused on here of unilateral segmental distribution following the lines of Blaschko is rarer still, considering the fact that this presentation counts for only 10% of this already uncommom disease and with only 40 cases being reported in English medical literature. Mutation in this gene is expressed in the skin and brain. The treatment of Darier disease can be challenging and is often difficult and sometimes unsatisfactory. Systemic retinoids are considered the drug of choice for treating Darier disease. However, their use is limited by potential side effects. We described the case a metalworker male with unilateral segmental Darier disease following Blaschko lines and we review the literature on this subject.
Authors and Affiliations
César Bimbi, Piotr Brzeziński Brzeziński
Keywords
Related Articles
- EP ID EP286964
- DOI 10.7241/ourd.20173.93
- Views 130
- Downloads 0
Spectrum of viral skin infections in patients attending the dermatology clinic at King Fahd Hospital of the University in Alkhobar, Kingdom of Saudi Arabia during the period 2010 – 2014
Introduction: Viral skin infections are an important disease group in the dermatology field. Many factors play a role in the incidence of skin diseases. There are only a few reports of surveys conducted on the prevalence...
Infeccion cutanea por mycobacterium fortuitum [Cutaneous infection by mycobacterium fortuitum]
Mycobacteria are aerobic, non-spore forming, gram positive, acid-fast bacilli, which affect skin, subcutaneous tissue, and other organs and systems. Mycobacterium fortuitum produces cellulitis, abscesses, papules-pustule...
Metastatic melanoma masquerading as a furuncle
Melanoma metastasizes to the skin in about 10-17% of patients. Although there are reports of metastatic melanoma masquerading as panniculitis and erysipelas, it is very uncommon for it to present as an inflammatory skin...
A rare case of familial reactive perforating collagenosis
Reactive perforating collagenosis is a rare disorder of transepidermal elimination in which genetically altered collagen is extruded out through the epidermis. This disease usually starts as asymptomatic or pruritic umbi...
Bazex syndrome - a case report
Acrokeratosis paraneoplastica of Bazex is a rare but distinctive paraneoplastic dermatoses. This condition is a cutaneous marker of supra-diaphragmatic neoplasia. This syndrome is important because the cutaneous finding...