SciCombinator

Discover the most talked about and latest scientific content & concepts.

Journal: Clinical and experimental dermatology

28

Idiopathic pure sudomotor failure (IPSF) is a subgroup of acquired idiopathic generalized anhidrosis, which is characterized by early age of onset, acute or sudden onset, concomitant sharp pain or cholinergic urticaria over the entire body, absence of autonomic dysfunction other than generalized anhidrosis, raised serum IgE level, and marked response to steroid. The aetiology of IPSF is still not well understood, but is thought to be caused by interference in cholinergic transmission in the eccrine glands of skin. IPSF after viral infection has rarely been reported in the literature. We describe a patient who developed generalized anhidrosis and cholinergic urticaria accompanied by heat intolerance after infectious mononucleosis infection. This is the first such case, to our knowledge, and the patient was successfully treated with steroid pulse therapy.

Concepts: Infectious disease, Cholinergic urticaria, Immunoglobulin E, Medical terms, Infection, Malaise

28

Background.  In previous studies, we made the unexpected finding that in mice, ultraviolet (UV)B irradiation of the eye increased the concentration of α-melanocyte-stimulating hormone (α-MSH) in plasma, and systemically stimulated epidermal melanocytes. Aims.  To compare the extent of the pigmentation induced by social and restraint stress (which activate the hippocampus-pituitary system) with that induced by UVB irradiation. Methods.  DBA/2 and sham-operated or hypophysectomized DBA/2 mice were subjected to local UVB exposure using a sunlamp directed at the eye, and two types of stress (social and restraint) were imposed. Results.  UVB irradiation of the eye or exposure to stress loading both increased the number of Dopa-positive melanocytes in the epidermis, and hypophysectomy strongly inhibited the UVB-induced and stress-induced stimulation of melanocytes. Irradiation of the eye caused a much greater increase in dopamine than did the stress load. Both UVB eye irradiation and stress increased the blood levels of α-MSH and adrenocorticotropic hormone (ACTH). In addition, the increase in plasma α-MSH was greater in animals subjected to UVB eye irradiation than in those subjected to stress loading, whereas the reverse occurred for plasma ACTH. UVB irradiation to the eye and stress loading increased the expression of prohormone convertase (PC)1/3 and PC2 in the pituitary gland. The increase in expression of pituitary PC2 was greater in animals subjected to UVB eye irradiation than to stress, whereas no difference was seen between the two groups for the increase in PC1/3. Conclusions.  UVB eye irradiation exerts a stronger effect on pigmentation than stress loading, and is related to increased levels of α-MSH and PC2.

Concepts: Skin, ACTH stimulation test, Sun, Sun tanning, Sunlight, Hypothalamus, Melanocyte-stimulating hormone, Melanin

27

The potent biomedical properties of Streptomyces secondary metabolites make them a possible source of promising new targets to use as wound-healing-promoting agents.

Concepts: Secondary metabolite, Metabolism

26

Chronic telogen effluvium (CTE), a poorly understood condition, can be confused with or may be a prodrome to female pattern hair loss (FPHL). The pathogenesis of both is related to follicle cycle shortening and possibly to blood supply changes.

Concepts: Alopecia, Symptoms, Immune system, Telogen effluvium

26

Naevus spilus (NS) is a naevoid disorder characterized by hyperpigmented macules or papules scattered over a café-au-lait macule. Such café-au-lait macules are often present at birth, and the darker pigmented speckles of NS slowly increase in number and size over a period of several years. NS can therefore be difficult to evaluate clinically for the development of melanoma. In vivo confocal microscopy (IVCM) is a novel method that allows examination at cellular resolution of cutaneous lesions in vivo. IVCM has been shown to have twice the specificity of dermoscopy for the diagnosis of melanoma, with comparable sensitivity. It has been shown to be useful in the detection and grading of dysplastic naevi, which are recognized precursors of melanoma in some cases. In this report, we highlight that IVCM can also be used as a tool complementary to dermoscopy to identify areas of dynamic change in clinically and dermoscopically equivocal lesions. IVCM may thereby assist in the early detection of melanocytic atypia and melanoma arising in NS, in turn leading to excision of melanoma at an early stage, which is associated with a favourable outcome. We also outline some of the difficulties encountered in confocal microscopy and histology when differentiating melanoma from dysplastic naevi.

Concepts: Cancer, Microscopy, Melanoma, Nevus, Cutaneous conditions, Dysplastic nevus

26

Mutations in MBTPS2 have been reported to cause a broad phenotypic spectrum of X-linked genodermatoses, including IFAP (ichthyosis follicularis; atrichia and photophobia) syndrome (OMIM 308205) with or without BRESHECK (brain anomalies, retardation of mentality and growth, ectodermal dysplasia, skeletal malformations, Hirschsprung disease, ear deformity and deafness, eye hypoplasia, cleft palate, cryptorchidism, and kidney dysplasia/hypoplasia) syndrome, keratosis follicularis spinulosa decalvans (KFSD; OMIM 308800) and an X-linked form of Olmsted syndrome. We report a recurrent intronic mutation in MBTPS2 (c.671-9T>G) in a Chinese patient with the typical triad of IFAP syndrome (i.e. ichthyosis, atrichia and photophobia), along with pachyonychia, palmoplantar and periorificial keratoderma, which were reminiscent of Olmsted syndrome. Interestingly, this mutation was previously reported in two cases of IFAP without keratoderma, which suggests clinical heterogeneicity of the same mutation in MBTPS2. The concomitance of Olmsted syndrome-like features in this patient with IFAP may challenge the existence of the X-linked form of Olmsted syndrome as an independent condition.

Concepts: Palmoplantar keratodermas, Rare diseases, Keratoderma, Keratosis follicularis spinulosa decalvans, Genetic disorders, Ichthyosis, IFAP syndrome, Genodermatoses

25

Pemphigus is a rare autoimmune blistering disease, reported to be associated with other coexisting and autoimmune diseases, including thyroid diseases, rheumatoid arthritis, alopecia areata, vitiligo, systemic lupus erythematosus, scleroderma and rare entities such as myasthenia gravis.

Concepts: Immunosuppressive drug, Corticosteroid, Lupus erythematosus, Myasthenia gravis, Autoimmune diseases, Rheumatology, Rheumatoid arthritis, Systemic lupus erythematosus

25

Eosinophilic folliculitis (EF) is an idiopathic dermatitis included in the spectrum of eosinophilic pustular follicular reactions. Demodex folliculorum has been implicated as contributing to the pathogenesis of human immunodeficiency virus-associated EF, but it has not been described outside this context. We present an immunocompetent 65-year-old white man with a 5-year history of recurrent pruritic erythematous and oedematous lesions on his face, neck and scalp. Histopathologically, an eosinophilic microabcess with Demodex folliculorum mite within a pilosebaceous follicle was seen, and considered the causal agent. There were also accumulations of eosinophil granules on collagen bundles, and flame figure formations in the dermis. We believe that ‘eosinophilic follicular reaction’ is an appropriate term to describe this case of EF induced by D. folliculorum and thus distinguish it from the idiopathic form of EF. Moreover, this case suggests that D. folliculorum can sometimes induce an eosinophilic immune reaction.

Concepts: Demodex brevis, Chemical reaction, Induced demand, Inflammation, Anatomical pathology, Demodex folliculorum, Demodex, Immune system

25

There is an unmet need for psoriasis therapies that provide long-term remission. Itolizumab is a humanized recombinant anti-CD6 monoclonal antibody shown to be effective in psoriasis. We report a patient who received itolizumab in a phase 2 clinical trial, and experienced long-term remission. At baseline, the patient’s Psoriasis Area and Severity Index (PASI) was 12.2, and Physician’s Global Assessment (PGA) score was 3. After 8 weeks of treatment, the patient achieved 97% improvement in PASI. She continued to have ≥ 90% improvement, initially for 4 weeks (follow-up phase), and later for 20 weeks (follow-up extension phase). She continued to visit the hospital after the final study visit; her most recent visit was on 10 May 2013. PGA results during the visits revealed sustained response for 4 years and 5 months after stopping itolizumab. Itolizumab could be therefore an important treatment option for moderate to severe psoriasis, with potential to provide long-lasting remission.

Concepts: Physician, Psoriasis Area Severity Index, Hospital, Infliximab, Patient, Clinical trial, Monoclonal antibodies, Psoriasis

15

Sleep is important for growth and renewal of multiple physiological systems. The effects of chronic poor sleep quality on human skin function and visible signs of ageing have not been elucidated.

Concepts: Human skin color, Affect, Ageing, Snoop Dogg, Skin