China
Africa
Explore chapters and articles related to this topic
Non-erythematous lesions
Published in Richard Ashton, Barbara Leppard, Differential Diagnosis in Dermatology, 2021
Richard Ashton, Barbara Leppard
Poikiloderma is identified by the combination of pigment, atrophy and telangiectasia. Poikiloderma of Civatte is the commonest type and occurs in middle aged and older patients on the sides of the neck. The skin becomes a mottled red-brown colour with atrophic areas. The area immediately under the chin and ears is spared. It is thought to be due to UV exposure, possibly associated with cosmetics acting as photo-sensitisers. It is very common and patients rarely bring it to the attention of their doctors. No treatment is available other than using a sunblock.
Monographs of fragrance chemicals and extracts that have caused contact allergy / allergic contact dermatitis
Published in Anton C. de Groot, Monographs in Contact Allergy, 2021
A woman from Thailand had the clinical picture of poikiloderma of Civatte on the anterior aspect and both sides of the neck; histopathology was consistent with this condition. She had a weak positive photopatch test to 6-methyl coumarin. This was apparently present in her perfume (which was negative on photopatch testing), but the authors also wrote that they found ‘coumarin listed in the ingredients on her perfume’s package’. In addition, as it could not be ascertained that the poikiloderma disappeared or improved after avoidance of the product (she had laser treatment), the authors’ proposition that photoallergy to 6-methyl coumarin may have played a role in producing poikiloderma of Civatte in this patient was insufficiently substantiated (24).
Dermatomyositis and its associated complications
Published in Biju Vasudevan, Rajesh Verma, Dermatological Emergencies, 2019
Iffat Hassan Shah, Saniya Akhtar
Among the connective tissue disorders, dermatomyositis can be confused with systemic sclerosis, systemic lupus erythematosus (SLE), and mixed connective tissue disease (MCTD). True lupus erythematosus and scleroderma may be present in the setting of an overlap syndrome. The papulosquamous lesions on the knees and elbows can be misdiagnosed as psoriasis. Airborne or allergic contact dermatitis or photo-aggravated dermatoses can mimic the heliotrope rash of DM. Poikiloderma in DM can be confused with cutaneous T-cell lymphoma. In cases of juvenile DM, atopic dermatitis is an important differential diagnosis.
Clinical practice guidance for juvenile dermatomyositis (JDM) 2018-Update
Published in Modern Rheumatology, 2020
Ichiro Kobayashi, Shinji Akioka, Norimoto Kobayashi, Naomi Iwata, Shunichiro Takezaki, Haruna Nakaseko, Satoshi Sato, Yutaka Nishida, Tomo Nozawa, Yuichi Yamasaki, Kazuko Yamazaki, Satoru Arai, Ichizo Nishino, Masaaki Mori
Heliotrope rash, Gottron’s papules, and Gottron’s sign are pathognomonic and tend to associate with disease activity, although Gottron’s sign may persist after achieving remission. The color of the heliotrope rash is rather dark reddish than purple in Japanese patients compared with Caucasians. Butterfly erythema which spreads over the cheeks on both sides of the root of the nose and often over the entire face is frequently an initial dermatological symptom of JDM. Inverse Gottron’s papules are frequently observed in adult patients with DM complicated with RP-ILD, whereas the correlation is not evident in JDM [15]. Periungual erythema is not specific but often observed as an initial symptom of JDM [16]. Elongation of the nail epithelium and dilated nail-fold capillaries are characteristic to both (J)DM and scleroderma. Poikiloderma is an intermix of pigmentation, depigmentation, dilatation of capillary vessels and skin atrophy in the same area, indicating chronic disease. Calcinosis is a characteristic cutaneous sign in JDM compared with DM [17]. Undermining ulcer is a possibly vasculitis-associated ulcerative lesion expanding from the dermis into subcutaneous tissues and associated with anti-MDA5 autoantibodies.
Clinical and genetic characteristics of nevus of Ota with choroidal melanoma in Chinese
Published in Ophthalmic Genetics, 2019
The exact etiology of nevus of Ota is still unknown. Although familial cases are rare, there may be an underlying genetic predisposition (13,14). Dr. Konstantinov found that GNAQ and BAP1 mutations in patients with nevus of Ota confer a greater risk for malignant melanoma and metastatic progression (7,15,16). In our series, we found two suspicious gene mutations involving FAM111B and DSC2 that might contribute to the etiology of the disease. The missense variants c.304T>C (p. Tyr102His) and c.2608G>C (p. Gly870Arg) were discovered in FAM111B and DSC2, respectively, according to our results. FAM111B is located in 11q12.1 and encodes a protein with a trypsin-like cysteine/serine peptidase domain in the C-terminus. Mutations in FAM111B are associated with hereditary fibrosing poikiloderma (HFP) (5). There is no current evidence to prove that FAM111B is associated with nevus of Ota. However, individuals with these gene mutations could display mottled pigmentation, which might indicate some relationship between FAM111B and nevus of Ota. DSC2, which is located in 18q12.1, encodes a member of the desmocollin protein subfamily. Mutations in this gene are associated with arrhythmogenic right ventricular dysplasia, reduced protein expression in several types of cancer, and tumor progression in esophageal cell carcinoma, gastric cancer, colon cancer, etc (6). No previous findings indicate whether DSC2 plays a role in nevus of Ota. DSC2 may be a new breakthrough for the disease. Further studies should identify the relationship between DSC2 and nevus of Ota.
Emerging drugs for the treatment of epidermolysis bullosa
Published in Expert Opinion on Emerging Drugs, 2020
Matthias Titeux, Mathilde Bonnet des Claustres, Araksya Izmiryan, Helene Ragot, Alain Hovnanian
KEB formerly known as Kindler syndrome has recently been classified as new subtype of EB [1]. This form is caused by recessive mutations in the FERMT1 gene coding for kindlin, a protein that functions in making up focal contact points of basal keratinocytes. The cleavage levels are multiple and can occur within keratinocytes, in the lamina densa and below the lamina densa, and are often associated with duplication of the basement membrane [11]. Patients present with epidermal skin fragility and blistering, photosensitivity and progressive poikiloderma, marked skin atrophy and periodontal disease.