Inflammatory myopathies in a patient with Darier disease, a possible association

Background: Darier disease (DD) is an autosomal dominant genetic disorder which develops from a mutation in the ATP2A2 gene. Inflammatory myopathies (IM) are the largest group of potentially treatable myopathies. In this case, we report development of IM in a patient with DD for the second time in the literature. Case presentation: The patient is a 59-year-old female, a known case of DD, who developed proximal muscle weakness 2 weeks prior to admission. Elevated muscle-enzymes, as well as typical electromyographic and radiologic confirmed the diagnosis of IM. Conclusions: Abnormalities in intracellular calcium homeostasis may explain the association between DM and DD, therefore it is noteworthy to keep this association in mind and conduct more research regarding this issue

Nail involvements as an indicator of skin severity in psoriatic patients

Psoriasis is a recurrent chronic inflammatory skin disease with various mild to severe clinical manifestations. The relationship between severity of the skin lesions and nail involvement has always been underestimated.Aim of the study was to evaluate the severity of skin involvement in psoriatic patients with and without nail manifestations.In this analytic cross-sectional study, patients with psoriasis referred to Razi University Hospital of Rasht from November 2015 to March 2016 were enrolled. Demographical features (i.e. age, gender) were obtained. Psoriasis severity and nail involvement criteria were assessed by Psoriasis Area and Severity Index (PASI) and Nail Psoriasis Severity Index (NAPSI), respectively. All the gathered data were analyzed by SPSS software.In this study, 71 psoriatic patients with a mean age of 39.23±17.9 years (mean ± Standard Deviation; range: 4 to 77 years old) were studied. 22 patients (31%) had nail involvements. PASI scores were 11.7±5.7 and 5.7±4.5 in the two groups with and without nail involvements, respectively (P&lt;0.001). There were no significant differences between age, age of onset, and duration of the disease between the two groups (P&gt;0.05).The correlation coefficient between PASI and NAPSI was 0.367, which was statistically significant (P&lt;0.001).Based on the findings of our study, nail involvement is an important criterion in determining the severity of skin manifestations in psoriatic patients. Additionally, a high percentage of such patients probably manifest both skin and nail manifestations. Therefore it is highly recommended to consider nail involvement when evaluating psoriasis. </p

A 36-Year-Old Renal Transplant Recipient Female with Leg Ulcer: A Case Report and Brief Review

Background. Opportunistic infections are common in organ transplant recipients. After 6 months of transplantation, patients have the highest risk of opportunistic infections such as cryptococcosis. Case Presentation. The report presents the case of a 36-year-old female renal transplant recipient, with complaints of few subcutaneous painful and warm nodules and large, warm, erythematous, nontender plaques on the mildly edematous right leg and ankle. Incisional biopsy of the subcutaneous nodule over the leg showed panniculitis with small- to medium-sized vasculitis associated with round yeast forms, and culture of the fragments revealed C. neoformans var. grubii. Conclusions. This article also reviews in brief the treatment of this rare complication. Reviewing the literature showed that since the cryptococcal cutaneous lesions are often nonspecific, the clinical picture solely is not enough to construct a definite diagnosis and there must be a high clinical suspicion

Evaluation of dermoscopic findings of longitudinal melanonychia in referred patients to dermatology clinics in Guilan, Iran

Introduction: Longitudinal melanonychia (LM) is a common clinical condition that is mostly identified by the presence of longitudinal, demarcated, and pigmented bands on the nail. Different benign or malignant pathologies can present with longitudinal melanonychia. Therefore, we aimed to investigate the frequency of dermoscopic features of LM in patients with LM referred to dermatology clinics in Guilan, Iran. Materials and Methods: This case-series study was conducted on 30 patients with LM who were referred to Besat clinic and Razi hospital, Rasht, Iran, from March 2022 to August 2022 with a complaint of LM. Demographical data and dermoscopic findings of patients were collected and analyzed using SPSS version 21. The LM and dermoscopic features were investigated using a dermatoscope (HEINE IC1, HEINE Optotechnik, Germany). Results: Out of 30 patients, 24 patients were female and 6 patients were male with a mean age of 30.08 ± 14.31 years old. Among these patients, five patients had a family history of LM, one patient with melanoma had Hutchinson’s sign, and three patients had pseudo-Hutchinson’s sign. The mean width of lesions of the nail was 2.42±2.12 mm with a mean time of onset of 7.42 ± 7.12 months. Also, the majority of the involved site of LM was hand (26.6%). Conclusion: According to our study, LM was more frequent in females and the trauma-related lesions of the nail were the most common dermatological findings among the patients

Association between Immunofluorescence Pattern and Mucosal Involvement in Patients with Bullous Pemphigoid

Bullous pemphigoid is an acquired autoimmune subepidermal blistering disease which is associated with mucocutaneous lesions. The type and amount of autoantibody deposition may have a role in mucosal lesions. We studied the association between mucosal involvement and direct immunofluorescence pattern in cutaneous lesions of patients with bullous pemphigoid. In this retrospective analytical cross-sectional study, we studied the demographic data, clinical presentations, and immunopathological findings of 69 patients with bullous pemphigoid admitted to our hospital 2008-2016. Patients were allocated into two groups on the basis of the mucosal involvement, and direct immunofluorescence patterns were evaluated. The data were analyzed using SPSS version18. The mean age of patients was 70.9±14.97 (mean ± Standard Deviation) years old. In our study, 56.5% of patients were women. All patients showed deposition of IgG and C3 in the dermoepidermal junction, with different severity. Patients with mucosal involvement (40.6% of cases) had a more prominent deposition of IgG, IgA, and C3 at the dermoepidermal junction compared with patients without mucosal involvement, which represented a statistically significant difference (P&lt;0.05). Logistic regression analysis showed that lower age, IgA, and C3 deposition (P&lt;0.05) were associated with mucosal involvement. Deposition of IgA and C3 (in addition to IgG) at the dermoepidermal junction seems to be a marker of mucosal involvement in patients with bullous pemphigoid. Attention to direct immunofluorescence pattern in patients with bullous pemphigoid may be helpful in prediction of mucosal involvement in these patients. </p

Gorlin syndrome: A case report

Gorlin syndrome is a rare autosomal dominant disorder which characterize by multi-organ abnormities such as odontogenic keratocysts in the jaw, skeletal abnormities and multiple basal cell carcinoma etc. We report a case of this syndrome in a young man with palmar pits, multiple facial BCC, clacifications of the falx cerebri and bifid rib

Extramammary Paget's Disease Associated With Genital Wart and Lichen Sclerosus

Extramammary Paget’s disease is an uncommon intraepithelial adenocarcinoma in genital and perianal regions. Genital wart is the most common sexually transmitted disease caused by human papilloma viruses and vulval lichen sclerosus is chronic pruritic dermatitis in genital area which could be able to change to invasive squamous cell carcinoma. We report a patient who had simultaneous lichen sclerosus, genital wart and extramammary Paget’s disease of the vulva. We could not find any significant association between them in literature