Gulsen Akoglu

Cutaneous mastocytosis : demographic aspects and clinical features of 55 patients

Background  Mastocytosis is a rare, heterogeneous group of disorder with abnormal increase of mast cells in one or more organ systems.

A case of lupus vulgaris successfully treated with antituberculous therapy despite negative PCR and culture

A 14-year-old boy presented with a pink firm plaque with well-defined borders in the right infra-orbital skin area. On diascopy, the infiltrate exhibited a typical apple-jelly appearance. No acid-fast bacilli could be demonstrated. A polymerase chain reaction (PCR) assay did not reveal the presence of mycobacteria in a lesional biopsy sample. Culture of biopsied tissue on Loewenstein-Jensen medium was negative. Although the tuberculosis culture and PCR did not confirm tuberculosis, a diagnosis of lupus vulgaris was made considering the clinical and histopathological findings. After a 9-month antituberculous therapy, the lesion disappeared. We believe that a diagnosis of lupus vulgaris still depends more on clinical and histopathological findings than on tuberculosis culture or PCR.

Prurigo pigmentosa successfully treated with low-dose isotretinoin.

Background/Aims: Prurigo pigmentosa (PP) is an uncommon inflammatory disease with pruritic reddish papules, papulovesicules or vesicules that are symmetrically localized on the trunk and nape, accompanied by reticular hyperpigmentation. Currently available therapeutic options seem somewhat unsatisfying. Herein, we report an 18-year-old female with PP successfully treated with low-dose isotretinoin. Methods: The patient presented with a symmetrical pruritic eruption on the lateral sides of her trunk with erythematous papules on a hyperpigmented background. Based on the typical clinical and associated histological findings, the patient was diagnosed as PP and a treatment with low-dose isotretinoin 0.3 mg/kg/day (20 mg/day) was started. Results: After a total of 3 months of 20 mg/day isotretinoin therapy, all erythematous macules and papules resolved and the residual pigmentation had almost disappeared. Conclusion: Low-dose isotretinoin is not only adequate for the improvement of erythematous lesions, it also helps resolve the reticular hyperpigmentation of PP. Further studies are needed to observe the efficacy of isotretinoin in the treatment of PP.

Total serum oxidant/antioxidant status and arylesterase activity in recurrent aphthous stomatitis.

Background Recurrent aphthous stomatitis (RAS) is a chronic relapsing inflammatory disorder of the oral mucosa with unknown etiology. Oxidative stress (OS) is suggested to play a main role in the etiopathogenesis in RAS. Objective In this study, we hypothesize that a systemic OS is present in patients with RAS. Methods Forty-four patients with active RAS lesions and 38 healthy controls were being included in the study. Serum total oxidant status (TOS), total antioxidant status (TAS), oxidative stress index (OSI), and paraoxonase 1 arylesterase (ARES) activity were being determined. Results RAS patients had significantly lower TAS levels and higher TOS and OSI values than controls. The patients had a lower ARES activity when compared to healthy controls. No correlations were observed between OS parameters and age, gender, duration of disease or frequency of RAS attacks per month. Conclusion A systemic OS is determined with an imbalance in oxidant/antioxidant status and lower ARES activity in RAS. Systemic OS may have an important role in the pathogenesis of RAS formation.

Clinical and histopathological response to acitretin therapy in lipoid proteinosis.

Abstract Lipoid proteinosis (LP) is a rare autosomal recessive genodermatosis associated with deposition of periodic acid-Shiff (PAS)-positive hyaline material in skin, mucosa, and other tissues. LP is caused by loss-of-function mutations in the extracellular matrix protein 1 gene (ECM1). No curative therapy is available. In this report, we describe the clinicopathological and genetic features of a Turkish LP family with four cases, and evaluate the response of acitretin therapy. Patients were presented with hoarseness and beaded eyelid papules, thickened frenulum, hyperkeratotic plaques and infiltrated warty papules and nodules. Skin biopsies revealed deposition of PAS-positive hyaline material in dermis. A homozygous nonsense mutation in exon 3 of the ECM1 gene, R53X, was detected in the family. Acitretin therapy was administered in two patients, in whom some regression and softening of skin lesions were achieved. However, no histopathological change in PAS-positive deposition could be detected. Although there is no current effective treatment for LP, acitretin may be helpful for patients, especially those who complain about hyperkeratosis.

Evaluation of total oxidant and antioxidant status in localized and generalized vitiligo

Vitiligo is an acquired depigmentation disorder, and oxidative stress is suggested to have a major role in its aetiopathogenesis.

Pachydermoperiostosis with myelofibrosis and renal amyloid A amyloidosis.

Pachydermoperiostosis (PDP) is a rare benign disorder characterized by periosteal reaction involving the distal extremities, clubbing of the fingers, and thickening of the skin, particularly of the scalp and the face. The disease is more prevalent in males. It has an autosomal dominant inheritance with variable penetrance. Several reports have proposed an association between PDP and bone marrow failure due to myelofibrosis. In this article, we describe a novel association of PDP with myelofibrosis, and renal amyloid A (AA) amyloidosis.

Membranoproliferative glomerulonephritis associated with psoriasis vulgaris.

Psoriasis is a hereditary, chronic inflammatory disorder of the skin. Generally, the psoriatic process is limited to the skin; however, internal organs such as the kidneys may be involved in the course. Several glomerular diseases have been distinguished due to renal histological findings of psoriatic patients to date. The underlying pathogenetic mechanisms of these associations remain unclear because of the limited number of cases. We report a case of primary membranoproliferative glomerulonephritis (MPGN) in a psoriatic patient. This is the first reported case that demonstrates the coexistence of MPGN and psoriasis.

Clinicopathological features of 25 patients with acquired perforating dermatosis

BACKGROUND Acquired perforating dermatosis (APD) is a rare group of skin disorders characterized by transepidermal elimination of dermal tissue material. There are only a few studies of APD. OBJECTIVE We aimed to review the clinicopathologic features of patients with APD and evaluate the outcomes of treatments. MATERIAL AND METHODS The medical records of 25 patients who were clinically and histopathologically diagnosed as having APD between 2006 and 2013 were retrospectively reviewed. The histopathologic sections and the results of elastic, trichrome and periodic acid Shiff (PAS) staining were evaluated. RESULTS Of the patients, 17 (68%) had ARPC, 7 (28%) had perforating folliculitis, 1 (4%) had Kyrles disease. Male/female ratio was 1.3. The mean age was 51.8 ± 12.8 years. The disease duration ranged between 15 days and 96 months. Diabetes mellitus was the most common disease associated with APD (48%). The walls of vessels were PAS positive in the lesions of 6 of 13 diabetic patients. After various treatments, almost all lesions regressed in 15 (60%) patients. CONCLUSION Acquired perforating dermatosis may present with various clinical features and is mostly associated with diabetes mellitus. Diabetes mellitus may be involved in the pathogenesis of APD via microangiopathy and other mechanisms.

AN UNUSUAL PRESENTATION OF ERYTHEMA TOXICUM NEONATORUM: DELAYED ONSET IN A PRETERM INFANT

patients is unusual. We report a child with chronic urticaria to cockroach. A 12-year-old boy was referred to our hospital because of chronic urticaria that had begun when he was 7 years old. It had improved with the use of antihistamine therapy but he had stopped taking the antihistamines. He did not develop allergic rhinitis, asthma, or atopic dermatitis. His weight and height percentiles were 25%. He had urticarial plaques but his breathing sounds were clear, and his heartbeat was regular, without murmur. Abdominal examination was normal without any organ enlargement. The white blood cell count was 7440/mm 3 , eosinophil percent was 9%, eosinophil count was 670/mm 3 , total immunoglobulin E level was 2066, sedimentation rate was 19 mm/hour, urine examination was normal, parasite examination was negative. He underwent a complete allergy evaluation. Antihistamines were withheld for 10 days before the tests. Skin prick test was performed. Only cockroach hypersensitivity was found. Chronic urticaria is a very common skin disease with a considerable impact on quality of life. Whereas atopics are at increased risk for acute urticaria /angioedema as well as some forms of physical urticaria, most patients with chronic urticaria/angioedema are, surprisingly, not atopic. The underlying cause of mast cell degranulation in the majority of patients with chronic urticaria /angioedema cannot be determined. Although there is a widespread belief that cockroach allergy is a common problem in patients with respiratory allergies (4), little is known about its possible role in chronic urticaria. Our patient’s chronic urticaria dramatically improved following the avoidance of cockroach. We have reported him because there are no data about chronic urticaria with isolated cockroach hypersensitivity. We propose that cockroach hypersensitivity be considered and investigated in chronic urticaria patients.