Hypotension, Syncope, and Fever in Systemic Mastocytosis without Skin Infiltration and Rapid Response to Corticosteroid and Cyclosporin: A Case Report

Mast cell disorders are defined by an abnormal accumulation of tissue mast cells in one or more organ systems. In systemic mastocytosis, at least one extracutaneous organ is involved by definition. Although, systemic mastocytosis usually represents with skin lesion called urticaria pigmentosa, in a small proportion, there is extracutaneous involvement without skin infiltration. Other manifestations are flushing, tachycardia, dyspepsia, diarrhea, hypotension, syncope, and rarely fever. Various medications have been used but there is not a definite cure for systemic mastocytosis. The principles of treatment include control of symptoms with measures aimed to decrease mast cell activation. We describe a case of systemic mastocytosis presenting with hypotension, syncope attacks, fever, and local flushing. In bone marrow biopsy, increased mast cell infiltration was demonstrated. She had no skin infiltration. A good clinicopathological response was obtained acutely with combination therapy of glucocorticoid and cyclosporine

Malignancy Associated Genodermatoses

The cutaneous features of some genetic diseases that manifest during childhood might be the first signs of predisposition to cancer; therefore, early recognition of such clinical findings as well as necessary follow-up are vital. This review focuses in particular on nevoid basal cell carcinoma, Cowden syndrome, neurofibromatosis type 1, tuberous sclerosis, Peutz-Jegher's syndrome, dyskeratosis congenita, and xeroderma pigmentosum. These genodermatoses, are characterised by predisposition to skin cancer or cancer in other organs; as such this review aimed to highlight their diagnostic clinical features.WoSScopu

Infantile Hemangioma

Infantile hemangioma (IH) is the most common benign tumor of infantile period. It appears few weeks after birth, grows rapidly in the first 4-6 months. Subsequently, growth stops and involution period which lasts about few years starts. It is more common in girls, premature infants, birthweight < 1500 gr, multiple gestations and Caucasians. Pathogenesis is not clearly known, however role of fetal hypoxic stres is considered. IH is a self-resolving benign lesion, yet in some localisations it may threaten organ function. Periorbital IH may cause ambylopia, astigmatism and strabismus. IH localised to beard area can lead to airway obstruction. Large facial IH can be a component of PHACE syndrome. Mid line lumbosacral IH can be associated with spinal dysraphism. Other complications of IH include ulceration, heart failure, hypothyroidism, cosmetic dysfiguration. Treatment is indicated in ulcerated lesions, life/function threatening situations and lesions that can cause cosmetic nuissance. Corticosteroids, propranolol, interferon, vincristin, laser and surgery are used for this purpose. (Turkderm 2011; 45 Suppl 2: 133-7)WoSScopu

Erythematous Dermatoses

Erythematous dermatoses are common diseases encountered in the dermatology clinic. Figurate erythemas comprise a huge part of erythematous dermatoses. Herein, figurate erythemas are discussed under the headings of erythema annulare centrifugum, erythema gyratum repens, erythema chronicum migrans, necrolytic migratory erythema, erythema marginatum rheumaticum, familial annular erythema, annular eruption of lupus erythematosus and erythema in chronic granulamatous disease carriers. Additionally, facial and palmar erythema are reviewed

Childhood Alopecia Areata: A Study Of 89 Patients

Background and Design: Alopecia areata (AA) is a frequent type of acquired hair loss with a sudden onset. The aim of this study was to investigate the epidemiological and clinical features of AA and diseases associated with AA in the Turkish pediatric population. Materials and Methods: We retrospectively evaluated the medical data of patients under 16 years of age who were admitted to the pediatric dermatology outpatient clinic at Hacettepe University Hospital between 2006 and 2011 with a diagnosis of AA, alopecia totalis (AT) and alopecia universalis (AU). Results: A total of 89 patients (34 females and 55 males), were included in this study. The mean age of the subjects was 9.8 +/- 3.4 years (range:18 months-16 years). 9% (n=8) patients had other autoimmune diseases: Hashimoto's thyroiditis in 5, vitiligo in 1, juvenile rheumatoid arthiritis (JRA) in 1 and morphea in 1 patient. AA was located on the scalp in 86.5% (n=77) of the patients, 7.9% (n=7) of the patients had AA on the scalp together with eyelashes, eyebrows and the body. AT was noted in 1, AU was noted in 4 patients. Severity of hair loss was <25% in 94.3% (n=84) of the patients according to the involved area. Hashimoto's thyroiditis was diagnosed in 5 patients; these patients had normal thyroid function tests (TFT). TFT abnormalities were detected in 27% (n=24) of the patients. 24.7% (n=22) of these patients were diagnosed with clinically insignificant thyroid disorder, 2 patients had compansated hypothyroidism (n=2). Eight patients (9%) patients were found to be positive for anti-thyroid peroxidase antibody (Anti-TPO ab). Antithyroglobulin antibody (Anti-TG Ab) elevation was noted in 3.3% (n=3) and antinuclear antibody (ANA) positivity was noted in 9% (n=8). Nail changes were detected in 12.3% (n=11) of the patients as leukonychia (n=2), longitudinal striation (n=2), trachyonychia (n=1), onychoschizia (n=1) and pitting (n=1). Conclusion: Childhood AA usually causes limited hair loss. TFT anomalies can accompany AA without clinical symptoms. Therefore, investigation and regular follow-up of baseline TFT and anti-TPO Ab levels are suggested in patients with AA in childhood.WoSScopu