Steatocystoma Multiplex Limited to the Vulva: Report of a Very Rare Case Successfully Treated by a Simple Surgical Method.

Aim: Steatocystoma multiplex (SM) is a rare malformation characterized by multiple cutaneous cystic lesions containing oily substance. SM has autosomal dominant mode of inheritance, though there are a significant number of sporadic cases reported as well. Characteristically, the lesions manifest around the time of puberty and are found on the sternal areas, face, trunk and scrotum. SM limited to the vulva is a very rare condition as there are only a five cases in the literature. Case: A 32-year-old woman presented with a 1 year-history of nodules, gradually increasing in size and number, on the labia majora. Similar lesions were not present on other parts of her body and family members. On physical examination, yellowish papules and nodules ranging in size from 2-15mm without punctum were present on the labia majora. Biopsy performed at the time revealed histological changes with steatocystoma multiplex. After a mini incision, evacuation of the cyst followed by the removal of the cyst wall was performed without complication. The lesions healed rapidly without scarring. The patient was followed by yearly controls. Recurrence was not observed until the fourth year of surgery. Smaller lesions were observed on previously untreated areas of vulva which were removed again successfully. Discussion: SM treatment is difficult. In addition to medical treatments including antibiotic and anti-inflammatory drugs and isotretinoin, there are different surgical treatments including total excision and grafting, cryotherapy and CO2 laser with limited success. Conclusion: This is a very rare case of SM limited to the vulva, simply and successfully treated by a simple surgical method, emphasizing the importance of cyst wall removal to achieve long time remission.

Relationship between retinal sensitivity and disease activity in patients with psoriasis vulgaris

OBJECTIVES: Psoriasis is a hyperproliferative chronic inflammatory skin disease of unknown etiology and ocular structures and visual pathways can also be affected during the course of this disease. Subclinical optic neuritis has previously been observed in psoriatic patients in visual evoked potential studies. This trial was designed to evaluate retinal sensitivity in patients with psoriasis vulgaris. METHODS: A total of 40 eyes of 40 patients with chronic plaque-type psoriasis and 40 eyes of 40 age- and sex-matched control subjects were included in this study. The diagnosis of psoriasis was confirmed by skin biopsy. The severity was determined using the Psoriasis Area and Severity Index and the duration of the disease was recorded. After a full ophthalmological examination, including tests for color vision and pupil reactions, the visual field of each subject was assessed using both standard achromatic perimetry and short wavelength automated perimetry. RESULTS: The mean Psoriasis Area and Severity Index was 22.05±6.40′. There were no significant differences in the visual field parameters of subjects versus controls using either method. There were correlations between disease severity and the mean deviations in standard achromatic perimetry and short wavelength automated perimetry and between disease severity and the corrected pattern standard deviation and pattern standard deviation of short wavelength automated perimetry (r = -0.363, r = -0.399, r = 0.515 and r = 0.369, respectively). CONCLUSIONS: Retinal sensitivity appears to be affected by the severity of psoriasis vulgaris. .

Terra firma-forme dermatosis.

Terra firma-forme dermatosis is characterized by 'dirty' brown-grey cutaneous patches and plaques that can simply be eradicated by forceful swabbing with alcohol pads. The pathogenesis has been attributed to abnormal and delayed keratinization. Although affected patients present with typical lesions, the disorder is not well-known by dermatologists. In this report, we describe two patients with terra firma-forme dermatosis in the setting of xerosis cutis and atopic dermatitis. From a clinical point of view, we lay emphasis on its unique expression and diagnosis/treatment. From a histological perspective, we highlight its resemblance to dermatosis neglecta and speculate on the role of 'neglect' in a patient with seemingly adequate hygiene. The role of urea containing emollients in the development of this disorder remains to be determined.