Dyschromatosis universalis hereditaria (DUH) is a rare genodermatosis reported initially and mainly in Japan. However, subsequent cases have been reported from other countries. We report a case of DUH in a south Indian woman with a positive family history with cosmetic disfigurement and severe psychological impairment.
A disorder first described by Zoon in 1952, Zoon`s balanitis or Plasma cell balanitis (PCB), is an uncommon clinical disorder seen in middleaged uncircumcised men. It is characterized by one or more indolent well demarcated, glazed, reddish brown patches on the glans penis or prepuce. The etiology of this disease is unknown. This balanitis does not respond to routine topical antifungals, steroid creams and systemic antifungals. Diagnosis is confirmed by biopsy. Herein we report 8 cases of PCB, presenting with a characteristic clinical picture of the disease. In all cases circumcision was done and histopathology confirmed the diagnosis of PCB. All patients were followed up for a period of 6 months and no recurrences were observed. Development of malignancy is not seen. In conclusion. We believe that for all cases of balanitis, not responding to routine topical antifungals, steroid creams and systemic antifungals, the diagnosis of Zoon`s balanitis should be considered.
StreszczenieChoroba po raz pierwszy została opisana przez Zoon`a w 1952 roku. Zapalenie żołędzi typu Zoona lub też plazmacytowe zapalenie żołędzi (PCB) jest rzadkim, klinicznym zaburzeniem obserwowanym u nieobrzezanych mężczyzn w średnim wieku. Charakteryzuje się ono jedną lub większą ilością łagodnych, dobrze odgraniczonych, połyskujących, czerwonawych plamek na żołędzi penisa lub napletku. Etiologia zmian pozostaje nieznana. Ten rodzaj zapalenia żołędzi nie odpowiada na typowe leczenie przeciwgrzybicze: miejscowe czy systemowe oraz na maści sterydowe. Diagnoza jest potwierdzana biopsją. W tym artykule opisujemy 8 przypadków PCB oraz ich obraz kliniczny. We wszystkich przypadkach został wykonany zabieg obrzezania a badanie histopatologiczne potwierdziło diagnozę. Wszyscy pacjenci byli obserwowani przez okres 6 miesięcy, nie zanotowano nawrotu choroby. Nie zanotowano transformacji nowotworowej. Wszystkie przypadki zapalenia żołędzi nie odpowiadające na leczenie przeciwgrzybicze miejscowe lub systemowe oraz na maści sterydowe powinny zostać ponownie rozpatrzone pod kątem diagnozy PCB.
IntroductionXeroderma pigmentosum is a rare, genetically heterogeneous, autosomal recessive disorder characterized by photosensitivity, cutaneous pigmentary changes, premature skin ageing, and the development of various cutaneous and internal malignancies at an early age. The basic defect underlying the clinical manifestations is a nucleotide excision repair (NER) defect leading to a defective repair of DNA damaged by ultra violet (UV) radiation [1]. XP is characterized by clinical and cellular hypersensitivity to UV radiation manifesting as intolerance of skin and eyes to light. The skin lesions are comprised of freckles on limbs and face with a dry skin covered with a mixture of mottled, hypopigmented and hyperpigmented, atrophic rounded and oval macules, giving the entire skin a checkered appearance associated with a generalized actinic keratoses, manifesting on black skin as palpable, rough, blackish spots covered with adherent scales [2,4]. These skin lesions cover both the sun exposed and covered areas. The skin later develops cutaneous malignancies. Ocular changes include photophobia, ocular pigmentary changes, conjunctivitis, corneal keratitis, ulcers, blindness, and malignancies. The diagnosis of XP can be established with studies performed in specialized laboratories. These studies include cellular hypersensitivity to UV radiation and chromosomal breakage studies, complementation
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