1/3. Reticulate acropigmentation of Dohi: a case report of autosomal recessive inheritance.Reticulate acropigmentation of Dohi is a rare dyschromic disorder that generally has an autosomal dominant pattern of inheritance. Most of the cases have primarily been described from japan. Only a few similar cases have been described elsewhere. We describe 3 black siblings, one boy and two girls, who had progressive reticulate hyperpigmented and hypopigmented macules over the dorsa of hands and feet, which began in early childhood. There were no palmar pits or breaks of the epidermal rete ridge pattern nor was there a family history of any pigmentary skin diseases. Three skin biopsies were performed on one patient; a biopsy specimen from a hyperpigmented macule showed increased melanin in all epidermal levels tapering towards the surface, a second biopsy specimen from a hypopigmented macule showed much less melanin, but it had a similar distribution. A third specimen from a hyperpigmented macule for electron microscopy showed a moderate number of stage III and IV melanosomes in the cytoplasm of the melanocytes. To our knowledge, these patients are the first cases reported from the middle east with an autosomal recessive pattern of inheritance, confirming previous reports.- - - - - - - - - - ranking = 1keywords = skin disease (Clic here for more details about this article) |
2/3. Segmental pigmented purpura.A segmental distribution of pigmented purpura (PP) is rare. Our patient presented at 5 years of age with red-brown petechial macules and telangiectases on her chest, the inner and dorsal aspects of the right upper extremity, and the dorsal aspect of the hand, which had developed slowly over the previous 7 months. The lesions were intermittently pruritic. A biopsy from the right arm revealed discrete hydropic degeneration of the basal layer of the epidermis, dilation of the blood vessels, numerous extravasated erythrocytes and lymphocytes, and lymphocytes in the lower part of the epidermis. The skin disease resolved almost completely within 18 months. The general physical examination revealed an open foramen ovale, slight hypogammaglobulinemia (G and A), and enhanced fragility of the blood vessels.- - - - - - - - - - ranking = 1keywords = skin disease (Clic here for more details about this article) |
3/3. prurigo pigmentosa in a patient with primary biliary cirrhosis and Sjogren syndrome.A 44-year-old Japanese woman suddenly developed severely pruritic erythematous papules on her trunk in a symmetrical distribution. biopsy specimens showed the typical histopathological findings of prurigo pigmentosa. She had had recurrent episodes of high fever spikes for several years, and lost 10 kg in the last year. She was diagnosed as primary biliary cirrhosis (PBC) associated with subclinical Sjogren syndrome (SjS). Predonisolone (60 mg/day) for two weeks was effective for the PBC and fever, but not for the prurigo pigmentosa. PBC may be involved in the pathogenesis of this rare skin disease.- - - - - - - - - - ranking = 1keywords = skin disease (Clic here for more details about this article) |