Jalali MirHadi Aziz; Tabaie Mehdi
Volume 13, Issue 1 , 2010, , Pages 27-29
Abstract
Rothmund–Thomson syndrome is a rare autosomal recessive genodermatosis characterized by early onset of poikiloderma and several other cutaneous and organ involvements. We are going to report a 14-year-old girl who has been diagnosed with Rothmund– Thomson syndrome since she was 3 years old ...
Read More
Rothmund–Thomson syndrome is a rare autosomal recessive genodermatosis characterized by early onset of poikiloderma and several other cutaneous and organ involvements. We are going to report a 14-year-old girl who has been diagnosed with Rothmund– Thomson syndrome since she was 3 years old and has been suffering from pain and swelling of the right elbow and forearm for about 6 months. There are few previous reports on Rothmund–Thomson syndrome associated with malignant fibrous histiocytoma. We decided to report this case as another supporting document for this association.
Atul Salodkar; Sanjiv Choudhary; Sankha Koley
Volume 12, Suppl.3 , 2009, , Pages 1-4
Abstract
Kindler syndrome (KS) is a rare autosomal recessive genodermatosis. We report two cases of KS with classical clinical presentations involving skin and mucus membranes. Clinically, both patients had four major features of KS in the form of acral skin blistering, photosensitivity, progressive poikiloderma, ...
Read More
Kindler syndrome (KS) is a rare autosomal recessive genodermatosis. We report two cases of KS with classical clinical presentations involving skin and mucus membranes. Clinically, both patients had four major features of KS in the form of acral skin blistering, photosensitivity, progressive poikiloderma, and diffuse cutaneous atrophy. Case 1 had associated features in the form of urethral stenosis, skin fragility and palmoplantar keratoderma with extension of the scaling onto the flexor aspect of the wrist and loss of palmar creases. Case 2 had associated features in form of anal stenosis, oesophageal stenosis, skin fragility and palmoplantar keratoderma with loss of palmar creases. An Interesting finding in our report is that both cases have prominent telengectasia involving face and neck regions.
Emadi Seyed Naser; Sorush Mohammadreza; Moeineddin Fatemeh; Davudi Seyed Masoud; Jalalian Hamid Reza; Ardakani Mohammadreza Khodaeeyani
Volume 10, Issue 4 , 2007, , Pages 345-352
Abstract
Sulphur Mustard is a potent chemical warfare agent that was widely used during first world war and Iran Iraq conflict. Over 100,000 Iranians were injured by sulphur mustard and one-third is of them were suffering from late effects until today. This vesicant agent has a lot of acute and chronic destructive ...
Read More
Sulphur Mustard is a potent chemical warfare agent that was widely used during first world war and Iran Iraq conflict. Over 100,000 Iranians were injured by sulphur mustard and one-third is of them were suffering from late effects until today. This vesicant agent has a lot of acute and chronic destructive effects on the skin, eye and respiratory system. Sulphur mustard via the alkylations of several cellular proteins (structural, cytoplasmic and enzymes) and cell nuclei, produce several toxic, mutagenic and carcinogenic effects on epidermis, dermis, dermal appendages and hypodermis. We report a 41-year-old man who was poisoned with sulphur mustard in 1988.After exposure he developed severe skin blisters on upper trunk, posterior aspect of both hands and genitalia along with ocular and respiratory complications. He also had atrophy, pigmentation and vascular changes on skin of penis with pigmentation, atrophy and related findings in histopathologic studies, so the diagnosis of "sulphur mustard induced poikiloderma" was postulated. The absence of any complication on palms despite of their recurrent exposure to sulphur mustard is another remarkable finding in this case. It has been suggested that palms can act as a transport vector of sulphur mustard to other sites of skin.