Iranian Journal of Dermatology

Abstract Palmoplantar keratodermas are a heterogenous group of diseases, one of them is Papillon-Lefevre Syndrome (PLS). This rare disease is inherited as autosomal recessive and characterized by focal hyperkeratotic plaques on elbows and knees, severe periodontal disease resulting in premature loss of teeth. We report two brothers with PLS who did not have a history of this disease in their family.

Abstract Distant metastases are rare during the course of ovarian carcinoma. Ovarian epithelial adenocarcinomas rarely metastasize to the skin which is seen in less than 4% of patients. It usually presents as subcutaneous nodules and, less commonly, as inflammatory metastases which mimic cellulitis or viral or bacterial skin infections. The histology in most of the tumours is grade 1 or 2 serous papillary adenocarcinomas. The prognosis in most patients is poor, thus, palliative treatment is usually indicated. We report a patient with ovarian carcinoma and widespread zosteriform skin metastases.

Abstract The case, who discribed here is a 66-year-old man with one year history of asymptomatic, keratotic papules with a linear distribution on the skin of his right palm near the wrist. On histopathological examination cornoid lamella-like parakeratotic columns above eccrine sweat ducts were observed. The acrosyringium was also dilated. He had been followed up for predominantly sensory polyneuropathy by neurologist from 6 years before. Endocrinologic evaluation revealed hyperthyroidism. In this article, we present a new case of Porokeratotic Eccrine Ostial and Dermal Duct Nevus (PEODDN) where the palmar skin was the primary site of presentation. The present case is consistent with the tarda variant of PEODDN, as the onset of lesions was around the age of 65 years.

Abstract Pyoderma gangrenosum is a rare non infectious neutrophilic dermatosis commonly associated with underlying systemic disease. Many treatment regimens have been reported. Herein, we report a case of refractory pyoderma gangrenosum with good response to combination of cyclosporine and mycophenolate mofetil.

Abstract Inflammatory linear verrucous epidermal nevus (ILVEN) is a unilateral skin disorder which usually manifests in infancy or childhood. Female predominance, frequent leg involvement, pruritus, resistance to treatment, and a psoriasiform histologic pattern are other common characteristics of this disease. Atypical presentations of ILVEN have been described and include late onset in life, widespread involvement and response to treatment. We report a 2-year-old otherwise healthy girl who had bilateral ILVEN and provide a review of this disease.

Abstract Lupoid leishmaniasis is an unusual complication of acute cutaneous leishmaniasis which can represent a diagnostic and therapeutic challenge. The disease is actually a reactivation of an earlier acute cutaneous infection. It usually starts close to or in the scar of previous lesion and can cause severe destruction. Its diagnosis is complicated by the sparsity of the microorganisms in the direct smear and the tissue. The present report deals with a middle age Iranian woman presented to us with multiple scars on the whole face and also multiple red-down papules and nodules around these scars. After three years of clinical, histopathological and laboratory investigations and treating with no or minimal success for the diagnosed of discoid lupus erythematosus, sarcoidosis and lupus vulgaris, finally was diagnosed and treated successfully as lupoid leishmaniasis with meglumine antimoniate (Glucantime).

Abstract Linear psoriasis is a rare entity which is commonly confused with inflammatory linear verrucous epidermal nevus. We report a 25-year-old man who had linear psoriasis along Blaschko lines in a unilateral distribution on the left side of the body. Our diagnosis was based on a combination of historical, clinical, histopathological evidence and response to treatment.

Abstract Spindle cell hemangioma is a benign vascular tumor presenting as multifocal red or bluish nodules on the distal limbs. We present a 57-year-old woman with five years history of multiple, soft, violaceous nodules on her hands. Pathologic study indicated spindle cell hemangioma.

Abstract In this article we present a 53-year-old lady, who had progressive indurated skin lesions on her both forearms since 3 months ago, without skin discoloration. Chest x-ray showed parahilar lymphadenopathy, in high resolution CT (HRCT) scan evaluation multiple lymph nodes in upper and middle mediastinum were reported. Angiotensin converting enzyme (ACE) level was increased to 81 (normal 5-52) units. Deep skin biopsy was taken and granuloma with epitheloid and giant cells without caseating necrosis in dermis was reported. The epidermis was spared. She was diagnosed as a case of subcutaneous sarcoidosis and treated successfully with steroid and azathioprine.

Abstract Ehlers-Danlos syndrome (EDS) is a heterogenous group of inherited disorders of connective tissue characterized by fragility of the skin and blood vessels, hyperextensibility of the skin and joint hypermobility. Cutis laxa is characteized clinically by lax, pendulous skin and histologically by loss of elastic tissue in the dermis. There are some reports of coexistence of cutis laxa with other disorders. We present a case of classic EDS with cutis laxa in an Iranian family.

Abstract Goltz syndrome / Focal Dermal Hypoplasia (FDH) is a rare syndrome resulting from developmental defects in tissues with mesodermal origin such as eye, skin, mouth, muscoloskeletal, and central nervous system. We report a 13-year-old girl with FDH who had several features of skin, eye, and mouth involvement. She also had revurrent otitis media, which has not been reported so far.

Abstract Port-wine stain is a vascular malformation characterized pathologically by ectasia of superficial dermal capillaris and clinically by persistent macular erythema. The association of a port-wine stain on a limb with soft tissue swelling, with or without bony overgrowth, is termed klippel-Trenaunay syndrome. Phakomatosis pigmento-vascularis is a combination of port-wine stain and cutaneous pigmentary abnormality.
The patient who is described here is a 25-year-old man with Klippel-Trenaunay syndrome in association with bilateral Ota nevus. He can be classified as phakomatosis pigmento vascularis type IIa which is a relatively rare finding with this presentation.