Iranian Society of DermatologyIranian Journal of Dermatology2717-072110Suppl.220071201Pachyonychia congenita type II: report of a case11101283ENAmir Hooshang EhsaniKatrin KiavashJournal Article19700101<span>Pachyonychia congenital (PC) is a rare genodermatosis affecting the nails, skin, oral mucosae, larynx, hair, and teeth. General clinical presentation includes thickening of finger and toenails, painful plantar keratoderma, hyperhidrosis, oral leukokeratosis, follicular keratosis, palmar keratoderma, cutaneous cysts, hoarseness, twisted hair and prenatal or natal teeth. Clinically, PC is divided into 4 distinct subtypes. Here, we present a three-year-old girl with PC type II.</span>Iranian Society of DermatologyIranian Journal of Dermatology2717-072110Suppl.220071201Piloleiomya associated with pruritus: report of a case22101284ENGuiti SadeghianHengameh ZiaiJournal Article19700101<span>A 30-year-old woman was referred to us with red and firm nodules on upper lateral aspect of left arm. These nodules were present for 15 years without pain but with intense pruritus.</span><br /><span>She reported uterine leiomyoma in the past, but family history for similar lesions was negative. Blood tests were normal, but pelvis ultrasound showed a large uterine leiomyoma.</span><br /><span>Histologic examination of cutaneous lesions showed proliferation of smooth muscle cells with fascicular pattern in dermis. Pruritus was controlled with oral anti-histamine (loratadine). Plastic surgeons refused to remove the skin lesions due to their location and widespread distribution.</span>Iranian Society of DermatologyIranian Journal of Dermatology2717-072110Suppl.220071201Ichthyosiform Parapsoriasis: report of a case33101285ENHayedeh Ghani-NejadMansour Nasiri-KashaniReza Mahmoud RobatiPedram MehryanAlireza Alavi-niaSoheil FatehJournal Article19700101<span>Acquired ichthyosis is a known paraneoplastic sign of lymphoproliferative malignancies with nonspecific histopathologic findings revealing no implication of the underlying neoplasm. However, ichthyosiform eruption is considered as a specific manifestation of mycosis fungoides (MF), i.e., ichthyosiform MF. There are a few reports of ichthyotic manifestation of MF, but only one report of this presentation in Parapsoriasis. We present a case of ichthyosiform Parapsoriasis in a 22-year-old woman with hyperpigmented ichthyosiform scaly patches on her trunk and extremities. The histopathologic findings were hyperkeratosis, parakeratosis and acanthosis with scattered lymphocyte infiltration, also could be seen in Parapsoriasis. The patient responded well to oral photochemotherapy.</span>Iranian Society of DermatologyIranian Journal of Dermatology2717-072110Suppl.220071201Becker's nevus and breast hypoplasia: report of a case44101286ENAkram AnsarBanafsheh HashemiJournal Article19700101<span>Becker's nevus is a unilateral hyperpigmented area of skin with geographical borders. It is usually located on shoulders, anterior chest, scapular region and upper arms. Sometimes it shows evidence of androgen hypersensitivity and co-localizing with other developmental anomalies. We report a 12-year-old girl with clinical and histopathological findings compatible with Becker's nevus on her left chest accompanied with ipsilateral breast hypoplasia.</span>Iranian Society of DermatologyIranian Journal of Dermatology2717-072110Suppl.220071201Congenital naevoid psoriasis55101287ENReza YaghoobiSeyyed Arash AbtahianJournal Article19700101Psoriasis is quite common in childhood, although congenital psoriasis is very rare. Here, we report a 2-year-old girl with typical linear psoriasis associated with bilateral hypopigmentation of breasts which has been present since birth.
Iranian Society of DermatologyIranian Journal of Dermatology2717-072110Suppl.220071201Nodular colloid milium of the ears66101288ENReza YaghoobiNiloofar SinaJournal Article19700101<span>Colliod milium is a degenerative change, which is characterized clinically by development of multiple yellowish, translucent papules and nodules on the light exposed skin and histologically by the presence of colloid in the dermal papillae. This is a rare condition and has some variants. We report a case of colloid millium with nodular and plaque lesions on his ears. Our patient was a farmer with a history of more than 47 years chronic sun exposure and showed photoaging.</span>Iranian Society of DermatologyIranian Journal of Dermatology2717-072110Suppl.220071201A family with Bart-Pumphrey syndrome77101289ENKamran BalighiFatemeh MoeineddinAhmadreza RajaeeJournal Article19700101<span>All member of family referred to our clinic complaining of white nails. Physical examination revealed clinical features of leukonychia totalis and also the presence of sensor ineural hearing loss (SNHL), palmoplantar keratoderma (PPK) and knuckle pads, the four essential criteria for the diagnosis of Bart Pumphrey syndrome. Three generations were affected with variable presentations in male and female and autosomal dominant pattern of inheritance.</span>Iranian Society of DermatologyIranian Journal of Dermatology2717-072110Suppl.220071201An uncommon presentation of pachyonychia congenital88101290ENAmirhushang EhsaniFatemeh MoeineddinAhmadreza RajaeeJournal Article19700101<span>A 10-month-old female presented with severe progressive wedge-shaped thickening and discoloration of all twenty nails. Further evaluations revealed palmoplantar keratoderma along with recurrent acral blisters causing residual crusted ulcers which were present during the past six months. Other findings include scalp kinky hair and dental caries. Patient history was remarkable for natal teeth and similar skin blisters in her older brother since his infancy.</span><br /><span>The patient's clinical presentation and history are consistent with pachyonychia congenita syndrome showing concomitant features of both subtypes 1 and 2, probably due to a new mutation in the responsible gene.</span>Iranian Society of DermatologyIranian Journal of Dermatology2717-072110Suppl.220071201Nicolau syndrome: report of a case99101291ENAkram Beheshti-rooyYunis ShafighBehzad BijaniJournal Article19700101<span>Nicolausyndrome or embolia cutis medicamentosa is a rare complication of intramuscular injection of some drugs. This syndrome has been reported after injection of NSAIDS, corticosteroids and antibiotics.</span><br /><span>Here we report a 16-year-old boy who developed this syndrome after intramuscular injection of benzathine penicillin.</span>Iranian Society of DermatologyIranian Journal of Dermatology2717-072110Suppl.220071201Bullous mycosis fungoides: report of a case1010101292ENPouran LayeghMohammad Reza KalantariAmir Omidvar-BornaYalda Nahidi0000-0001-8161-6939Journal Article19700101<span>Mycosis fungoides is a common form of cutaneous T-cell lymphoma, with characteristic histopathology and chronic clinical course. Vesiculobullous mycosis fungoides is a rare occurrence. Here we present a 55-year-old man with multiple bullous plaques of one year duration who was finally diagnosed as a case of mycosis fungoides.</span>Iranian Society of DermatologyIranian Journal of Dermatology2717-072110Suppl.220071201Hereditary leiomyomatosis and renal cell carcinoma (HLRCC syndrome): a case report1111101293ENHayedeh GhaninejadFatemeh MoeineddinAhmadreza RajaeeAhmad SalimzadehMasoud AsgariJournal Article19700101<span>Recently multiple cutaneous leiomyomas, uterine leiomyoimatosis and renal cancer have been described as a cancer syndrome with autosomal dominant pattern of inheritance.</span><br /><span>We report a 79-year-old man who presented with multiple hyperkertotic erythematous nodules on his right leg with histological diagnosis of pilar leiomyoma. In his past medical history several systemic complaints such as gross hematuria, weight loss and bone pain were noted and further investigation confirmed histological diagnosis of metastatic papillary renal cell carcinoma. History of uterine leiomyomas and hysterectomy was peresent in his sister.</span><br /><span>All these findings in this case can be attributed to hereditary leiomyomatosis and renal cell carcinoma syndrome (HLRCC Syndrome).</span>Iranian Society of DermatologyIranian Journal of Dermatology2717-072110Suppl.220071201Report of a rare case of bullous mycosis fungoides1212101294ENKamran BalighiFatemeh MoeineddinAhmadreza RajaeeAzita NikooMostafa Mahdavi-niaJournal Article19700101<span>Mycosis fungicides (MF), on extremely rare occasions, can be associated with vesiculobullous eruptions. We describe a 74-year-old man with previous documented histopathologic diagnosis of poikilodermic type of MF who recently developed some flaccid acral bullae on erythemoatous MF plaque and normal skin. Histopathology and direct immunoflourscence studies revealed extensive lichenoid changes with intraepidermal bulla. Atypical lymphocyte infiltration was presented in dermoepidermal junction and bulla fluid and peripheral blood smear, but absent in lymph node and bone narrow tissue specimens. The plausible cause of blister formation in cutaneous lymphoma bullosa may be due to excessive epidermotropism or toxic effects of cytokines secrected by the tumoral infiltrate.</span>