Iranian Journal of Dermatology

Abstract 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.

Abstract 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.
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.
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.

Abstract 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.

Abstract 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.

Abstract Psoriasis 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.  

Abstract 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.

Abstract 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.

Abstract 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.
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.

Abstract 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.
Here we report a 16-year-old boy who developed this syndrome after intramuscular injection of benzathine penicillin.

Abstract 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.

Abstract Recently multiple cutaneous leiomyomas, uterine leiomyoimatosis and renal cancer have been described as a cancer syndrome with autosomal dominant pattern of inheritance.
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.
All these findings in this case can be attributed to hereditary leiomyomatosis and renal cell carcinoma syndrome (HLRCC Syndrome).

Abstract 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.