Dyskeratosis congenita without oral involvement: a rare hereditary disease

Dyskeratosis congenita [DC] is a rare, progressive, multi-system, inherited disorder of telomere biology, first described in 1906 as the Zinsser-Engman-Cole syndrome. The condition presents with the classic triad of nail dystrophy, reticulate skin pigmentation, and oral leukoplakia. Variable somatic abnormalities may be present; these include pulmonary, gastrointestinal, genitourinary, cerebral, and dental complications. Pancytopenia eventually develops, progressing to bone marrow failure. In our patient, the progression of DC did not follow the [classic triad] typically seen in the condition. Here we report the case of a 27-year-old male who had features consistent with this syndrome but who had a normal oral cavity associated with aplastic anemia for which longstanding nail dystrophy and reticular pigmentation were the dominant clinical features. The patient was advised to avoid smoking and severe exposure to the sun, and to return regularly for follow-up. Because all symptoms of this disorder are not necessarily seen collectively in any one patient, physicians should consider it as a probability in all patients showing signs of reticular hyperpigmentation, especially accompanied by aplastic anemia. Our report emphasizes the fact that not all components of the syndrome need to be present and in all cases of genodermatosis, pleomorphism may be expected with variable clinical manifestations. Even though many variants have been described in literature, sparing of the oral cavity as in our patient is extremely uncommon. Therefore, physicians should be aware of the mucocutaneous manifestation of DC and its diagnosis and refer the patient for better evaluation

Incidence of non-melanoma skin cancers in Isfahan

Non-melanoma skin cancers [NMSC] are one of the most prevalent malignancies of the human. Although the morality rate is not high for these cancers, they may cause high mortality for the patients. In addition to the skin type, factors such as sun exposure, head and neck radiotherapy, inappropriate use of sunscreen and cultural changes may increase the incidence of these cancers. The objective of this study was to determine NMSC in Isfahan. We used data of the cancer resistery center for performing this study and the incidence of NMSC per 100,000 papulation was determined. Overall 1681 cases were registered. The incidence of NMSC was calculated to be 10.67/100.000 population. The most common site for BCC and SCC was middle face. NMSC incidence in Isfahan is lower than west countries although this incidence is 10.67/100,000 in a year. It means that 5-6 thous and cases of NMSC occur in Iran each year. We can prevent its morbidity and mortality by early correct diagnosis

Dermatology case diagnosis, Steatocystoma multiplex

A patient with multiple subcutaneous mobile lesions limited to forearms is presented. Pathologic review of the biopsy confirmed the diagnosis

Efficacy of 3% diclofenac gel for the treatment of actinic keratoses: a randomized, double-blind, placebo controlled study.

BACKGROUND: Actinic keratoses (AKs) are premalignant skin lesions caused by excessive sun exposure. AIMS: To explore the therapeutic efficacy of 3% diclofenac in 2.5% hyaluronan gel in the topical treatment of AK. METHODS: Sixty-four lesions in 20 patients were evaluated. They were randomized to receive either the active treatment, 3% diclofenac in 2.5% hyaluronan gel or placebo, which consisted of the inactive gel vehicle, hyaluronan for a period of three months. The collected data were analyzed by using Student t- tests. RESULTS: There was a reduction in the lesion size in 64.7% of diclofenac-treated lesions and 34.3% of control lesions during the three-month course of treatment. Only 9.3% of the lesions in the diclofenac group were completely cleared during three months of treatment. During the treatment, no significant side-effect was observed in both groups. CONCLUSION: Considering the malignant potential of actinic keratoses and the importance of clearing them to prevent their transformation to squamous cell carcinoma, the efficacy of diclofenac gel seen in our study seems to be low. This treatment may be useful for patients who do not tolerate other, more effective kinds of treatment for actinic keratoses.

[Disseminated cutaneous leishmaniasis on lymphedema following radiotherapy: a case report]

Cutaneous leishmaniasis[CL] is a parasitic disease, which is hyperendemic in Isfahan, usually caused by L.major and L.tropica. Herein we report a patient with post-mastectomy lymphedema on right upper limb accompanying with the lesions of cutaneous leishmaniasis on the right and left forearms. Following radiotherapy, the lesions on the limb with lymphedema were disseminated. But the lesions on left side showed no change. This finding may be the result of immune disorder due lymphedema and radiotherapy