Iranian Journal Of Dermatology
Volume:1 Issue: 3, 1998

Thalidomide, a hypnosedative drug introduced in the 1950, has been used in a variety of dermatologic conditions during the past few decades. Although originally withbrawn from world market on discovery of its teratogenic effect, it has since been selectively reintroduced for use in various disorders thought to have an autoimmune or inflammatory basis. A review of the literature focused on clinical uses of thalidomide in the treatment of dermatologic diseases was performed. Diseases for which thalidomide has been found effective include erythema nodosum leprosum, prurigo nodularis, actinic prurigo, discoid lupus erythematosus, aphthous stomatitis, Behcet’s syndrome, and graft versus-host disease. Side effects such as teratogenicity and peripheral neuropathy remain its limiting factors. Thalidomide is a useful addition to the therapeutic armamentarium for treatment-resistant dermatoses as long as proper vigilance for adverse effects is maintained.

There is not yet a pathognomonic laboratory test for the diagnosis of Behcet’s Disease (BD). The diagnosis is therefore based on clinical manifestations. Mucocutaneous lesions are the most important and the most frequent clinical manifestations of BD.

To study the mucocutaneous lesions systematically. In a great number of patients. Some of these clinical pictures are poorly defined in the literature, and some of them are still unknown.

From 1992 to 1998 we screened systematically all patients attending the Behcet’s Disease Research Unit (BDRU), Rheumatology Research Center, Tehran University for Medical Sciences, for mucocutaneous lesions. Only recurrent lesions were taken in consideration. A total of 3751 patients were seen and registered in our database. The mean number of new patients seen every year, during the last 10 years, was 302 patients per year.

Besides the classical lesions, we observed unusual and rare forms of mucous membrane, cutaneous, and subcutaneous lesions. Mucous Membrane lesions: 1) Oral aphthosis is the most important lesion of BD (96.1%±0.6). They are not different from other aphthosis, but they are often smaller. There are also the Punctiform, the Miliaria, and the Herpetiform aphthosis. 2) Genital aphthosis was seen in 63.9%±1.5 of patients. In females they are often larger, deeper, and very painful. Giant forms are seen sometimes. In male they are seen more often on the scrotum. Recurrent conjunctival aphthosis is rare. It is small and ephemera. 3) Ulceration and erosions are different from aphthous lesions. They can take multiple and various shapes without a specific characteristic. 4) Purpura and erythema may also be seen on the mucous membrane. Skin Lesions: Is the second most frequent manifestation of BD. It was seen in 67.4%±1.5 of the patients. The pathergy phenomenon, which is a skin hyper reactivity to trauma, was seen frequently in BD. The most important cutaneous lesions of BD is the pseudo folliculitis. It is a small erythematous lesion, surmounted in the center by a round and non-acuminated pustule, which is dome shaped, sterile, and without hair in the center. They are situated mainly on the lower limbs. Other lesions are 1) Small round erythemato-edematous lesions. 2) Cutaneous aphthosis, which is the most characteristic lesion of BD. It is a round and punched out ulceration with a white yellowish necrosis on the bottom. It leaves a scar after healing. 3) Small nodules. 4) Behcet’s Cellulitis; it is a large painful erythematous lesion. It is not infectious nor a superficial thrombophlebitis. In the literature, it was mistakenly taken as Sweet syndrome. The biopsy in all lesions shows a vasculitis, leucocytoclastic or lymphocytic. Subcutaneous Lesions: Erythema nodosum is seen in 22.8%±1.3 of cases. It is a frequent and relapsing lesion. It is a subcutaneous node, painful, red mauve, with different sizes, and often surrounded with an erythematous and edematous ring. Other lesions are rarely seen 1) Subcutaneous lesions, like Erythema Induratum of Bazin. 2) Suppurative panniculitis.

Our study showed a wide variety of muco-cutaneous lesions. The lesions are not specific of BD, but they have their own characteristic. Those characteristics, along with the coexistence of different forms, can help to diagnose BD.

Cutaneous leishmaniasis caused by leishmania tropica is endemic in Iran. Kerman is the fifth most infected foci of dry type of cutaneous leishmaniasis after Tehran, Mashhad, Naishabor and Shiraz. We studied the clinical features of this disease in 664 patients referred to the dermatology and private clinic during the period March 1992 to March 1993 in Kerman. Females were dominated. The disease was more frequent below 10 years of age (50%). Papule and plaque were the most common clinical presentations of the disease followed by papule+plaque. The face was the most frequent site of involvement (30%); followed by right and left upper extremities. The trunk was the least common site (0.4%). Many of the patients were presented with one lesion, and the highest number of lesions in one patient was ten. The highest number of patients came with acute leishmaniasis (Duration of disease less than 1 year) and few patients with chronic from (Duration of disease more than 1 year).

Thirty to forty percent of the patients referred to dermatologic clinics are estimated to have psychiatric problems. These patients either are those who have developed dermatologic problems as a consequence of their primary psychiatric disorders or those who have developed psychiatric disorders following their chronic and disfiguring dermatologic problems. The third group are psychophysiologic disorders which may aggravate following psychiatric or emotional stress.

Our purpose is to stress the significance of the psychodermatologic problems for both dermatologists and psychiatrists; to have an overview and a brief classification of these and discussing the approach to the patients with psychodermatologic disorders.

The dermatologists and psychiatrists’ pitfalls in approaching the psychodermatologic disorders are discussed. Psychodermatologic disorders are classified and their underlying psychopathologies are mentioned.

Both dermatologists and psychiatrists should be trained more to manage these relatively frequent patients well; for this purpose establishing the dermatology-psychiatry liaison clinics and or helding the dermatologic-psychiatric conferences are advised.

Rosacea is a chronic disorder of unknown etiology with facial and ocular manifestations. It frequently affects women in the fourth to seventh decades, and patients often present to ophthalmologists with complaints relating to blepharitis, meibomianitis, or tear disturbances. Characteristic skin change of flushing, erythema, telangiectasis, papules, and pustules should be sought, as patients frequently benefit from treatment. At this time, oral doxycycline and topical metronidazole, combined with local measures for ocular manifestations and appropriate patient counseling appear to be the best management options.

Harlequin Ichthyosis is a dermal disorder that always lead to an early death after birth. Although the clinical characteristics of this disorder has been described perfectly but the molecular basis of which isn’t clear well. Harlequin fetus is an Autosomal recessive trait and prenatal diagnosis is possible by embryo skin biopsy after fetoscopy. This case was a male newborn who died 4 days after the birth. The parents of the infant have a consanguineous marriage and live in a village around Shahrood (East of Iran). This family had also another infant with same disorder, who died also on 4 days of her life but they have a 7 years old healthy girl.

A 47-year-old man with 3 years history of a pruritic plaque, 1×3 cm on his left leg attended a dermatology clinic. An incisional biopsy was done with the differential diagnoses of: 1) Hypertrophic lichen planus, 2) Lichen simplex chronicus, 3) Psoriasis, 4) Bowen's disease. A diagnosis of “Fibroepithelioma” was suggested by histopathologist who was not compatible with clinical diagnosis. Careful review of slide showed that the specimen had been cut horizontally in the laboratory, which had misled the pathologist. By vertical sectioning the block and doing another biopsy, the diagnosis of hypertrophic lichen planus was confirmed. The patient was treated with intralesional triamcinolone injection and 4 weeks later the lesion was completely healed with residual hyperpigmentation.

Dermatofibrosarcoma protuberans is a tumor of intermediate malignancy characterized by its aggressive local growth and marked propensity to recur after surgical excision. Many forms of the tumor has been reported. We report a pedunculated form of tumor. A 45-year-old woman felt a growing tumor on her trunk for 6 years but did not seek medical consultation until it had ulcerated 6 months ago. Skin biopsy confirmed the diagnosis of dermatofibrosarcoma protuberans. Radiological and sonographical examination failed to reveal any metastatic lesion.