Darier Disease

DARIER, Jean (Jean Darier, born in 1856), a famous French dermatologist. He received his medical education in Paris (under Fournier and Bénard). From 1909 to 1923, D. held the post of Medecin de l'Hôpital S. Louis, also serving as honorary president of the French dermatological and syphilidological society and a member of the Medical Academy of Biological Sciences. D.'s numerous works include the highly popular "Anatomie et physiologie de la peau" (Pratique dermatologique, sous la direction de E. Besnier, L. Brocq et L. Jacquet, t. I, P., 1900) and the textbook "Précis de dermatologie" (4th ed., P., 1928), translated into many languages. D. was the first to describe follicular psorospermosis (1889; see Darier disease), cutaneous tuberculides ("Des tuberculides cutanées", Annales de dermatologie et syphiligraphie, t. VII, 1896), sarcoids ("Deux nouveaux cas de sarcoïdes multiples sous-cutanées", Bulletin de la Société française de dermatologie et syphiligraphie, t. XV, 1904), and melanosarcoma ("Melanoses, melanomes et melanosarcomes", ibidem, t. XXXII, 1925). DARIER DISEASE (syn.: keratosis follicularis, keratosis vegetans, ichthyosis follicularis, ichthyosis sebacea, psorospermosis follicularis vegetans), a dermatosis first described by D. in 1899 under the name of vegetating follicular psorospermosis; subsequently this disease was named after Darier. Over 50 cases of Darier disease have accumulated in the literature; among them some belong to Russian authors (works by N.P. Mansurov, T.P. Pavlov and I.F. Zelenyev). The disease is considered rare. The clinical picture is characterized by dense nodules ranging in size from a pinhead to a lentil, pink, yellow-brown or brown in color. The nodules are densely arranged, covered with a yellowish-brown crust or scale and sit on a reddened base. Upon removal, the surface is found to be dry or weeping, especially in areas prone to excessive sweating. On the scalp, the process proceeds under the guise of seborrhea or as flat nodules and wart-like formations. The nodules almost always correspond to individual follicles and are located mainly on the scalp, face, especially in the temple and nasolabial folds, and less often on the chin; further favorite sites of involvement are the external auditory canal, the area of the ears, the neck, the axillary fossae, the area under the breasts, the abdomen (especially in the navel region), the groin folds, the external genital organs, the anus, and the spine (especially in the sacral part). Sometimes nodules are observed on the back, below the scapulae, and on the lateral parts of the trunk. On the upper and lower extremities, the flexures are rarely affected, but the hands and feet are relatively often affected, and the eruptions here have a somewhat different character. As observations by Buzzi, Schwenninger, and Pavlov have shown, these lesions are often in the form of keratotic calluses with an indentation in the center, which are considered especially characteristic of this disease. In recent times, attention has begun to be paid to mucosal involvement in the form of flat red nodules on the lips, tongue, hard and soft palate, and the conjunctiva. The nails are often affected, but without the distinctive signs characteristic of the disease; they appear dull and pitted with furrows. Lymph nodes rarely enlarge. The disease is almost not accompanied by subjective sensations (occasionally only complaints of weeping and mild itching). The age of patients ranges from 20 to 35 years, but in a number of described cases, children became ill, even in the first months of life; usually the disease begins in the second decade of life. The observation of Hallopeau of a patient aged 60 is exceptional. Both sexes can contract the disease (men more often); the disease has a definitely hereditary character, often occurring in one family. In all described cases, the majority of patients were degenerates; for example, of 4 cases in the skin clinic of MSU, two patients suffered from dementia, and two others were mentally underdeveloped. Since RW was positive in some observations, there is an opinion (Burnier, Rejsek) that hereditary syphilis may play an etiological role. In sharply expressed cases, the diagnosis is not difficult, however, the true picture of the disease can often be obscured by eczema phenomena, and only after removing the crusts is the nature of the lesion clarified. Weeping and localization behind the ears and on the head can lead to confusion with seborrheic eczema, the nodules of which, however, are less distinct. With lichen planus, the nodules have much in common, but differ from them by greater moisture, absence of polyhedrality, gloss, and central indentation. In addition to this, the localization of nodules in Darier disease is different: mainly the head is affected, while in lichen planus the extremities are affected. Mucosal involvement can also easily be confused, therefore it should be borne in mind that nodules on the mucosa in Darier disease are denser and lack the gloss characteristic of lichen planus. Hyperkeratosis on the palms and soles can resemble hereditary keratoma, differing from the latter by central indentation and the presence of other accompanying signs of Darier disease on the head and mucous membranes. Histologically, there is hypertrophy of the epidermis, mainly of the horny and granular layers, which wedge-shapedly penetrate the Malpighian layer. Due to the growth of the mentioned layers, the papillae elongate and soon atrophy. As a result of the separation of the epidermis from the papillary layer, so-called Becket's fissures and vacuoles (Beck) of various shapes and sizes are formed. In addition, a large number of cornified bodies containing grain nuclei (grains) are observed in the horny layer. In the granular and Malpighian layers, homogeneous, shiny, round bodies (corps ronds) are visible, surrounded by a double shell, which, contrary to D.'s initial view, which considered them psorospermia or parasites of the genus Protozoa, are nothing other than improperly cornified epidermal cells. The etiology of Darier disease is still not entirely clear; apparently it is developmental anomalies. The course of the disease is chronic, it drags on for years, sometimes until death; only temporary remissions and insignificant improvement occur, which depends on the general condition of the patient. Diseases of internal organs can cause new flare-ups of the dermatosis. Combinations of Darier disease with other dermatoses have been described, for example with ichthyosis (Burnier and Rejsek) or with trichophytia (the case of Brusilovskaya from the clinic of MSU); however, these combinations are merely coincidental. The prognosis for life is favorable, for complete cure - none. For treatment, the following are recommended: arsenic injections, green soap rubs, thermocauterization, radiotherapy, radium (Iordan), pyrogallic acid, thyroidin internally. In recent times, intravenous infusions of 10% Natr. hypersulfurosum have been used with success. As with any chronically running disease, all these means and methods of treatment bring only relative success.