Lentiginosis

Description:

Lentiginosis (Xing.: лентиго profuse, a melanosis lentikulyarny generalized) – group of the deryomatoz which are characterized by widespread rashes лентиго – the nevus pigmentosus of yellowy-brown or almost black color with a diameter of 1,5-3 mm with accurate contours appearing on a body irrespective of influence of sunshine (unlike freckles) and remaining constantly. Histologically in a basal layer of epidermis reveal the increased quantity of melanocytes, in a derma – accumulations of melanophores.

Lentiginosis reasons:

The disease concerns to group of hereditary lentiginoses which except a syndrome of Peyttsa-Egersa-Touraine includes inborn and tsentrolitsevy lentiginoses. The reason and the mechanism of development of a dermatosis are not finalized. Consider that the periorifitsialny lentiginosis results from the neuromesenchymal dysplasia caused by the genovariation which is transferred autosomal it is prepotent. There are messages on family cases. It is revealed that the pathological gene of STK.11 is a gene suppressor of a rvet of a tumor.

Lentiginosis symptoms:

The disease develops right after the birth or a bit later. Emergence of a multiple small nevus pigmentosus from light brown till black color, oval or roundish outlines is characteristic. Spots are localized around and in an oral cavity, on lips, especially lower, perinazalno, periorbitalno, is more rare - on extremities (palms and soles, a dorsum of fingers), a conjunctiva. A rash like лентиго can be limited and are located only in one of the above-stated areas. In an oral cavity there can be also papillomatous changes. Only the skin form of a disease meets very seldom. At most of patients the combination of damages of skin, mucous membrane to multiple polyps of digestive tract, especially small intestine is observed. With age Pigmentation becomes intensive and clinical manifestations a polypose of a stomach and intestines develop: pains, vomiting, bleedings, symptoms of impassability, invagination, secondary anemia, cachexia. The increased risk of malignant regeneration of polyps, preferential a stomach, a 12-perstny gut, a large intestine is noted. In literature cases of emergence of polyps on mucous membranes of other bodies (a bladder, a renal pelvis, an ureter, bronchial tubes, a nose) are described.

Histopathology. Increase in a pigment in keratinotsita, quantities of melanocytes in a basal layer is noted. In a derma accumulation of macrophages is observed. Polyps represent benign adenoma.

Treatment of a lentiginosis:

Surgical removal of polyps, dispensary observation for patients and inspection of relatives of the first degree of relationship is necessary.