Differential diagnosis of malformations of lymphatic vessels in dermatological practice

ABSTRACT

Lymphangiomas (LG) are uncommon, rare congenital anomalies or acquired lymphatic dilations of a benign flow that can involve the skin [1, 2]. There are main groups of lymphangiomas 1) a superficial variant, characterized by grouped vesicles (circumscriptum lymphangioma), 2) a deeper variant in the form of a cavernous lymphangioma. The prevalence of LG may be focal or diffuse. Secondary acquired LG with a rarer frequency are known [3, 4]. LG can be one of the manifestations of a symptom complex, for example, Gorham-Stout syndrome, which is characterized by progressive osteolysis [5]. The rare occurrence of LG, clinical diversity, undulating course of congenital forms, the possibility of an acquired nature of the disease causes a high risk of diagnostic errors in establishing the final diagnosis. At the Department of Dermatovenereology, Cosmetology and Additional Professional Education of Smolensk State Medical University for the period from 2018 to 2021, 5 patients (age from 5 to 17 years) with LG were observed. Of these in four children, the disease existed from birth, in one girl it had an acquired character and developed after covid infection [4]. Gender characteristics 4 girls (5, 6, 12 and 17 years old) and 1 boy (9 years old). All patients are urban residents. The time to establish the final diagnosis from the moment of seeking medical help ranged from 15 months to 12 (!) years, the average value being 6.5 years. The range of diagnoses of LG 'masks' herpetic infection, molluscum contagiosum, atopic dermatitis, contact dermatitis, epidermolysis bullosa. A frequent change in diagnoses was established in the same patient. Clinical case 1. The boy is 12 years old. The debut of skin lesions from birth and progression to 3 years of age, then spontaneous regression within 4 years (without signs of dermatosis). From 7 years to the present, there has been an increase in the number of rashes. Localization the skin of the lateral surface of the trunk. Features of the rash flesh-colored and/or reddish- purple bubbles. A pathognomonic symptom of 'frog calves' is found. The frequent autodestructive effect on the rash provokes its subsequent progression. Family history his father is a liquidator of the atomic catastrophe in Chernobyl. Previous diagnoses molluscum contagiosum, herpes zoster. Clinical case 2. The girl is 17 years old. The debut of the disease from birth. Lack of progression up to 5 years of age (up to 5 years of age did not apply to dermatologists). At the age of 5, she began to engage in rhythmic gymnastics (she continues to practice professionally at the present time) and noted an active increase in the number and size of the elements of the rash. She repeatedly consulted dermatologists diagnoses of molluscum contagiosum (laser removal), herpetic infection (courses of antiherpetic therapy without effect) were assumed. The diagnosis was established 12 years after the moment of seeking medical help. Unilateral location of the rash along the inner surface of the right upper limb with transition to the axillary region;on the right lateral surface of the body, the right inguinal-femoral fold and the inner surface of the right thigh. Focuses of a rash in the form of different sizes of vesicular elements with a tendency to lymphorrhea and oozing, areas of maceration around. Single elements with a hemorrhagic component. The patient notes an increase in the inflammatory response and vesicle lymphorrhea after each workout. Dermatoscopy yellow-pink lacunae alternating with single dark red lacunae. Histological examination multiple dilated lymphatic vessels in the papillary and reticular dermis. Clinical case 3. Girl 6 years old. Sick from birth. The diagnosis of LG of the genitals was established at the age of 1, 5 years. Due to the localization of the rash in the external genital area, the girl's parents (at the age of 1 month) consulted an obstetrician-gynecologist, who suggested a hemangioma and referred to a dermatologist. The disease is of a family nature her grandmother (on the maternal side) and her lder brother have similar rashes on the skin of the trunk and in the mouth. The diagnosis was verified by histological examination. The pathological process is localized in the area of the labia majora and labia minora multiple vesicular rashes with translucent contents, easily traumatized and accompanied by itching, were found. Conclusions: LG is a multidisciplinary problem, which is caused by mimicry of manifestations, varied localization and prevalence of the rash. To verify the diagnosis, the following algorithm should be followed 1) the debut more often at birth or in the first months of life (with the exception of acquired forms of LG);2) the nature of evolution a stable state or slow progression in the absence of traumatic factors;3) clinical features the formation of grouped deep vesicles that resemble 'frog eggs'. The color of the bubbles is transparent or red-purple due to the hemorrhagic component. LG lesions may have hemangioma components. It should be remembered about the frequent localization of LG on the mucous membrane of the cheeks, tongue and floor of the oral cavity, which can manifest itself as bleeding from the elements of the rash when chewing or when providing dental care;4) biopsy reveals dilated lymphatic vessels in the upper layers of the dermis.