Annular elastolytic giant cell granuloma in a patient with Ehlers–Danlos syndrome

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Abstract

The article presents a clinical case of annular elastolytic giant cell granuloma (AEGCG) in a young patient with a vascular type of Ehlers–Danlos syndrome. The first clinical manifestations of AEGCG appeared on the skin in the right subclavian area about two years ago. Subsequently, new rashes appeared on the skin of the upper and lower extremities up to four new foci per year. The patient underwent ambulatory therapy as a solution of calcium gluconate 10% 5.0 ml No 10 i/v in every other day; a solution of chloropyramine hydrochloride 1.0 ml No 10 i/m every other day; betamethasone + salicylic acid ointment applied to the affected areas of the skin 2 times a day for 2 weeks. The treatment was ineffective, the rashes did not regress. External therapy with tacrolimus was carried out next, 0.1% ointment 2 times a day in the form of applications for 24 weeks but also without effect. The patient by herself started to take a dietary supplement containing 400 mg of collagen in 1 tablet; 3 types of amino acids 20 mg; vitamins B2 1.1 mg; B6 1.5 mg; calcium pantothenate 5 mg 2 tablets a day during meals. A month after the start of the application, she noticed a slight paling of the rashes. At the moment, the patient is under follow up.

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Figure 1. Thin skin with translucent blood network, acrogeria. The patient's hands pressed the biopsy sites with gauze swabs.

Figure 2. Thin skin with a translucent blood network in the décolleté area.

Figure 3. The photo was taken on 04.09.2021. There are ring-shaped rashes on the skin in right area under the buttocks, formed by small, contiguous, dense, hemispherical, slightly flattened shiny red dermal papules.

Figure 4. The photo was taken on 04.01.2022. Ring-shaped rashes formed by small, contiguous, dense, hemispherical, slightly flattened dermal papules of red color have been preserved on the skin in right area under the buttocks. There is peeling on the surface of the papules. Positive dynamics compared to 04.09.2021: the color of the rash is paler; the resolution of the process is in the center of the focus.

Figure 5.

A - an annular focus on the right shin measuring 3 cm in diameter, arrow 1 indicates the location of the biopsy.

B - dermatoscopic picture of rashes on the border with healthy skin.

C - histological microphotograph of skin lesion: arrows 2 indicate giant multinuclear cells, arrow 3 shows the inflammatory infiltration in subepidermal zone. Hematoxylin and eosin, x200

D - histological microphotograph of skin lesion: arrows 4 indicate giant multinuclear cells; arrow 5 shows keratin cyst with keratin layers inside. Hematoxylin and eosin, x200

Figure 6.

A - four ring-shaped foci on the left shin, the maximum focus is 2.5 cm in diameter, arrow 1 indicates the location of the biopsy.

B - dermatoscopic picture of rashes on the border with healthy skin.

C - histological microphotograph of skin lesion: arrow 2 indicates keratin cyst with basophilic substance inside. Hematoxylin and eosin, x200

D - histological microphotograph of skin lesion: arrows 3 indicate giant multinuclear cells among the inflammatory infiltration. Hematoxylin and eosin, x200

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About the authors

Tatiana A. Gaydina

Pirogov Russian Scientific Research Medical University; Federal Center of Brain Research and Neurotechnologies of FMBA of Russia

Author for correspondence.
Email: doc429@yandex.ru
ORCID iD: 0000-0001-8485-3294
SPIN-code: 5216-2059

MD, Cand. Sci. (Med.), assistant professor

Russian Federation, 1, Ostrovityanova str., Moscow, 117997; 1, Ostrovityanova str., bldg 10, Moscow, 117513

Olga I. Patsap

Federal Center of Brain Research and Neurotechnologies of FMBA of Russia

Email: cleosnake@yandex.ru
ORCID iD: 0000-0003-4620-3922
SPIN-code: 6460-1758

MD, Cand. Sci. (Med.)

Russian Federation, 1, Ostrovityanova str., bldg 10, Moscow, 117513

Raisa T. Tairova

Pirogov Russian Scientific Research Medical University; Federal Center of Brain Research and Neurotechnologies of FMBA of Russia

Email: info@fccps.ru
ORCID iD: 0000-0002-4174-7114

MD, Cand. Sci. (Med.)

Russian Federation, 1, Ostrovityanova str., Moscow, 117997; 1, Ostrovityanova str., bldg 10, Moscow, 117513

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Supplementary files

Supplementary Files
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1. Fig. 1. Thin skin with translucent blood network, acrogeria. The patient’s hands pressed the biopsy sites with gauze swabs

Download (716KB)
2. Fig. 2. The photo was taken on 04.09.2021. There are ring-shaped rashes on the skin in right area under the buttocks, formed by small, contiguous, dense, hemispherical, slightly flattened shiny red dermal papules

Download (41KB)
3. Fig. 3. A — an annular focus on the right shin measuring 3 cm in diameter, arrow 1 indicates the location of the biopsy. B — dermatoscopic picture of rashes on the border with healthy skin

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4. Fig. 4. A fragment of the dermis with the presence of a scattered lymphocytic infiltrate, a giant multinucleated cell (circled with a black line). There is deposition of hemosiderin granules, fullness of capillary vessels, acantholysis sites, degradated elastic fibers. Staining with hematoxylin and eosin, ×400

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5. Fig. 5. Dystrophic and reactive changes of the epidermis, pronounced mitotic activity. There is no mucin deposition in the dermis. PAS stain, ×400

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6. Fig. 6. A — four ring-shaped foci on the left shin, the maximum focus is 2.5 cm in diameter, arrow 1 indicates the location of the biopsy. B — dermatoscopic picture of rashes on the border with healthy skin

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7. Fig. 7. In the dermis, fragments of collagen and elastic fibers with deformation and fragmentation, lymphocytic infiltrate (region 1), giant multinucleated cell (region 2). Staining with hematoxylin and eosin, ×400

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8. Fig. 8. Elastophagocytosis. A giant multinucleated cell with intracytoplasmic inclusion of an elastic fiber fragment was found subepidermally. Mallory stain, ×400

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9. Fig. 9. Elastolysis. Moderate hypertrophy and deformation of collagen fibers, degenerative changes and disorganization of elastic fibers in the dermis. Mallory stain, ×400

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Copyright (c) 2022 Gaydina T.A., Patsap O.I., Tairova R.T.

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