Clinicopathological Correlation Generalized Maculopapules with Whitish Center

Czech version

Authors: M. Důra ^1,2; L. Řandová ¹; M. Petráčková ¹; J. Štork ¹
Authors‘ workplace: Dermatovenerologická klinika 1. LF UK a VFN ¹; Bioptická laboratoř s. r. o. ²
Published in: Čes-slov Derm, 100, 2025, No. 6, p. 231-234
Category:

Overview

The authors present a case of a 66-year-old patient with skin manifestations of disseminated maculopapules with a porcelain-white center and a rim of ectatic capillaries on the periphery. Six months after the onset of skin manifestation, neurological impairment resembling a stroke occurred, with diplopia and left-sided hemiparesis. Skin biopsy and brain magnetic resonance imaging performed 16 months after the onset of skin manifestation were consistent with the diagnosis of malignant atrophic papulosis. Progressive neurologic involvement led to the patient’s death 18 months after the onset of skin lesions. The authors present a current overview of knowledge about this disease.

Keywords:

histopathology – differential diagnosis – malignant atrophic papulosis – pathognomonic clinical signs – neurologic symptoms

Sources

ANKER, J. P., KAMINSKA-WINCIOREK, G., LALLAS, A. et al. The dermoscopic variability of Degos disease at different stages of progression. Dermatol Pract Concept, 2014, 4(3), p. 59–61.
BOLOGNIA, J. L., SCHAFFER, J. V., CERRONI, L. Dermatology. 5th Edition. Philadelphia: Elsevier/Saunders, 2024, 2 vol., p. 412–413.
DEGOS, R., DELORT, J., TRICOT, R. Dermatite papulo-squameuse atrophiante. Bull Soc Fr Dermatol Syphiligr, 1942, 49, p. 148–150.
JANG, M. S., PARK, J. B., YANG, M. H. et al. Degos-li-ke lesions associated with systemic lupus erythematosus. Ann Dermatol, 2017, 29(2), p. 215–218.
KALETA, K. P., JARIENĖ, V., THEODORIDIS, A., NIKOLAKIS, G., ZOUBOULIS, C. C. Atrophic papulosis (Köhlmeier-Degos disease) revisited: a cross-sectional study on 105 patients. J Eur Acad Dermatol Venereol, 2022, 36(11), p. 2190–2194.
KIM, E., MOTAPARTHI, K. Benign atrophic papulosis (Degos disease) with lymphocytic vasculitis and lichen sclerosus-like features. Am J Dermatopathol, 2018, 40(4), p. 272–274.
KIM, P. J., LYTVYN, Y., KASHETSKY, N., BAGIT, A., MUFTI, A., YEUNG, J. Clinical manifestations and treatment outcomes in degos disease: a systematic review. J Eur Acad Dermatol Venereol, 2021, 35(8), p. 1655–1669.
KÖHLMEIER, W. Multiple Hautnekrosen bei Thromboangiitis obliterans. Arch Dermatol Syphilol, 1941, 181, p. 792–793.
POCK, L., ŠIMLOVÁ, J., MACHÁČKOVÁ, R., KOSKUBA, J., HERCOGOVÁ, J. Papulosis maligna atrophicans (morbus Degos). Čes-slov Derm, 2007, 82(4), p. 190–193.
SATTLER, S. S., MAGRO, C. M., SHAPIRO, L. et al. Gastrointestinal Kohlmeier-Degos disease: a narrative review. Orphanet J Rare Dis, 2022, 17(1), p. 172.
THEODORIDIS, A., KONSTANTINIDOU, A., MAKRANTONAKI, E., ZOUBOULIS, C. C. Malignant and benign forms of atrophic papulosis (Kohlmeier-Degos disease): systemic involvement determines the prognosis. Br J Dermatol, 2014, 170, p. 110–115.
ZOUBOULIS, C. C., THEODORIDIS, A., MAKRANTONAKI, E. Inflammation and thrombo-occlusive vessel signalling in benign atrophic papulosis (Köhlmeier-Degos disease). J Eur Acad Dermatol Venereol, 2022, 36(11), p. 2195–2198.

Do redakce došlo dne 9. 12. 2025.

Adresa pro korespondenci:
MUDr. Miroslav Důra, Ph.D.
Dermatovenerologická klinika 1. LF UK a VFN
U Nemocnice 499/2 128 00 Praha 2
e-mail: miroslav.dura@vfn.cz

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Article was published in

Czech-Slovak Dermatology

2025 Issue 6