ECP 2022 Abstract Book

Virchows Archiv (2022) 481 (Suppl 1):S1–S364 13 E-PS-05-030 A case of pseudoxanthoma elasticum with severe, early-onset ocular manifestations D. Koumoundourou*, G. Kyriakou, V. Tzelepi *Patras University Hospital Pathology Department, Greece Background & objectives: Pseudoxanthoma elasticum (PXE), is a rare genetic metabolic disease caused by ABCC6 gene mutations. In PXE, dystrophic calcification frequently leads to cutaneous, ocular, cardio- vascular, neurological, and other manifestations. Herein we present a case with significant premature ocular manifestations. Methods: A 22-year-old female was referred to the Dermatology Department complaining for a “plucked chicken” appearance of the skin on her neck present for two years. Physical examination revealed multiple, small, yellowish papules on the lateral aspects of the neck. Thickened, lax and wrinkled skin was observed at the flexural surfaces of her body. A skin biopsy was performed. Results: Microscopically in the middle dermis, degenerative changes affecting the elastic fibres were recognized. The fibres were basophilic and irregular, appearing as widely dispersed granular material. Their presence was best evaluated using a Von Kossa technique. The diagnosis was compatible with pseudoxanthoma elasticum. The patient was immediately referred to the Opthalmology Department where a dilated funduscopic examination was performed and revealed the presence of bilateral angioid streaks accompanied by peau d’orange changes in the retina consisting of fine yellow drusenlike pigment irregularities. Conclusion: Although the cutaneous manifestations usually precede the ocular findings by many years, in our patient, they appeared concurrently. Moreover, macular involvement is rarely found before the age of 40 years. Despite the fact that there is no established therapy available for PXE, early diagnosis and regular ophthalmologic counseling are prerequisites in order to prevent and control the possible adverse events caused by the disease. E-PS-05-031 Histoprognostic factors of cutaneous melanoma in relation to metastasis at the sentinel lymph node H. Douik*, K. Tlili, G. Sahraoui, R. Doghri, L. Charfi, B. Hedhli, H. Guizani, K. Mrad *Salah Azaiz Institute of Cancer, Tunisia Background & objectives: Identification of histoprognostic factors of melanoma is closely related to the metastatic progression and provides an important argument for the management of patients. Our aim was to identify histoprognostic factors that can predict the sentinel lymph node metastasis in melanoma. Methods: Retrospective study of 38 melanoma with sentinel lymph node biopsy collected at Salah Azaiez Institute over a period of 15 years (January 2006-December 2021). We divided the cases into 2 groups according to sentinel lymph node status (positive or nega- tive). For each group (N+ and N- groups), we studied the following parameters: Breslow’sthickness, ulceration and mitotic rate. Results: Metastatic sentinel lymph node was found in 10 cases with median age of 64 years and without sex predominance. The median age in the N-group was 63 years with female predominance. For N+ group, Breslow depth varied from 7 to 11 mm with an aver- age of 8 mm versus an average of 5 mm in the N- group (1-22). The mitotic rate varied from 7 to 12 mitoses/10HF with an average of 8in N+ group and from 0 to 9 mitoses/10HF with an average of 6 in the other group.The ulceration was present in all N+ cases and in 80% of cases in the other group. Conclusion: The N+ group seems to have the worst histoprognos- tic factors. This study should be carried out on larger samples to be able to confirm our results E-PS-05-032 Cutaneous Kikuchi-Fujimoto disease (KFD) post COVID-19 vaccination: case report M.A. Barrios Villalba*, P. Pose Lapausa, J. Magdaleno Tapìal, V. Sabater Marco * Department of Pathology, Hospital General Universitario de Valencia, Spain Background & objectives: Kikuchi-Fujimoto disease is an usually cervical histiocytic necrotizing lymphadenitis, self-limited and of unknown aetiology. Recently, several patients with KFD have been described post COVID-19 vaccination. However, in this context, exclu- sively skin involvement has not been reported. Methods: A 45-year-old man with a history of HIV infection, stage A2, on antiretroviral treatment and HHV-8 associated mul- ticentric Castleman disease, presented multiple infiltrated erythe- matous nodular lesions on the forehead, cheek, arms and trunk, without lymphadenitis. Skin lesions appeared 2 days after receiving mRNA-based COVID-19 vaccine (Moderna) and due to clinical suspicion of lymphoma, a skin biopsy was performed. Results: Histopathological study showed epidermis with basal hydropic degeneration and dyskeratosis. The dermis presented a perivascular and periadnexal lymphohistiocytic infiltrate with karyorrhectic debris without neutrophils next to plasma cells. Immunohistochemically, the histiocytes expressed CD68, CD163, and myeloperoxidase. Most lymphocytes expressed CD3 and CD8, and plasma cells expressed CD138, Kappa and Lambda. Immu- nostaining for ALK, CD10, BCL6, BCL2, S100, CD1a, CD23, CD21, CD123, Treponema, and HHV-8 was negative. Real-time PCR revealed 30 copies of EBV/100,000 cells and no HHV-6. A diagnosis of cutaneous Kikuchi-Fujimoto disease post COVID-19 vaccination was reached. Currently, the patient is stable from his immunodeficiency and without skin lesions. Conclusion: Kikuchi-Fujimoto disease in post-COVID-19 immu- nization patients is a self-limited process and is clinically charac- terized by cervical lymphadenopathy and fever, but patients with HIV infection may present exclusively skin involvement probably due to their immunodeficiency. The temporal association between the administration of the COVID-19 vaccine and the appearance of skin lesions is a clue to suspicion. The definitive diagnosis is histopathological when demonstrating a dermal lymphohistiocytic infiltrate without neutrophilic karyorrhectic debris and co-expres- sion of CD68 and myeloperoxidase in the histiocytes. E-PS-05-033 Erythema elevatum diutinum in a patient with Crohn’s disease: a very rare variant of chronic cutaneous vasculitis G. Moreno De Juan*, G.G. Yange Zambrano, I. Gallego Gutiérrez, P. Martín Soler, M.S. Rodríguez Duque, C. González Vela *Hospital Universitario Marqués de Valdecilla, Spain Background & objectives: Erythema elevatum diutinum (EED) is a very rare variant of chronic cutaneous vasculitis that evolves into a concentric fibrosis. EED has been associated with hematologic disor- ders, autoimmune processes and infections. Very few cases have been described with inflammatory bowel disease. Methods: A 38-year-old man with history of factor VII deficiency and Crohn’s disease (CD) of 13 years of evolution, who consulted S214