Acta Dermato-Venereologica 99-13CompleteContent | Page 27

SHORT COMMUNICATION 1297 Severely Itching Dermatitis and Palmoplantar Keratoderma as First Manifestation of T-cell Prolympho­ cytic Leukaemia Silke C. HOFMANN 1 , Konstantin KOUTCHER 2 , Mario FABRI 3 , Linus WAHNSCHAFFE 4 , Marco HERLING 4 and Ekaterina ZILBERMAN 5 1 Center for Dermatology, Allergy and Dermatosurgery, HELIOS University Hospital Wuppertal, University Witten/Herdecke, Heusnerstr. 40, DE-42283 Wuppertal, 2 Medical Clinic 1, Division of Oncology and Palliative Care, HELIOS University Hospital Wuppertal, Wuppertal, 3 Department of Dermatology, University of Cologne, 4 Department I of Internal Medicine, Center for Integrated Oncology Aachen-Bonn- Cologne-Duesseldorf, Excellence Cluster for Cellular Stress Response in Aging-Associated Diseases, and Center for Molecular Medicine Cologne, University of Cologne, Cologne, and 5 University of Witten/Herdecke, Germany. E-mail: [email protected] Accepted Aug 12, 2019; E-published Aug 13, 2019 T-cell prolymphocytic leukaemia (T-PLL) is a rare and aggressive haematological malignancy that typically presents with hyperlymphocytosis, splenomegaly and lymphadenopathy, but also with frequent cutaneous involvement (1, 2). To raise awareness of potential cuta- neous presentations underlying a T-PLL, we report here 2 patients who both initially had therapy-resistant pruritus and subsequently developed progressive cutaneous lesions mimicking atopic eczema. CASE REPORTS Patient 1. A 62-year-old man with a history of type 2 diabetes mellitus and depression initially presented with therapy-resistant generalized pruritus for the past 3 years. Examination revealed a very small number of erythematous, excoriated small papules on his trunk and legs (Fig. 1A, B), xerosis cutis, Herthoge’s sign posi­tivity, Dennie-Morgan infraorbital folds, and a white dermogra­ phism. Laboratory parameters showed a mild peripheral blood (PB) leukocytosis (12/nl, normal < 10) with a lymphocytosis of 4.68/nl (< 3.0), eosinophilia of 6% (< 4), and a serum lactate dehydroge- nase (LDH) of 421 U/l (< 266). Liver and kidney tests were within normal limits, as were total IgE, C-reactive protein (CRP), thyroid stimulating hormone (TSH), vitamin, and ferritin levels. Histology of an erythematous papule demonstrated spongiotic dermatitis. In the following months, the patient was hospitalized repeatedly due to persistent pruritus and newly developed palmar keratoderma (Fig. 1C). He received treatments with corticosteroids, photo­ therapy, and H1-blockers, all without significant benefit. Five months after initial presentation the patient reported weight loss of 7 kg within 2 months. Clinical examination revealed hepatosple- nomegaly and generalized lymphadenopathy. A mild leukocytosis (10.18/nl) entailed a lymphocytosis of 5.13/nl. Cytomorphology of PB smears revealed prolymphocytes with convoluted nuclei. The lymphocytes were predominantly mature CD4 + T cells, which expressed CD2, CD5 and CD7. Refined flow cytometry showed intracellular expression of the TCL1A oncogene and a clonal do- minance of the TCR-Vß-1 chain. Fluorescence in situ hybridization (FISH) analysis detected a deletion of TP53, a MYC amplification, and a 14q11-rearrangement. Those findings led to the diagnosis of mature T-cell leukaemia with cutaneous tropism, precisely T- PLL. A skin biopsy showed dense perivascular dermal infiltrates with atypical lymphocytes expressing CD3 and CD4 (Fig. 1D–F), but no epidermotropism. The patient received alemtuzumab th- erapy (anti-CD52 antibody) and his pruritic skin lesions resolved rapidly, but cessation of antibody therapy was necessary due to reactivation of cytomegalovirus. Six months after initial diagnosis an allogeneic stem cell transplantation (ASCT) was performed due to progressive disease. The subsequent complete remission is ongoing since August 2015. Patient 2. An 83-year-old woman presented in 2015 with a 10-year-history of extremely pruritic generalized maculopapular exfoliative rashes refractory to topical corticosteroids and ul- traviolet A (UVA)-phototherapy (Fig. 1G–I). Histology showed superficial spongiotic dermatitis and total IgE was elevated (214 kU/l, normal < 100) leading to the diagnosis of late-onset atopic eczema. A leukocytosis of 14.83/nl was noted at initial presenta- tion, but was attributed to previous systemic corticosteroid therapy. Fig. 1. Clinical and immuno­ histochemical findings. (A) Minimal eczema on the back and (B) pruritic papules on the lower legs (B) at initial presentation of patient 1. Two months later, he presented with additional palmar hyperkeratosis (C). Immunohistochemistry of skin lesions showed expression of (D) CD3 and (E) CD4 within dermal lymphocytic infiltrates (x200). (F) Proliferation marker Ki67 was expressed in 70% of the lymphocytes. (G-I) Patient 2 presented with generalized maculopapular erythematous lesions with exfoliation and erythematous infiltrates in the arm folds one year prior the diagnosis of T-PLL. (J) A blood smear showed medium sized lymphatic cells with oval- shaped nuclei, intermediate chromatin density and prominent eccentric nucleoli suggestive of prolymphocytes. This is an open access article under the CC BY-NC license. www.medicaljournals.se/acta Journal Compilation © 2019 Acta Dermato-Venereologica. doi: 10.2340/00015555-3288 Acta Derm Venereol 2019; 99: 1297–1298