Papuloerythroderma‐like cutaneous involvement of a CD62L− subclone of T‐cell prolymphocytic leukemia

We report the case of an 88‐year‐old Japanese man with erythrodermic involvement of T‐cell prolymphocytic leukemia (T‐PLL). He had a history of pharyngeal diffuse large B‐cell lymphoma successfully treated with polychemotherapy including cyclophosphamide and epirubicin, 6 years before the current illness. He presented with numerous reddish, coalescing, flat‐topped papules on the trunk and extremities, sparing the skin folds of the abdomen, the features of which mimicked those of papuloerythroderma. Immunohistochemistry showed perivascular and epidermotropic infiltration of CD3⁺ CD4⁺ T cells in the cutaneous lesion. However, flow cytometric analysis revealed that the skin infiltrating T cells were negative for surface CD4, and that CD3⁺ CD4^? CD8^? cells made up 92% of the T‐cell fraction of peripheral blood. The circulating atypical T cells had a round or oval nucleus and prominent nucleoli, and the deletion of chromosomes 6q, 13 and 17. These cytological profiles were consistent with those of T‐PLL and distinct from those of Sézary cells. The same T‐cell clone was detected in the cutaneous lesion and peripheral blood, but the expression of CD62L was absent in the skin infiltrates and present in the circulating cells. No specific mutation was detected in STAT3 or STAT5B. Although low‐dose oral etoposide had a beneficial effect on the skin rash, a fatal crisis of marked leukocytosis (169 × 10³/μL) occurred 19 months after the illness onset. CD62L‐leukemic cells of T‐PLL may infiltrate the skin to form papuloerythroderma‐like cutaneous lesions.     

Extramedullary acute leukemia developing in a pre‐existing osteoma cutis

Primary osteoma cutis (cutaneous ossification) is an uncommon disease in which there is bone formation within the skin in the absence of a demonstrable pre‐existing condition. Osteoma cutis is a chronic and benign condition. We report a case of a 45‐year‐old man who developed extramedullary acute leukemia with a myeloid immunophenotype (myeloid sarcoma) with its initial presentation within an isolated pre‐existing osteoma cutis in the post‐auricular scalp without evidence of systemic acute leukemia or chronic myeloid stem cell disorders. The tumor was surgically excised without complications. Four months later, acute leukemia recurred in the contralateral posterior mandible and showed an immunophenotype consistent with acute lymphoblastic leukemia/lymphoma. The patient now has been treated by standard protocols for acute leukemia. The diagnosis of an extramedullary acute leukemia is challenging because of its inconsistent clinical and histopathologic presentations. Extramedullary acute leukemia developing in a pre‐existing osteoma cutis is very unusual and has not been previously reported in the literature.     

Indolent,waxing and waning cutaneous presentation of HTLV‐1‐associated adult T‐cell leukemia/lymphoma in an HIV‐1‐positive patient

We present an unusual case of human T‐cell leukemia‐lymphoma virus type 1 (HTLV‐1)‐associated adult T‐cell leukemia/lymphoma in an human immunodeficiency virus (HIV) patient who presented with non‐diffuse, papular, waxing and waning cutaneous eruptions. The patient is a 61‐year‐old Haitian male with history of HIV on highly active antiretroviral therapy (HAART) who presented with multiple painful pink papules on his distal fingers and back for more than a year with a waxing and waning course. Skin biopsy demonstrated a CD4+, CD25+, CD8? lymphocytic proliferation with a clonal T‐cell receptor gene rearrangement. Peripheral blood demonstrated lymphocytosis with a CD4:CD8 ratio greater than 20:1 and an identical T‐cell receptor (TCR) clone as that in the biopsy. HTLV‐1 antibodies and PCR testing for HTLV virus were positive. Retrospective review of CBCs during the past 8 years demonstrated chronic lymphocytosis with a sharp increase in absolute CD4 counts corresponding to the onset of rash. The patient lacked systemic symptoms after 6 months follow‐up.     

Primary cutaneous T‐cell lymphoma presenting as mycosis fungoides with a T‐/null‐cell phenotype: report of two cases

Variations in the clinical and histological presentation of cutaneous T‐cell lymphoma (CTCL) can hamper diagnosis. We report two cases of a novel presentation of CTCL characterized by an aberrant immunophenotype with complete loss of pan T‐cell antigens including T‐cell receptor β chain and showing the clinical and histopathological appearance of erythrodermic and plaque‐stage mycosis fungoides.     

Neurotropic T‐cell lymphocytosis: a cutaneous expression of CLIPPERS

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is an inflammatory disease of the central nervous system that predominantly involves the pons and cerebellum and that improves with immunosuppressive treatment. Only recently described, the etiology is unknown, diagnosis is difficult and long‐term neurological sequelae may occur without aggressive treatment. Herein, we describe a 59‐year‐old woman who presented with subcutaneous nodules affecting her face, trunk, limbs and an indurated annular erythematous lesion on her forearm. This was associated with marked dysesthesia of her skin, refractory to treatment. There was a 4‐year history of dysequilibrium, vertigo, truncal and gait ataxia with progressive neurological symptoms. Skin biopsy of the annular nodular lesion showed a lymphohistiocytic infiltrate in dermis and subcutis with a striking lymphocyte‐dominant infiltrate that was perineural and formed a nodular collection extending along a prominent subcutaneous nerve. Immunophenotyping indicated a marked predominance of T cells that were CD3 positive with a 2 : 1 CD4 : CD8 ratio. Scattered histiocytes were present but no well‐formed granulomas or vasculitis. Magnetic resonance imaging studies showed changes in the pontine, brain stem and cerebellar region, which subsequently were defined as characteristic for CLIPPERS, but no brain biopsy was pursued. The marked neural skin symptoms and the cutaneous histopathological findings indicate that the skin may be an additional target organ in CLIPPERS, and the immune response may be directed against a common neural antigen. In radiologically typical CLIPPERS, identification of clinical skin lesions particularly subcutaneous nodules and biopsy may potentially form a basis for tissue diagnosis in this syndrome.     

Leukemia cutis in a patient with acute monocytic leukemia presenting as unique facial erythema

A 67‐year‐old woman was referred to our department with a 1‐month history of facial exanthemas. She had been diagnosed as having acute monocytic leukemia (French–American–British classification, M5b) based on the histological findings of bone marrow. Physical examination revealed diffuse edematous erythema on her cheeks, eyelids and glabella with scattered reddish papules. Histological examination demonstrated dense infiltration of atypical mononuclear cells in the dermis. Specific cutaneous lesions could occur in acute monocytic leukemia more frequently than in other types of leukemia, but rarely show symmetrical edematous erythema limited to the face.     

A unique presentation of calcinosis cutis in a patient with cystic fibrosis after double lung transplants

Calcinosis cutis is the deposition of insoluble calcium salts in the skin and subcutaneous tissue. We report the case of a 28-year-old Caucasian woman with cystic fibrosis in whom strikingly symmetrical and reticulate calcinosis cutis developed on the lower extremities, which was noted on histology to spare the eccrine glands. Careful review of the literature fails to reveal any previous report with these remarkable cutaneous and histologic manifestations.     

Regional incidences of adult T‐cell leukemia/lymphoma with cutaneous involvement in Japan

Between 2008 and 2015, 462 newly‐diagnosed adult T‐cell leukemia/lymphoma (ATLL) patients with cutaneous involvement were found from the nationwide registry for Japanese patients with cutaneous lymphoma, of which 391 were selected for the study. They ranged in age from 28 to 93 years (median, 69 years), and included 215 men and 176 women (male : female ratio = 1.2). The 391 patients comprised 193 (50%) with smoldering type, 52 (13%) with chronic type, 44 (11%) with lymphoma type and 102 (26%) with acute type. The total number of patients in Kyushu/Okinawa was 8.8‐times higher than that in Kanto, which was set as the reference value, while the estimated prevalence of human T‐lymphotropic virus 1 (HTLV‐1) carriers in Kyushu/Okinawa has been reported to be only 2.5‐times higher than that in Kanto. In this study, the annual incidence of ATLL per 100 000 residents in Kyushu/Okinawa was 32‐times higher than that in Kanto. Our results indicated the higher incidence rate of ATLL in the endemic area than those in the non‐endemic areas in Japan, compared with the regional differences of HTLV‐1 prevalence determined by serological HTLV‐1 screening for blood donors. In addition, this analysis revealed that regional differences of mycosis fungoides/Sézary syndrome incidence rates were very small compared with those of ATLL.     

Hypopigmented interface T‐cell dyscrasia: A form of cutaneous T‐cell dyscrasia distinct from hypopigmented mycosis fungoides

Hypopigmentation in cutaneous T‐cell lymphoproliferative disease should not always be equated with hypopigmented mycosis fungoides (MF). A form of hypopigmented pre‐lymphomatous T‐cell dyscrasia falling under the designation of the so‐called hypopigmented interface variant of T‐cell dyscrasia has recently been proposed. The aim of the present study was to establish hypopigmented interface T‐cell dyscrasia as its own entity apart from other T‐cell dyscrasias and MF using a patient case series. Twenty four cases of hypopigmented interface T‐cell dyscrasia were identified in the dermatopathology database of Weill Medical College of Cornell University. There were 17 females and seven males (mean age, 36 years). In children and adolescents, the patients were most commonly of African American extraction. Truncal photo‐protected areas manifesting as large solitary patches or multiple smaller macules were characteristic; disease progression to MF occurred in only one patient. The lesions responded to topical steroids and light therapy. The pathology was defined by a cell poor interface associated with degeneration of keratinocytes and melanocytes, and by lymphocytes whose nuclei showed low‐grade cerebriform atypia, and which expressed a significant reduction in CD7 and CD62L expression. In 50% of the cases, the implicated cell type was of the CD8 subset. Clonality was not identified. Hypopigmented interface T‐cell dyscrasia is a distinct entity separate from and rarely progressive to MF.     

Intralymphatic atypical T‐cell proliferation in a cutaneous hemangioma

We report an unusual case of atypical T‐cell proliferation involving the lymphatic vessels within a cutaneous hemangioma from an elderly woman. Despite the blastic morphology, the CD4 restricted phenotype and the very high proliferation index, the clinical presentation (single skin lesion in a healthy woman), the benign clinical course and the absence of T‐cell receptor (TCR) clonal rearrangement favored a reactive nature of the process. Since the atypical cells showed an effector/memory‐like regulatory T‐phenotype (CD45RO+, CD25+ and FOXp3+), expressed the migration‐associated molecule CCR7 and were exclusively located within podoplanin+ lymphatic vessels, we speculate that the process might reflect an unusual local immune response, with migration of T‐cells to draining lymph nodes. Ardighieri L, Lonardi S, Vermi W, Medicina D, Cerroni L, Facchetti F. Intralymphatic atypical T‐cell proliferation arising in a cutaneous hemangioma.