Co-Occurrence of Pityriasis Amiantacea and Cutis Verticis Gyrata Secondary to Leukaemia Cutis of the Scalp.

Introduction: Pityriasis amiantacea (PA) is a rare presentation characterized by the presence of extensive adherent scaling that tightly encircles and affixes tufts of hairs secondary to inflammatory or infectious dermatoses. However, the occurrence of PA as a consequence of leukaemia cutis has not been previously reported. Case Report: A 32-year-old man with B-cell acute lymphoblastic leukaemia (B-ALL) presented with severe scalp scaling and hair loss for 2 months. Examination revealed extensive, tightly adherent scales encircling and binding down hairs, along with haemorrhagic crusts. Skin examination showed nontender, partially blanchable papules forming ill-defined plaques, giving a cobblestone appearance. Trichoscopy revealed white crusts, tufting, and micro-haemorrhages. Scalp biopsy confirmed precursor B-ALL infiltration. He was diagnosed with PA with secondary cutis verticis gyrata due to leukaemia cutis and referred to a haemato-oncologist for further management. Discussion: Leukaemia cutis is a rare condition where leukaemia cells infiltrate the skin, often associated with acute myeloid leukaemia and ALL. It can lead to secondary verticis gyrata. The co-occurrence of PA and cutis verticis gyrata is rare and previously unreported, highlighting the need for increased awareness among clinicians.

Long-term remission of extramedullary cutaneous relapse of acute myeloid leukaemia (leukaemia cutis) treated with decitabine-venetoclax.

In February 2020, a 74-year-old female was diagnosed with myelomonocytic acute myeloid leukaemia with FLT3 mutation and blasts positive for CD33, BCL-2 and CD68/PGM1. Not responding to a standard Cytarabine-containing regimen plus Midostaurin, the patient achieved a complete remission (CR) of the disease in the bone marrow following a reinduction therapy with high-dose Cytarabine but simultaneously relapsed developing leukaemia cutis with disseminated lesions in 80% of the body surface area. After receiving 10 cycles of Decitabine plus Venetoclax the patient achieved and maintains a continuous CR.

A case of leukaemia cutis in a dog with T-cell chronic lymphocytic leukaemia.

Leukaemia cutis (LC) is the infiltration of neoplastic leukocytes into the skin, characterised by haemorrhagic papules, nodules, and plaques. LC has been reported in human leukaemia patients, but it is extremely rare in dogs. A 13-year-old spayed female Golden Retriever that was previously diagnosed with chronic lymphocytic leukaemia was managed with chlorambucil (20 mg/m2 orally, every 2 weeks) and prednisolone (2 mg/kg orally, every other day) for 8 months; however, immunosuppression was temporarily discontinued because of a bacterial urinary tract infection. Cutaneous signs, including multifocal ecchymosis and white plaques, appeared 1 month after cessation of chemotherapy. Histopathological examination revealed small- to intermediate-sized lymphocytes with mild atypia in a perivascular to interstitial pattern within the superficial dermis. The bands of atypical cells within the superficial dermis were strongly and extensively positive for CD3 on immunohistochemistry. Polymerase chain reaction analysis of the biopsied skin revealed clonal rearrangement of the T-cell receptor gamma locus gene. Given the evidence of clinical signs, peripheral immunophenotyping, histopathology, immunohistochemistry, and clonal gene arrangement, LC was diagnosed. The lesions disappeared when chemotherapy was restarted but were occasionally observed when chemotherapy was stopped. To the authors' best knowledge, this is the first case report of LC in a dog.

Leukaemia cutis for clinicians, a literature review.

Leukaemia cutis (LC) describes infiltration of the skin by leukaemia cells, resulting in clinically identifiable cutaneous lesions. LC has a wide range of clinical manifestations, which can make it difficult to distinguish LC from other skin changes. In a group of patients, LC can be the first manifestation of leukaemia, therefore skin biopsy is crucial for the diagnosis. In this mini review, we discuss various types of leukaemia most frequently represented in leukaemia cutis, in both children and adults and skin changes in multiple myeloma, focusing on the clinical presentation of LC and prognosis in patients.

Specific cutaneous infiltrates in patients with haematological neoplasms: a retrospective study with 49 patients.

BACKGROUND: Haematological neoplasms account for around 9% of all cancers, and they are recognised as an important cause of skin infiltration. However, studies analysing cutaneous metastasis of haematological neoplasms are scarce. We describe the clinical spectrum and outcomes of specific cutaneous manifestations of leukaemias, lymphomas, multiple myeloma (MM), and blastic plasmacytoid dendritic cell neoplasm (BPDN) and make a review of the literature. METHODS: Data from 49 patients diagnosed with secondary cutaneous infiltration of systemic haematological neoplasms over the last 10 years in a tertiary dermatology centre were retrospectively collected, and clinical-evolutive features were analysed. RESULTS: Most cases were lymphoma (44.9%, n = 22), followed by leukaemia cutis (38.8%, n = 19), secondary plasmacytoma (10.2%, n = 5) and BPDN (6.1%, n = 3). Nodules were the predominant type of lesion, and most patients presented with multiple (≥3) lesions. In 51% (n = 25) of cases, cutaneous infiltration was detected before the diagnosis of the underlying malignancy. The patients in diverse nosological groups did not differ in terms of survival (P = 0.052). CONCLUSIONS: We recognise the clinical heterogeneity of specific cutaneous infiltrates. The high proportion of cases in which skin involvement was key to the diagnosis of systemic malignancy emphasises the role of the dermatologist in recognising and correctly managing these patients.

Neutrophilic dermatosis: a new skin manifestation and novel pathogenic variant in a rare autoinflammatory disease.

Sideroblastic anaemia, B-cell immunodeficiency, periodic fever and developmental delay (SIFD) is caused by mutations of TRNT1, an enzyme essential for mitochondrial protein synthesis, and has been reported in 23 cases. A 6-month-old girl was evaluated with recurrent fever, failure to thrive, skin lesions and anaemia. She received blood transfusions and empirical antibiotics. Skin lesions, previously interpreted as insect bites, consisted of numerous firm asymptomatic erythematous papules and nodules, distributed over trunk and limbs. Skin histopathology revealed an intense dermal neutrophilic infiltrate extending to the subcutaneous, with numerous atypical myeloid cells, requiring the diagnosis of leukaemia cutis, to be ruled out. Over the follow-up, she developed herpetic stomatitis, tonsillitis, lobar pneumonia and Metapneumovirus tracheitis, and also deeper skin lesions, resembling panniculitis. Hypogammaglobulinaemia was diagnosed. An autoinflammatory disease was confirmed by whole exome sequencing: heterozygous mutations for TRNT1 NM_182916 c.495_498del, p.F167Tfs * 9 and TRNT1 NM_182916 c.1246A>G, p.K416E. The patient has been treated with subcutaneous immunoglobulin and etanercept. She presented with developmental delay and short stature for age. The fever, anaemia, skin neutrophilic infiltration and the inflammatory parameters improved. We describe a novel mutation in SIFD and the first to present skin manifestations, namely neutrophilic dermal and hypodermal infiltration.

Cutaneous-group histiocytoses associated with myeloid malignancies: A systematic review of 102 cases.

BACKGROUND: Histiocytoses are haematological disorders of bone marrow origin that share many biological and clinical features with haematological neoplasms. The association between histiocytoses of the cutaneous-group and myeloid malignancies is a poorly investigated topic of high biological and clinical impact. METHODS: We performed a systematic review of the scientific literature, compliant with PRISMA guidelines, to unravel the clinical and pathological features of this intriguing association. FINDINGS: We gathered and analysed 102 patients. Most were children with generalised cutaneous eruptions and displayed risk organ involvement (i.e. bone marrow, spleen, liver). Interestingly, all these features are uncommonly encountered in C-group histiocytosis not associated with haematological neoplasms. CONCLUSIONS: Our review shows that generalised eruptions and risk organ involvement in cutaneous-group histiocytosis should raise a suspicion for a concomitant myeloid neoplasm both in children and in adults and warrant further investigations. A rapid recognition of this association is required to start a prompt and effective therapeutic management given the aggressive behaviour of the associated myeloid neoplasm in most instances.

Leukaemia cutis as a specific skin involvement in chronic myelomonocytic leukaemia and review of the literature: Acknowledgments.

The skin involvement of myeloid leukaemia is conventionally divided into specific malignant lesions and non-specific benign lesions, and these categories are also applicable in chronic myelomonocytic leukaemia (CMML). According to the 2016 World Health Organization (WHO) classification of tumours of haematopoietic and lymphoid tissues, CMML is defined as a myeloid neoplasm with characteristics of myelodysplastic syndrome (MDS) and myeloproliferative neoplasms (MPNs). As a specific cutaneous sign of extramedullary infiltration, leukaemia cutis (LC) is a rare occurrence in patients with CMML, and only approximately 89 cases have been reported in the literature thus far. The clinical features of LC are varied, and LC in CMML exhibits heterogeneous histopathologic features, with manifestations as cutaneous nodules or papules that are composed of blast cells showing either granulocytic or monocytic differentiation. Skin biopsy and further immunohistochemical examination are essential at the time of diagnosis to evaluate pathological type and determine the clinical course. Generally, once diagnosed as LC in CMML, this unusual skin lesion might be an indicator of transformation to acute myeloid leukaemia (AML) and is associated with a poor prognosis. The main treatment is allogeneic stem cell transplantation (ASCT). Therefore, early diagnosis and accurate identification have important therapeutic and prognostic significance in CMML patients with skin infiltration.

Vemurafenib-induced histiocytoid neutrophilic panniculitis simulating myeloid leukaemia cutis.

Neutrophilic panniculitis is an infrequent but characteristic adverse event under therapy with BRAF inhibitors (BRAFi). Since the approval of vemurafenib for treatment of metastatic melanoma in 2011, only two cases of neutrophilic panniculitis in malignancies other than melanoma have been published. Histiocytoid infiltrates of immature neutrophils resembling histiocytes or myelocytes have been reported in Sweet's syndrome and rarely in other neutrophilic dermatoses. We describe a novel variant of neutrophilic panniculitis with histiocytoid myeloid cells in an early lesion from a patient treated with vemurafenib in combination with an anti-EGFR (epidermal growth factor receptor) agent for metastatic colon carcinoma, three weeks after initiation of therapy. Recognizing this variant of panniculitis associated to BRAFi can avoid misinterpretation of the atypical subcutaneous infiltrate as myeloid leukaemia cutis.

Aleukemic Leukemia Cutis Presenting as a Sole Sign of Relapsed Paediatric Acute Lymphoblastic Leukemia.

The author describes paediatric case of relapsed acute lymphoblastic leukaemia (ALL) presented as aleukemic leukaemia cutis (ALC). A 2 year old child was admitted in tertiary oncology centre. He suffered from pre B cell ALL with absent Philadelphia chromosome. This patient received multiagent induction chemotherapy as per Berlin-Frankfurt-Munster (BFM) protocol for ALL. He achieved remission after 28 days of treatment. Subsequently he presented with multiple skin lesions in the form of multiple small erythematous violaceous macules, papules, plaques and nodules on face, chest and back regions. Histopathological examination of biopsy of skin revealed diffuse infiltration of tumor cells with prominent nucleoli, scant eosinophilic cytoplasm and numerous mitotic figures consistent with LC. Immunohistochemistry was positive for CD 10, CD 19, CD 22, CD 24, CD 79-a and TdT while negative for surface immunoglobulin. At the time of presentation his peripheral blood smear and bone marrow examination was negative for malignant cells. Sanctuary sites including central nervous system and testicles were not involved. So patient was diagnosed as ALC. He was managed as per BFM relapse protocol for ALL. Skin lesions disappeared completely after 2 weeks of treatment. Unfortunately patient developed bone marrow and testicular relapse after 2 months. He was given testicular radiotherapy and systemic chemotherapy for relapsed ALL. But his marrow was showing persistent activity and he expired after 4 months.

Clinical characteristics of haematological malignancy patients diagnosed with leukaemia cutis: Experience of a single centre.

BACKGROUND/OBJECTIVES: We evaluated the clinical characteristics of patients with haematological malignancies at our centre who were diagnosed with leukaemia cutis (LC). In addition, we describe the spectrum of other skin lesions, including, secondary skin malignancies and nonspecific benign skin lesions in haematological malignancy patients. METHODS: We defined 58 skin lesions that developed in 54 inpatients hospitalised in the Department of Haematology, Trakya University Medical Faculty, Turkey. All skin lesions that developed in inpatients between 2006 and 2012 had been evaluated by a dermatologist. The patients' clinical features, skin biopsy results and therapies were obtained from hospital files. The diagnosis of LC was based on clinical features and histopathological examinations of the skin biopsy. RESULTS: There were 11 patients with LC. Six (54.5%) had acute myeloblastic leukaemia. In nine patients (82%), LC was present at the initial presentation. Secondary skin malignancy was detected in 11 patients (five basal cell carcinoma, four Kaposi's sarcoma, one squamous cell carcinoma, one malignant melanoma); and malignancy was present in two patients (18%) at the initial presentation. Nonspecific benign skin lesions, the most frequent of which were drug eruptions, were determined in 32 of our patients. LC had a significantly higher likelihood of being present at initial presentation than other skin lesions (P < 0.01). The median survival in LC patients was quite short (4.5 months). CONCLUSIONS: LC was usually diagnosed at the initial presentation of the patient or during the early course of the disease. Having LC was a poor prognostic factor.

Leucemia cutis: expressión clínica de recaída de leucemia linfocítica crônica / Leukaemia cutis: clinical manifestation of chronic lymphocytic leukaemia relapse

Presentamos un paciente varón de 71 años con lesión tumoral en piel y antecedente de LLC. Los estudios histopatológicos y de inmunohistoquímica confirman el diagnóstico de Leucemia Cutis. Se realiza tratamiento con Clorambucilo y corticoides vía oral, remitiendo su enfermedad hematológica y cutánea. Actualmente luego de ocho meses del diagnóstico de LC, el paciente se encuentra libre de enfermedad.

We present a 71 year old male patient with previous records of Chronic Lymphocytic Leukaemia who presented with a tumoral skin lesion. Histological and immunohistochemical studies confirmed the Leukaemia Cutis diagnosis. The patient underwent treatment with clorambucile and systemic steroids with remision of both haemathological and skin manifestation. The patient is still under close clinical follow up and remission continues eight months so far.