De novo myeloid sarcoma in a 4‐month‐old infant: a case report and review of the literature

Myeloid sarcoma is a rare tumor of immature myeloid cells in an extramedullary site. Myeloid sarcoma may present in a variety of locations; skin is one of the common sites. It may precede or occur concurrently with acute myeloid leukemia, chronic myeloid leukemia, other forms of myeloproliferative disorders/myelodysplastic syndrome or de novo. We report a case of a 4‐month‐old female who presented with cutaneous lesions without evidence of leukemia, determined to be de novo myeloid sarcoma. She had erythematous nodules in multiple skin sites. Biopsy revealed a diffuse atypical mononuclear cell infiltrate involving the entire dermis and extending to the subcutis. The infiltrate was diffusely positive for lysozyme, CD43, CD15, CD33, CD68 and CD117 and was negative for CD3, CD20, CD34, CD56, CD79a, CD99, myeloperoxidase, desmin, chromogranin and synaptophysin, supporting a diagnosis of myeloid sarcoma. No leukemic involvement was found on evaluation of peripheral blood or bone marrow aspiration. Chromosomal abnormalities were found at chromosomes 7, 10 and 11. The skin lesions resolved following multiple chemotherapy courses, then recurred requiring additional treatment. De novo myeloid sarcoma involving skin without evidence of leukemia can occur in an infant and may present a diagnostic challenge.     

Myeloid sarcoma in a newborn: A rare manifestation of congenital acute myeloid leukemia

Cutaneous myeloid sarcoma is rarely present prior to the diagnosis of congenital acute myeloid leukemia (AML); the former is typically diagnosed with or after the leukemia. We report a 2-day-old male born with multiple cutaneous red to violaceous nodules. Histopathologic and immunohistochemistry findings from a skin nodule were suspicious for myeloid sarcoma. Bone marrow biopsy was initially negative for aberrant blasts; however, at age 4 months, AML with a KMT2A gene rearrangement was identified via bone marrow biopsy.     

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.     

Disseminated Juvenile Xanthogranuloma

Juvenile xanthogranuloma (JXG) is the most common type of non‐Langerhans cell histiocytosis occurring predominantly in infants and children. Typical lesions are asymptomatic red‐yellow papules and nodules on the scalp or in the axillae or groins. Multiple lesions are more common in children than in adults. A 2‐year old girl presented with approximately 20 red‐brown lesions, leading to the diagnosis of disseminated JXG. Internal involvement was excluded. Spontaneous regression occurred over 6 months of follow‐up. A wait‐and‐see strategy is recommended for cutaneous JXG.     

Panniculitis in a 3‐year‐old child with Fanconi anemia‐associated bone marrow hypoplasia heralds transformation to acute myeloid leukemia

Fanconi anemia is a rare, autosomal recessive genomic instability disorder characterized by congenital limb anomalies, panmyelopathy and a high risk of malignancy, principally acute myeloid leukemia. Hematologic malignancy presenting with acute febrile neutrophilic dermatosis (Sweet syndrome), both deep and superficial forms, is well described in Fanconi anemia patients but is a rare phenomenon in otherwise healthy children. We present a case of panniculitis (presumptive subcutaneous Sweet syndrome) heralding transformation to acute myeloid leukemia in a 3‐year‐old boy with a severe Fanconi anemia phenotype.     

Ponatinib‐induced neutrophilic panniculitis

Ponatinib is a bcr‐abl tyrosine‐kinase inhibitor (TKI) used to treat resistant and refractory chronic myeloid leukemia and Philadelphia chromosome‐positive acute lymphoblastic leukemia that express bcr‐abl. Neutrophilic panniculitis has been described in rare cases of patients on other TKIs in the same class as ponatinib. We present the first case of neutrophilic panniculitis following treatment with ponatinib and summarize the other cases of panniculitis caused by TKIs.     

Cutaneous macroglobulinosis deposits in a patient with IgM paraproteinemia/incipient Waldenström macroglobulinemia

A 43‐year‐old healthy patient developed disseminated flat skin‐colored to red‐brown papules over a few months. These papules were the result of cutaneous IgM deposits representing the first symptom of a hitherto undiagnosed IgM paraproteinemia. This extremely rare skin manifestation of IgM paraproteinemia e. g. possibly incipient Waldenström macroglobulinemia should be included in the histopathological differential of eosinophilic dermal deposits.     

Permanent makeup removal using Q‐switched Nd:YAG laser

Red‐brown tattoos are usually treated with a frequency‐doubled Q‐switched (QS) neodymium : yttrium–aluminum–garnet Nd:YAG laser (532 nm), because red and pink pigments show maximum absorption between 500 and 570 nm. Using a QS laser for red‐brown tattoo removal has sometimes led to paradoxical darkening of the tattoo pigments, and this darkened grey‐black colour may be difficult to remove. A woman with red‐brown cosmetic tattoos on her eyebrows was treated using a QS Nd:YAG laser (1064 nm) initially with low fluence and subsequently with increasing fluences at 6‐weekly intervals. After the first treatment, a slight darkening of the tattoo pigments was seen, but this faded and complete clearance was achieved after five treatments. There was no downtime during every treatment and there were no scars, pigmentary alterations or textural changes.     

Annular lichenoid dermatitis of youth – a further case in a 12‐year‐old girl

Annular lichenoid dermatitis of youth was first described by Annessi et al. in 2003. Clinical criteria are persistent erythematous macules and annular lesions with a red‐brown edge and a central hypopigmentation usually found on the flanks and groins of children and adolescents. Histologically, the disease is characterized by a lichenoid interface dermatitis with necrotic keratinocytes at the tip of the rete ridges. In our case a 12‐year old girl developed annular red‐brown macules with papules at the borders in an inframammary location. The histology of the lesion's border showed a lichenoid lymphocytic infiltrate with apoptotic keratinocytes at the tip of rete ridges. The lesions cleared with 0.03% tacrolimus ointment. Annular lichenoid dermatitis of youth is probably a new entity in the group of lichenoid dermatoses.     

A Case of Congenital Leukemia Cutis

Congenital leukemia is a rare disease that develops from birth to 6 weeks of life. Leukemia cutis involves cutaneous infiltration by leukemic cells and is an unusual manifestation of leukemia, and has been documented in 25~30% of patients with congenital leukemia. The authors report a case of congenital leukemia cutis. A newborn male presented with widespread firm dusky red papules and nodules on almost his entire body surface. Skin biopsy specimens confirmed the presence of leukemic infiltrations, and bone marrow cytology was consistent with acute myeloid leukemia of the FAB M5 type.     

The atopic skin‐like microenvironment modulates the T‐cell‐polarising cytokine production of myeloid dendritic cells,as determined by laser scanning cytometry

Because it is not known exactly when or where myeloid dendritic cells (mDCs) acquire their atopic dermatitis (AD)‐specific T‐cell‐polarising ability in patients with this condition, we used laser scanning cytometry (LSC) to determine whether isolated peripheral blood mDCs from AD patients differed from cells from controls in their cytokine expression profiles de novo and after stimulation with Staphylococcus enterotoxin B (SEB) and thymic stromal lymphopoietin (TSLP), which represents an AD‐like microenvironment. Unstimulated mDCs from AD patients showed pluripotent T‐cell‐polarising capacity, and the surrounding skin microenvironment was essential for the distinctive, disease‐specific activity of mDCs (Th2‐Th22 bias). We also emphasise that LSC is an attractive technique to study the effect of new DC‐targeted therapeutic modalities in AD.     

Poxvirus‐induced angiogenesis after a thermal burn

Orf (contagious ecthyma) is a zoonotic infection caused by a dermatotropic parapoxvirus that commonly infects sheep, goats, and oxen. Parapoxviruses are transmitted to humans through contact with an infected animal or fomites. Orf virus infections can induce ulceration, and papulonodular, pustular, or ecthymic lesions of the skin after contact with an infected animal or contaminated fomite. Rarely, orf virus provokes extensive vasculo‐endothelial proliferation as a skin manifestation. Here, we present the case of an 8‐year old female with poxvirus‐induced vascular angiogenesis that developed 10 days after a thermal burn. An 8‐year‐old female presented at our outpatient clinic with red swellings and a yellow‐brown crust on them. After a thermal burn with hot water, she went to a clinic and the burn was dressed with nitrofurazone and covered for 2 days. When the dressing was removed after 2 days, nodules were seen in the burnt areas. When the clinical findings were considered with the histopathological features, a reactive vascular proliferation due to a viral agent was suspected. Following PCR, parapoxvirus ovis was detected. Viral infections such as pox virus can trigger pyogenic granulomas or pyogenic granuloma‐like vascular angiogenesis. Infectious agents must be considered when dealing with pyogenic granuloma‐like lesions.     

Tumor‐educated myeloid cells: impact the micro‐ and macroenvironment

Immune escape mechanisms of cancers include some of the mechanisms normally used for immune homeostasis, particular those preventing autoimmunity; one of these is the polarisation of myeloid cells. Thereby, tumors, i.e. the cancerous and stromal cells, also condition distant sites like spleen and bone marrow via soluble factors and membrane vesicles such as exosomes in order to create a tumor‐educated macroenvironment. Albeit these mechanisms are currently in the focus of (tumor‐)immunologic research, the first evidence had been published almost 40 years ago. One of these early reports will be discussed here.     

Angiolymphoid hyperplasia with eosinophilia affecting the scrotum: A rare case report with molecular evidence of T‐cell clonality

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare, benign entity of unknown pathogenesis. It often presents as painful or pruritic intradermal or subcutaneous red to brown papules or nodules on the head and neck of young adults. A 38‐year‐old man had a gradually enlarging and mild pruritic plaque on the scrotum for half a year. Pathological findings showed dermal proliferation of anomalous blood vessels lined by plump endothelium with a significant perivascular inflammatory infiltrate composed of lymphocytes, histiocytes, scattered plasma cells and many eosinophils. They were consistent with the diagnosis of ALHE. In addition, the inflammatory infiltrate was analyzed by immunohistochemistry and T‐cell receptor (TCR) gene rearrangement. They were mostly CD3⁺ T cells and a monoclonal T‐cell population. To the best of our knowledge, this is the first case of ALHE affecting the scrotum to be reported in the published work. We present this case to expand the anatomical distribution of this rare tumor. The molecular study of our case supports that ALHE might be a low‐grade T‐cell lymphoproliferative disorder.     

CD8‐positive pseudolymphoma in lues maligna and human immunodeficiency virus with monoclonal T‐cell receptor‐beta rearrangement

A 42‐year‐old Caucasian man suffered from disseminated plaques and ulcerated nodules for 6 weeks. He had weight loss and generalized lymphadenopathy. Underlying diseases were not known up till then. Based on a skin biopsy, the diagnosis of CD8‐positive cutaneous T‐cell lymphoma, type mycosis fungoides was made in a pathological reference center for lymphoma. A reproducible T‐cell receptor (TCR)‐beta rearrangement was detectable. Before starting therapy, a new biopsy was taken and the previous diagnosis was re‐evaluated taking clinical images and symptoms into account. Based on both, the diagnosis of a CD8+ pseudolymphoma in lues maligna and human immunodeficiency virus was made. We highlight histopathologic clues for the correct diagnosis, and we emphasize the indispensability of clinical‐pathological correlation. Furthermore, we discuss the differential diagnosis of CD8+ lymphoproliferative disorders.     

Nasal‐type extranodal natural killer/T‐cell lymphoma presenting with extensive leg ulcers

Primary cutaneous T‐cell lymphomas are rare and can be difficult to classify precisely. We present a case of extranodal natural killer (NK)/T‐cell lymphoma in a previously healthy, immunocompetent man who presented with extensive necrotic leg ulcers and disseminated skin nodules. Immunohistochemical studies revealed that the tumour cells were positive for CD3, CD30, granzyme B and T‐cell intracellular antigen‐1, and negative for CD5 and CD56, with positive staining for Epstein–Barr virus (EBV) RNA on in situ hybridization. A diagnosis of extranodal NK/T‐cell lymphoma was made, based on the presence of cytotoxic granules and positive EBV RNA staining. The patient was treated with a regimen of chemotherapy comprising corticosteroids, intravenous methotrexate, ifosphamide, L‐asparginase and etoposide with initial response.     

Disseminated cutaneous leiomyomas

Cutaneous leiomyomas are benign smooth muscle tumors. Depending on the site of origin, one distinguishes three different types--piloleiomyoma, angioleiomyoma and genital leiomyoma. They appear between the first and third decades of life. A 56-year-old woman presented with painful red - brown papules and nodules on her trunk. Based on clinical and histological criteria the diagnosis of disseminated cutaneous leiomyomas was made. Because of the widespread cutaneous involvement, surgical treatment was not possible. Therefore we decided to employ a pharmacologic anti-depressive treatment. With this approach, the patient experienced considerable pain reduction.     

Side‐effects of henna and semi‐permanent ‘black henna’ tattoos: a full review

Henna, the dried and powdered leaf of Lawsonia inermis, is widely used as a dye for the skin, hair, and nails, and as an expression of body art, especially in Islamic and Hindu cultures. As it stains the skin reddish‐brown, it is also called red henna. Black henna is the combination of red henna with p‐phenylenediamine (PPD), and is used for temporary ‘black henna tattoos’. This article provides a full review of the side‐effects of topical application of red and black henna, both cutaneous (allergic and non‐allergic) and systemic. Red henna appears to be generally safe, with rare instances of contact allergy and type I hypersensitivity reactions. In children with glucose‐6‐phosphate dehydrogenase deficiency, topical application of henna may cause life‐threatening haemolysis. Black henna tattoos will induce contact allergy to its ingredient PPD at an estimated frequency of 2.5%. Once sensitized, the patients may experience allergic contact dermatitis from the use of hair dyes containing PPD. There are often cross‐reactions to other hair dyes, dyes used in textiles, local anaesthetics, and rubber chemicals. The sensitization of children to PPD may have important consequences for health and later career prospects. Systemic toxicity of black henna has been reported in certain African countries.     

Nodular lesions arising in a large congenital melanocytic naevus in a newborn with eruptive disseminated Spitz naevi

Congenital malignant melanoma within a pre‐existing large congenital melanocytic naevus (CMN) is exceedingly rare. Its incidence is difficult to determine due to the small number of reported cases and because of problems associated with diagnosis. Some benign nodular proliferations (called proliferative nodules) arising in CMN, while rare, are significantly more common and can mimic malignant melanoma clinically or histologically. There are no reported cases of congenital melanoma or benign proliferative nodules in CMN in patients who also had eruptive disseminated Spitz naevi. We describe a girl who was noted to have a dark‐brown plaque with several large erythematous nodules affecting the scalp at delivery, in addition to multiple erythematous dome‐shaped papules that developed in a disseminated manner over several months, beginning at 10 days of age. It was difficult, not only clinically but also histologically, to determine the benign or malignant nature of all of these lesions. As primary cutaneous melanoma, atypical proliferative nodules in CMN, bland CMN or CMN with foci of increased cellularity and Spitz naevi show clear differences in the genetic aberration patterns, comparative genomic hybridization (CGH) could be a diagnostic help in ambiguous cases such as this. CGH performed on this patient showed multiple DNA copy number changes in the most atypical nodule, but such alterations could not be found in the remainder of the lesions. CGH showed differences between the nodular lesions that occurred in the CMN and helped us in supporting the diagnosis of this unique case of benign proliferative nodules and a possible congenital melanoma arising in a large CMN, associated with multiple widespread eruptive Spitz naevi.