Febrile Ulceronecrotic Mucha‐Habermann’s Disease with Pulmonary Involvement

Abstract: We report a case of febrile ulceronecrotic Mucha‐Habermann disease (FUMHD) in a 12‐year‐old boy. After 1 week of typical cutaneous appearance of pityriasis lichenoides et varioliformis acuta (PLEVA), he was given a percutaneous injection of measles virus vaccine, and then extensive polymorphous, papular, and ulcerohemorrhagic skin lesions developed along with intermittent high temperature, hepatic dysfunction, and hypoalbuminemia. Thoracic CT scan showed parenchymal nodular infiltration at the tip of vascular structures. Skin biopsy showed a combination of the classic features of PLEVA and an allergic vasculitis. We treated him with prednisolone and methotrexate (MTX) successfully. The evolution in our patient suggests that FUMHD may have been triggered by virus vaccination. We also emphasize that FUMHD in children may be successfully treated with a combination of systemic MTX and high‐dose corticosteroids.     

Pentoxifylline and Cyclosporine in the Treatment of Febrile Ulceronecrotic Mucha‐Habermann Disease

Abstract: We describe two pediatric cases of febrile ulceronecrotic Mucha‐Habermann disease (FUMHD) with response to pentoxifylline and cyclosporine therapies. Based on our review of the literature, we are presenting the first case of FUMHD treated with pentoxifylline and the third case of FUMHD successfully treated with cyclosporine. These agents may be of therapeutic benefit in the treatment of FUMHD, in part by suppressing tumor necrosis factor‐alpha, which we believe may mediate the disease process.     

Febrile Ulceronecrotic Mucha–Habermann Disease: Two Cases with Excellent Response to Methotrexate

Febrile ulceronecrotic Mucha–Habermann disease (FUMHD), a severe form of pityriasis lichenoides et varioliformis acuta (PLEVA), featuring large, ulcerative, necrotic skin plaques, high fever, and other systemic symptoms, is a rare disorder of unknown etiology. No randomized controlled trials have established treatment guidelines and multiple modalities are often employed, making it difficult to assess the efficacy of any single agent. We report two cases of this condition in which treatment with methotrexate plus antibiotic treatment for superinfection led to rapid improvement.     

Subcutaneous Panniculitis‐Like T‐Cell Lymphoma with Hemophagocytic Syndrome in a Child

Subcutaneous panniculitis‐like T‐cell lymphoma (SPTCL) characterized by subcutaneous infiltration of pleomorphic T‐cells of the α/β phenotype rarely affects children. Development of hemophagocytic syndrome (HPS) leads to a poor prognosis with this otherwise indolent lymphoma. We report a case of SPTCL in a 5‐year‐old child complicated by HPS treated successfully with combination chemotherapy. We discuss the potential pitfalls in reaching an early diagnosis and challenges in its management. Previously reported cases of SPTCL with HPS in children are briefly reviewed.     

Subcutaneous Panniculitis‐Like T‐Cell Lymphoma in Children: A Review of the Literature

Subcutaneous panniculitis‐like T‐cell lymphoma (SPTCL) is a type of skin lymphoma. Pleomorphic T‐cells infiltrate the subcutaneous tissue and mimic lobular panniculitis. We report a case occurring in a 12‐year‐old Chinese boy who presented with multiple indolent erythematous subcutaneous nodules on both extremities without systemic symptoms. He had a protracted course of multiple erythematous subcutaneous nodules for 1 year and underwent biopsy of lesional skin for histology and T‐cell receptor (TCR) gene analysis. Histopathology showed infiltration of medium to large atypical pleomorphic cells involving the subcutis with characteristic rimming of fat spaces. TCR gene rearrangement shows monoclonal rearrangements of the TCR β and γ chains. Immunophenotypic studies showed that CD3, CD4, and CD8 were strongly and diffusely positive in lesional cells and that CD56 was focally positive. In contrast, these cells were negative for CD20, CD30, and CD68. The combined morphology, characteristic histologic features, and positive T‐CR gene rearrangement supported a diagnosis of SPTCL. He is being treated with combination chemotherapy of cyclophosphamide, doxorubicin, vincristine, and prednisolone.     

Basal Cell Carcinoma in a Girl after Cobalt Irradiation to the Cranium for Acute Lymphoblastic Leukemia: Case Report and Literature Review

A 12-year-old girl had a lesion located on the upper part of the scalp that was clinically interpreted as a melanocytic nevus and corresponded histologically to a basal cell carcinoma. At age 2 years she had been diagnosed as having acute lymphoblastic leukemia L1, for which she was treated with systemic chemotherapy. She also received telecobalt therapy to the whole cranium for prophylaxis of meningeal leukosis. Having rejected other possible causes that can favor the appearance of basal cell carcinoma during childhood (basal cell nevus syndrome, nevus sebaceus, albinism, etc.), we believe that this case should be added to the five previously reported in the literature as basal cell carcinoma developing at an early age in cranial radiation portals with megavoltage radiotherapy. We draw attention to the need to explore radiated areas of skin as part of the long-term follow-up of these patients, and to the advisability of obtaining a biopsy from any persistent lesion in these areas.     

Follicular Mucinosis in a Male Adolescent with a History of Acute Myelogenous Leukemia and Graft‐versus‐Host Disease

Although many cases of follicular mucinosis are idiopathic, numerous others are associated with mycosis fungoides or, rarely, other neoplastic or inflammatory disorders. There are only three reported cases, all in adults, of follicular mucinosis arising in association with acute myelogenous leukemia, two of which involved mycosis fungoides–associated follicular mucinosis, including one case in which the patient had a preceding bone marrow transplant. We present the first reported case of follicular mucinosis arising in an adolescent with acute myelogenous leukemia and acute graft‐versus‐host disease after an allogeneic bone marrow transplantation.     

Eosinophilic Pustular Folliculitis in Children after Stem Cell Transplantation: An Eruption Distinct from Graft‐Versus‐Host Disease

Eosinophilic pustular folliculitis (EPF) is a rare cutaneous disorder that typically occurs in three clinical contexts: men, individuals who are immunosuppressed or have human immunodeficiency virus, and infants. A fourth subtype occurring 2 to 3 months after hematopoietic stem cell transplantation (HSCT) has recently been described in several adults. We report two cases of EPF arising in children after HSCT. It is important to recognize this form of EPF after HSCT and differentiate it from graft‐versus‐host disease since it responds readily to topical steroids and appears to have an excellent prognosis.     

Proteomic analysis of stratum corneum in Cutaneous T‐Cell Lymphomas and psoriasis

Stratum corneum collected by tape stripping from 10 and 24 subjects with cutaneous T‐cell lymphomas (CTCL) or psoriasis, respectively, were compared using quantitative label‐free mass spectrometry analysis. A non‐supervised statistical analysis (Posneg NMF) based on 352 differentially expressed proteins in both CTCL and psoriasis samples was able to separate the two disease groups and finally able to identify a set of 112 proteins that contributed most and significantly to the separation when compared to non‐lesional samples. In addition, Luminex assay revealed that the increase in the amount of chemokines related to the inflammatory response, and immune cell infiltration and recruitment in lesional stratum corneum in CTCL, including CXCL8, CXCL9, CXCL10, CCL27, TNF and sICAM‐1 was in agreement with published data on entire skin biopsies. Proteome analysis using quantitative methods including mass spectrometry and Luminex technology offered the possibility to investigate the relevant protein signature in CTCL and may be helpful to diagnose and investigate the efficacy of treatments in clinical investigations using non‐invasive methods in future.     

Ecthyma Gangrenosum–Like Lesions Secondary to Methicillin‐Sensitive Staphylococcus aureus in an Immunocompetent Child

We describe a previously healthy 15‐month‐old girl who developed ecthyma gangrenosum (EG)–like lesions secondary to methicillin‐sensitive Staphylococcus aureus (MSSA). Her systemic symptoms and negative blood cultures suggested MSSA toxin‐mediated effects. When toxin‐mediated systemic symptoms accompany such lesions, pathogens other than Pseudomonas aeruginosa should be considered.     

Primary Cutaneous CD4+ Small to Medium‐Size Pleomorphic T‐Cell Lymphoma in a 12‐Year‐Old Girl

Primary cutaneous CD4+ small to medium‐size pleomorphic T‐cell lymphoma (PCSM‐TCL) is a rare disease that has been added as a provisional entity to the World Health Organization European Organization for Research and Treatment of Cancer (WHO‐EORTC) classification of lymphomas with primary cutaneous manifestations. Patients commonly present with a solitary nodule or plaque on the head or upper trunk, but are usually otherwise in good health. The prognosis is favorable, but the optimal treatment has not been defined. Recent publications have described the expression of programmed death‐1 in PCSM‐TCL and T‐cell pseudolymphoma, suggesting a diagnostic value of this marker in the differential diagnosis of PCSM‐TCL in contrast to other types of cutaneous T‐cell lymphoma. We present the case of a 12‐year‐old girl with a tumor of the right supraorbital area. She was treated as an outpatient four times with intralesional triamcinolone acetonide at intervals of 3 to 4 weeks. In addition to the case history, this report includes the clinical and histologic findings and a review of the current literature.     

Human T‐lymphotropic virus type I proviral loads in patients with adult T‐cell leukemia–lymphoma: Comparison between cutaneous type and other subtypes

Adult T‐cell leukemia–lymphoma (ATL), characterized by various clinicopathological features, is divided into four clinical subtypes, namely, acute, lymphoma, chronic and smoldering types, and the treatment strategy differs according to the clinical subtype. The designation cutaneous type ATL has been proposed to describe a peculiar subgroup of smoldering type ATL in which the skin is predominantly affected. However, diagnostic criteria and prognostic factors for cutaneous type ATL remain to be determined. Therefore, we performed a retrospective study to obtain a precise method for subtype classification and to clearly define cutaneous type ATL. A total of 87 ATL patients (acute, n = 31; lymphoma, n = 6; chronic, n = 24; smoldering, n = 26) were enrolled. The human T‐lymphotropic virus type I (HTLV‐1) proviral load in peripheral blood and the serum soluble interleukin‐2 receptor (sIL‐2R) level were evaluated with respect to the clinical features of the different types of ATL. The HTLV‐1 proviral load was significantly increased in the acute and chronic type and the serum sIL‐2R level was increased in the acute and lymphoma type. The HTLV‐1 proviral load was significantly lower in cutaneous than other smoldering types of ATL without skin lesions. The clinical findings of cutaneous type ATL were also different from other subtypes. These results indicate that, in combination, determination of the HTLV‐1 proviral load and the serum sIL‐2R level is useful for distinguishing among the different types of ATL, and strongly suggest that cutaneous type ATL is a distinct clinical entity.     

Epstein–Barr virus‐associated T‐cell lymphoproliferative disorder affecting skin and lung in an elderly patient

A 70‐year‐old man presented with papular skin lesions and was diagnosed with Epstein–Barr virus (EBV)‐associated T‐cell lymphoproliferative disorder (T‐LPD). The patient showed infiltration of a large number of EBV‐encoded RNA‐positive T cells in the skin and lung, presence of EBV load in the peripheral blood, and expansion of clonal EBV‐infected γδ T cells and CD8⁺ T cells in the blood and skin, as assessed by EBV‐terminal repeat Southern blot, T‐cell receptor polymerase chain reaction and flow cytometric analyses. In the Japanese or East Asian fatal cases of EBV‐associated T/natural killer (NK)‐LPD, there are two peaks in age at death, approximately 20 years and 60 years. The former age group is associated with chronic active EBV infection (CAEBV), and the latter group typically suffers from extranodal NK/T‐cell lymphoma. Our case is characterized not only by the unique skin and lung manifestations but also the late onset age of the disease, indicating that the skin manifestation of CAEBV can be seen even in elderly patients.     

Primary Cutaneous B‐Cell Lymphoblastic Lymphoma Arising from a Long‐Standing Lesion in a Child and Review of the Literature

Precursor B‐cell lymphoblastic lymphoma (B‐LBL) is a rare entity and primary cutaneous B‐LBL is an even more uncommon diagnosis that typically affects children. A 4‐year‐old boy presented with a persistent rash on his left cheek for almost 2 years and was found to have primary cutaneous B‐LBL. We report this case to emphasize that B‐LBL should be in the differential diagnosis for an otherwise unimpressive persistent lesion in the head and neck region and review all reported pediatric cases of primary cutaneous B‐LBL without extracutaneous involvement.