Crusted (Norwegian) scabies with adult T-cell leukemia

Crusted (Norwegian) scabies occurred in a patient with adult T-cell leukemia. It was not noted at the time of hospital admission. Scabies should be included among the opportunistic pathogens in immunocompromised hosts.     

Langerhans cell hyperplasia in scabies: a mimic of Langerhans cell histiocytosis

AIM: In the absence of mites, the histologic diagnosis of human scabies can be difficult. Scabies can mimic a variety of inflammatory and lymphoproliferative disorders. It is under-recognized that scabies can also mimic Langerhans cell histiocytosis. METHODS: Sixteen examples of scabies were reviewed histologically and immunohistochemically (CD1a, CD3, CD20, CD30 and S100). RESULTS: Immunohistochemical labeling showed florid CD1a and S100 positivity in most cases, indicative of Langerhans cell hyperplasia. Scattered CD30+ lymphocytes were also typically present, within a dense infiltrate, primarily composed of T lymphocytes and eosinophils. CONCLUSION: Because of the prominent CD1a+/S100+ component, scabies can mimic Langerhans cell histiocytosis. This finding should be considered in conjunction with scattered CD30+ cells and clinical features to avoid misdiagnosis.     

Crusted (Norwegian) scabies in a patient with smoldering adult T-cell leukemia

Crusted (Norwegian) scabies is described in a patient with smoldering adult T-cell leukemia (ATL). The patient is an 84-year-old Japanese woman who presented with widespread scaling over the trunk and limbs and crusted lesions on the scalp and ears. Microscopical examination of scrapings from the scalp and ears showed extraordinarily large numbers of scabies mites. The white blood cell count was 5.1 x 10(9)/L with 6% abnormal lymphocytes with mature nuclei that showed convolution and lobulation. Anti-HTLV-I antibodies were positive. Southern blot analysis revealed that the cellular DNA extracted from this patient's peripheral blood cells, digested with Pst I, showed the same pattern of provirus genome as the DNA from ATL patients. A diagnosis of crusted scabies with smoldering ATL was made. It is possible that crusted scabies could be an opportunistic infection and a pre-diagnostic sign of ATL.     

Multisystem Langerhans cell histiocytosis in adult

Langerhans cell histiocytosis (LCH), is a rare disorder, clinically presents with heterogeneous manifestations, and has an unpredictable outcome. Commonly seen in infancy or early childhood, the disorder is characterized by proliferation of abnormal and clonal Langerhans cell in skin, bone, lymph nodes, lungs, liver, spleen, and bone marrow. Occurrence of LCH in adults is rare. Here, we report the case of an adult with acute onset of polymorphic eruptions all over the body, which on biopsy showed features of multisystem LCH, and was confirmed by immunohistochemistry. Although multisystem LCH has a poor prognosis, our patient responded well to chemotherapy.     

Acute disseminated histiocytosis X (Langerhans cell histiocytosis) with fatal course in an adult

Histiocytosis X is very rare in late adulthood, usually taking a benign course with a good prognosis. A 56-year-old patient developed acute disseminated histiocytosis X with multiorgan involvement. Despite extensive diagnostic work-up the diagnosis could only be established on the grounds of a skin biopsy. Histiocytosis X cells are S 100 protein-positive and express the membrane antigens CD 1, CD 4 and HLA-DR. Electron microscopy reveals Birbeck granules. On static cytophotometry, histiocytosis X cells exhibited normal DNS content without aneuploidy. One year after the onset of disease the patient's condition deteriorated rapidly despite aggressive polychemotherapy (CHOP), and he died of multiorgan failure due to histiocytosis X.     

Langerhans cell histiocytosis in an adult: good response of cutaneous lesions to acitretin

A 57‐year‐old man presented with a 2‐year history of bilateral erosive lesions on the inguinal region, and erythematous, brown and crusted papules over the trunk. Histological examination of one lesion in conjunction with immunohistochemical study and electron microscopy led to the diagnosis of Langerhans’ cell histiocytosis. After a thorough examination, the only associated findings were retroperitoneal fibrosis and hypergonadotrophic hypogonadism with a granulomatous testicular infiltrate. The patient was treated with oral acitretin for 1 year (with a topical corticosteroid for the inguinal lesions), resulting in clearing of the cutaneous lesions. He underwent placement of bilateral double‐J ureteral catheters and was started on hormone replacement therapy. At follow‐up 1 year after treatment with acitretin ceased, the patient remained free of cutaneous lesions and his overall condition, including the retroperitoneal fibrosis, had improved. This case had an uncommon combination of features, with a good response to acitretin.     

Crusted (Norwegian) scabies in a patient with dystrophic epidermolysis bullosa

A 13-year-old girl with severe non-mutilating recessive dystrophic epidermolysis bullosa (EB) was admitted to hospital because of a Staphyloccus aureussepsos, deterioration of her general condition and worsening of her skin disease, which itched severely. In addition to the blisters and erosions normally seen, she was covered from head to toe with scales and hyperkeratotic crusts. Despite intensive topical therapy, her skin condition did not improve significantly until scabies was detected and treated 1 week after admission. Because of the huge number of mites found and the crusted appearance, a diagnosis of crusted (Norwegian) scabies was made. She was successfully treated with two doses of ivermectin orally and one application of lindane ointment. Permethrin cream was not tolerated. In this patient crusted scabies may have developed because of: (i) a modified host response due to malnourishment; (ii) inability to scratch because of the absence of fingernails; and (iii) abnormal scratching behaviour because of the vulnerability of EB skin, or a combination of these factors. Limited isolation measures were taken on admission and full measures were taken immediately after the diagnosis of crusted scabies was made. Prophylactic treatment of ward personnel was not undertaken. Fortunately, there was not an outbreak of scabies in the hospital.     

Crusted scabies in an immunocompetent child: treatment with ivermectin

An 11-year-old girl presented to our clinic with recalcitrant crusted scabies despite repeated applications of topical scabicides. She had no history of corticosteroid use prior to onset of the eruption and no evidence of immunodeficiency. A combination of oral ivermectin, topical lindane, and keratolytics cleared the infestation. Our patient is exceptional in that she had no risk factors commonly associated with a propensity to develop crusted scabies. While topical therapy remains the first-line treatment for children with classic scabies, in the unusual instance of a child with recalcitrant, crusted scabies, ivermectin may offer an efficacious alternative, although it should be used with caution. We discuss the use of oral ivermectin for treatment of crusted scabies and the challenging comprehensive management needed for this socially stigmatizing condition.     

Treatment of adult Langerhans cell histiocytosis with etoposide.

BACKGROUND: Langerhans cell histiocytosis (LCH) is a reactive disease in which abnormal Langerhans cells accumulate in various body sites and cause damage to affected organs. Adults often do not respond to conventional therapy with local or systemic steroids. OBJECTIVE: Our purpose was to investigate intravenous etoposide as monotherapy in the treatment of adult patients with severe or resistant LCH. METHODS: In an open study, three adult patients with LCH (one with single-system skin disease and two with multisystem disease) were treated with etoposide, 100 mg/m2/day, for 3 days. This was repeated every 3 or 4 weeks for three or four cycles. RESULTS: All patients achieved clinical remission that persisted during a 12- to 14-month follow-up. No serious side effects were noted. CONCLUSION: We recommend the use of etoposide monotherapy in severe or restricted LCH in adults.     

Crusted scabies in an adult T-cell leukemia/lymphoma patient successfully treated with oral ivermectin

We report an adult T-cell leukemia/lymphoma (ATL) patient whose crusted scabies was successfully treated with oral ivermectin. This 63-year-old man had previously been treated with oral prednisolone, sobuzoxane and etoposide for approximately 1 year. When he developed crusted scabies, he received two doses of oral ivermectin (200 microg/kg) 10 days apart and the concomitant topical application of crotamiton containing 30% benzyl benzoate. This produced remarkable results, suggesting that oral ivermectin should be considered for the treatment of crusted scabies even in immunocompromised patients. While ivermectin may be useful for treating intractable scabies, attention must be paid to the possible appearance of ivermectin-resistant mites.     

Widespread skin-limited adult Langerhans cell histiocytosis: long-term follow-up with good response to interferon alpha

Langerhans cell histiocytosis (LCH) most frequently involves bone, but also involves the skin in 40% of cases; in 10% of patients it is limited to the skin. Cutaneous findings of skin-limited LCH are scaly papules, vesicles, nodules, tumours with erosion, ulceration, or crusting and/or purpura. We report a case of widespread adult-onset LCH confined to skin in which topical carmustine, photochemotherapy, systemic steroids, and 2-chlorodeoxyadenosine were only partially effective. However, longer remission was achieved by the use of subcutaneous interferon-alpha2b.     

单纯皮肤受累的成人朗格汉斯细胞组织细胞增生症一例

患者男,60岁, 躯干泛发褐色大斑片3年,斑片上及其周边出现结节半年合并溃疡2个月。患者3年前腹部皮肤出现红褐色斑片约1.0 cm × 2.0 cm,无明显自觉症状。当时未予任何处理,期间皮疹面积逐渐增大,并泛发到背部、臀部、侧腰部及腹股沟上方,颜色逐渐变深呈褐色,予口服及外用药治疗（具体不详）,皮损未见好转。半年前背部斑块上成簇出现粉红色黄豆大小结节,结节逐渐增大并变硬,数量增多,并很快发展到其他斑块及周围皮肤上,期间伴随着新结节的出现及老结节的消退……