A case of multiple nodular cutaneous B-cell pseudolymphoma successfully treated with glucocorticoid, methotrexate, and hydroxychloroquine.

Cutaneous pseudolymphomas (CPL) is a group of benign, reactive, and polyclonal lymphoproliferative dermatoses that simulate cutaneous lymphomas (CL) clinically and histologically. Based on the predominating component of lymphocytic infiltrate, CPL can be divided into cutaneous B-cell pseudolymphomas (CBPL), cutaneous T-cell pseudolymphomas (CTPL), mixed (T-/B-cell) pseudolymphomas, CD30-positive pseudolymphomas, and non-classifiable pseudolymphomas. Most patients with localized nodular CBPL present with a solitary nodule. However, few patients develop multiple skin lesions, rarely in generalized forms. Here we describe a rare case of multiple nodular CBPL on both sides of the patient's neck, which was treated successfully with intramuscular injection of compound betamethasone, oral methotrexate, and hydroxychloroquine for 4 months. No recurrence was observed in the patient at the one-year follow-up. This combined treatment may be a promising treatment choice for multiple nodular CBPL.

Cutaneous B cell pseudolymphoma treated with rituximab and methotrexate.

Cutaneous B cell pseudolymphoma (CBPL), or cutaneous lymphoid hyperplasia, is the most common pseudolymphoma. It typically responds well to local treatment and follows a benign course. Herein, we describe the unique case of a patient with CBPL that was refractory to a variety of treatments, with subsequent response to rituximab followed by methotrexate. This case explores the complex interplay of T and B lymphocytes, and the potential role of perifollicular T cells in treatment resistant CBPL. Further, it describes the additive therapeutic effect of rituximab and methotrexate to target both B cell and T cell populations in CBPL, a strategy already employed in a number of other conditions.

Ipilimumab/nivolumab-induced pseudolymphoma in a patient with malignant melanoma.

Combination therapy with ipilimumab and nivolumab is an adjuvant treatment approach for metastatic melanoma that boasts increased 3-year survival when compared with a single immunotherapy agent. Combination therapy, however, is associated with increased toxicities, especially cutaneous side-effects. Here we present a patient with metastatic melanoma and a sudden eruption of painful nodules on the face and arms 10 days after the administration of the fourth dose of combination ipilimumab/nivolumab. Biopsies demonstrated lymphoid hyperplasia, not clinically or pathologically consistent with an infectious, malignant or autoimmune etiology; a diagnosis of pseudolymphoma secondary to ipilimumab/nivolumab was made. After a steroid taper, the lesions resolved, and the patient was restarted on nivolumab monotherapy 2 weeks later without recurrence of symptoms or disease.

A case of cutaneous pseudolymphoma with a distinctive appearance treated successfully by intralesional interferon alpha-1b and corticosteroids.

Cutaneous pseudolymphoma (CPL) encompasses various forms of benign lymphocytic proliferative dermatoses that mimic the clinical and/or pathological changes of lymphoma. The clinical manifestations of CPL vary due to differences in the pathogenesis, and accordingly, no specific treatment has been identified. Here, we report a case of CPL on the nose, which had a distinctive appearance and was treated successfully using a combination of intralesional interferon alpha-1b and compound betamethasone (betamethasone sodium phosphate and betamethasone dipropionate). This combination may be a good option for localized CPLs at particular anatomical sites.

Use of topical rapamycin in acral pseudolymphomatous angiokeratoma of children (APACHE): A report of two cases and review of the literature.

Acral pseudolymphomatous angiokeratoma of children (APACHE) is a rare, benign disease characterized clinically by multiple, asymptomatic, erythematous papules in the acral regions. We report APACHE in a 12-year-old girl with erythematous-violaceous papules on the lateral dorsum of her foot and toes, and a 3-year-old girl with erythematous papules on the plantar aspect of her foot. Topical rapamycin ointment improved the lesions and both patients tolerated the medication well. Topical rapamycin appears to be a potentially efficacious, well-tolerated, non-invasive therapy in APACHE, although further studies are needed.

IgG4-rich reactive lymphoid hyperplasia of the lacrimal gland.

We describe an otherwise healthy 24-year-old woman with reactive lymphoid hyperplasia of one lacrimal gland. She was found to have modest elevation of serum IgG4 and high density of IgG4+ plasma cells in her lacrimal gland, ranging from 40% to 60% of IgG+ plasma cells. The patient transiently responded to systemic corticosteroids before treatment with rituximab. IgG4-rich reactive lymphoid hyperplasia of the lacrimal gland is neither an established part of IgG4-related disease spectrum nor a known precursor lesion to lymphoma. Although there is a plausible risk of transforming to IgG4-related disease or to lymphoma, the biological potential and natural history of IgG4-rich reactive lymphoid hyperplasia remains to be determined.

Lymphoma or pseudolymphoma: A report of six cases and review of the literature.

To explore and compare the clinical presentations and pathologic features of cutaneous pseudolymphomas (CPL) with primary cutaneous lymphomas. Review literature in order to improve the treatment of CPL. Six cases of CPLs were collected. The clinical, pathologic, and immunohistochemical features were performed and analyzed in Peking Union Medical College Hospital in 2018. Of six patients, the distributions and clinical manifestations of skin lesions are varied. The pathologic features consisted of atypical prominent lymphocytes infiltration. Of them, two cases imitated mycosis fungoides, one case mimicked primary cutaneous aggressive pidermotropic CD8+ cytotoxic T-cell lymphoma, one case was diagnosed as Jessner-Kanof lymphocyte infiltration and two cases primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder. With respect to etiology, one was result from insects bite and the others were on account of drugs. All cases were treated with systemic or local glucocorticoid. The skin lesions and systemic symptoms showed notable improvement after treatment. Follow-up visits were 2 years, half a year, and months, respectively, without relapse. These unique types of CPL were similar to cutaneous lymphomas in clinical manifestation and pathology. They were all sensitive to the treatment of externally or orally using glucocorticoid. The prognosis is generally good but needs long-term follow-up.

Areolar lymphocytoma in a child: A rare cutaneous presentation of borreliosis.

Lyme disease is a common tick-borne infection caused by Borrelia burgdorferi in the United States, where infection is most prevalent in the northeastern and mid-Atlantic states. Although classically associated with erythema migrans, Lyme disease caused by Borrelia species found in Europe may also present with other cutaneous findings. Here we report the case of a girl who was clinically diagnosed with Lyme disease based on her history of recent travel and the appearance of an areolar lymphocytoma; this was confirmed by testing. Testing for European Lyme disease does not follow the testing algorithm that the Centers for Disease Control and Prevention recommends and may be easily missed. Our case serves as an important reminder that common infections can have varying presentations depending on their region of acquisition and may require specialized testing for accurate diagnosis.

Borrelial Lymphocytoma in Adult Patients.

BACKGROUND: Information on the course and outcome of borrelial lymphocytoma (BL) is limited. METHODS: The study comprised 144 adult patients (75 female, 69 male; median age, 49 years) who had BL diagnosed at a single center between 1986 and 2014 and were followed up for 1 year. RESULTS: BL was located on the breast in 106 patients (73.6%), on the ear lobe in 27 (18.8%), and elsewhere in 11 (7.6%). The median duration of BL before diagnosis was 27 days (interquartile range [IQR], 9-68 days). Concomitant erythema migrans was registered in 104 of 144 patients (72.2%); other objective manifestations of Lyme borreliosis (LB) were present in 11 (7.6%). Immunoglobulin M and/or G borrelial serum antibodies were present in 72 patients (50%). Borreliae were isolated from BL lesions in 14 of 42 patients (33.3%) who had not received antibiotics before skin biopsy. Of 13 typed Borrelia strains, 11 were B. afzelii, 1 was B. garinii, and 1 was B. bissettii The median duration of BL after starting antibiotic treatment was 21 days ([IQR], 10-30 days); the average duration was longer in patients who were older, had longer BL duration before treatment, or had signs of disseminated LB. Treatment failure occurred in 14 of 144 patients (9.7%). Patients with signs or symptoms of disseminated LB before treatment had nearly 4 times higher odds of treatment failure (95% confidence interval, 1.22-13.07) than those without such symptoms. All patients with treatment failure had uneventful outcome after retreatment. CONCLUSIONS: BL is a rare manifestation of early localized LB. Fourteen-day antibiotic treatment, as used for erythema migrans, is effective.

Clinical spectrum of skin manifestations of Lyme borreliosis in 204 children in Austria.

The spectrum of skin manifestations of Lyme borreliosis in children is not well characterized. We conducted a retrospective study to analyze the clinical characteristics, seroreactivity to Borrelia burgdorferi sensu lato, and outcome after treatment in 204 children with skin manifestations of Lyme borreliosis seen in 1996-2011. Solitary erythema migrans was the most common manifestation (44.6%), followed by erythema migrans with multiple lesions (27%), borrelial lymphocytoma (21.6%), and acrodermatitis chronica atrophicans (0.9%). A collision lesion of a primary borrelial lymphocytoma and a surrounding secondary erythema migrans was diagnosed in 5.9% of children. Rate of seroreactivity to B. burgdorferi s.l. was lower in solitary erythema migrans compared to other diagnosis groups. Amoxicillin or phenoxymethylpenicillin led to complete resolution of erythema migrans within a median of 6 (solitary) and 14 days (multiple lesions), respectively, and of borrelia lymphocytoma within a median of 56 days. In conclusion, erythema migrans with multiple lesions and borrelial lymphocytoma appear to be more frequent in children than in adults, whereas acrodermatitis chronica atrophicans is a rarity in childhood. The outcome after antibiotic therapy was excellent in children, and appears to be better than in adults.

Enlargement of the infraorbital nerve: an important sign associated with orbital reactive lymphoid hyperplasia or immunoglobulin g4-related disease.

OBJECTIVE: To describe the clinical, histopathologic, and radiologic features of a recently identified cause for enlargement of the infraorbital canal. DESIGN: Retrospective, noncomparative case series. PARTICIPANTS: Consecutive patients were identified from the orbital databases at Moorfields Eye Hospital, London, England, and the Royal Victorian Eye and Ear Hospital, Melbourne, Australia. METHODS: A retrospective, noncomparative review of the clinical case notes, radiology, and histopathology was performed. The English-language medical literature was reviewed for reports of enlargement of the infraorbital canal or nerve. MAIN OUTCOME MEASURES: Extent of clinical and radiologic changes in patients with enlargement of the infraorbital canal. RESULTS: A total of 14 patients (10 male) presented between the ages of 29 and 76 years with proptosis, eyelid swelling or a mass (10/14 cases), and periocular ache (5/14 cases). Clinical evidence of bilateral involvement was present in 6 of 14 patients. None had impairment of visual functions or facial sensation, but 4 of 14 patients had some reduction in ocular motility. Imaging showed a focal orbital mass in 10 of 14 patients (16/28 orbits), and all patients (22/28 orbits) had enlargement of some extraocular muscles. The infraorbital canal was enlarged in 20 of the 28 orbits, with associated ipsilateral orbital changes in 19 of 20 (all 14 patients) and ipsilateral maxillary sinus changes in 12 of 20 (11 patients). Biopsy-proven chronic orbital inflammation was present in all patients; this resembled reactive lymphoid hyperplasia (RLH) in 7 patients and immunoglobulin (Ig) G4-related sclerosing inflammation in 7 patients. When tested, serum IgG4 was elevated in 6 of 7 patients. Clinical or histologically proven enlargement of cervical lymph nodes was present in 7 of 14 patients. All patients responded well to systemic corticosteroid therapy, although some had a relapse upon withdrawal. One patient developed diffuse large B-cell lymphoma and subsequently leukemia, of which he later died 20 years after presentation. CONCLUSIONS: Enlargement of the infraorbital nerve and canal is rare and strongly suggests a diagnosis of RLH or IgG4-related disease, especially in the presence of ipsilateral extraocular muscle enlargement, sinus disease, or focal orbital disease.

HIV-related CD8+ cutaneous pseudolymphoma: efficacy of methotrexate.

Human immunodeficiency virus (HIV)-related CD8+ cutaneous pseudolymphoma (CD8+ cytotoxic T cell skin infiltrative disease) is an inflammatory process resulting from a massive infiltration of the skin by activated, oligoclonal, HIV-specific, cytotoxic T lymphocytes. Usually, CD8+ cutaneous pseudolymphoma affects patients with a deep immunosuppression, and is rare in patients with mild immunosuppression. In deeply immunocompromised patients, highly active antiretroviral therapy (HAART) is considered as the first-line treatment. In contrast, the choice of therapy in moderately immunocompromised patients and/or patients already receiving HAART remains nonconsensual. We report a case of HIV-related CD8+ cutaneous pseudolymphoma in a moderately immunocompromised patient who was successfully and safely treated with methotrexate. We review the literature on HIV-related CD8+ pseudolymphoma and the use of methotrexate in HIV-positive patients.