Experience With Bexarotene to Treat Cutaneous T-Cell Lymphomas: A Study of the Spanish Working Group of Cutaneous Lymphomas.

BACKGROUND AND OBJECTIVES: Bexarotene has been approved to treat advanced stage cutaneous T-cell lymphomas (CTCL) since 1999. However, very few data have been published on its long-term safety and efficacy profile. The aim of this study is to determine the tolerability to bexarotene and outcomes by collecting the 2nd largest case series to date on its long-term use vs CTCL. MATERIAL AND METHOD: This was a multicenter retrospective review of 216 patients with mycosis fungoides (174), or Sézary syndrome (42) on a 10-year course of bexarotene alone or in combination with other therapies at 19 tertiary referral teaching hospitals. RESULTS: A total of 133 men (62%) and 83 women (38%) were included, with a mean age of 63.5 year (27-95). A total of 45% were on bexarotene monotherapy for the entire study period, 22% started on bexarotene but eventually received an additional therapy, 13% were on another treatment but eventually received bexarotene while the remaining 20% received a combination therapy since the beginning. The median course of treatment was 20.78 months (1-114); and the overall response rate, 70.3%. Complete and partial response rates were achieved in 26% and 45% of the patients, respectively. Treatment was well tolerated, being the most common toxicities hypertriglyceridemia (79%), hypercholesterolemia (71%), and hypothyroidism (52%). No treatment-related grade 5 adverse events were reported. CONCLUSIONS: Our study confirms bexarotene is a safe and effective therapy for the long-term treatment of CTCL.

Experience With Bexarotene to Treat Cutaneous T-Cell Lymphomas: A Study of the Spanish Working Group of Cutaneous Lymphomas. / [Artículo traducido] Experiencia con bexaroteno en linfoma cutáneo de células T: un estudio del Grupo Español de Linfomas Cutáneos (GELC).

BACKGROUND AND OBJECTIVES: Bexarotene has been approved to treat advanced stage cutaneous T-cell lymphomas (CTCL) since 1999. However, very few data have been published on its long-term safety and efficacy profile. The aim of this study is to determine the tolerability to bexarotene and outcomes by collecting the 2nd largest case series to date on its long-term use vs CTCL. MATERIAL AND METHOD: This was a multicenter retrospective review of 216 patients with mycosis fungoides (174), or Sézary syndrome (42) on a 10-year course of bexarotene alone or in combination with other therapies at 19 tertiary referral teaching hospitals. RESULTS: A total of 133 men (62%) and 83 women (38%) were included, with a mean age of 63.5 year (27-95). A total of 45% were on bexarotene monotherapy for the entire study period, 22% started on bexarotene but eventually received an additional therapy, 13% were on another treatment but eventually received bexarotene while the remaining 20% received a combination therapy since the beginning. The median course of treatment was 20.78 months (1-114); and the overall response rate, 70.3%. Complete and partial response rates were achieved in 26% and 45% of the patients, respectively. Treatment was well tolerated, being the most common toxicities hypertriglyceridemia (79%), hypercholesterolemia (71%), and hypothyroidism (52%). No treatment-related grade 5 adverse events were reported. CONCLUSIONS: Our study confirms bexarotene is a safe and effective therapy for the long-term treatment of CTCL.

Prognostic factors in patients with primary cutaneous anaplastic large cell lymphoma: a multicentric, retrospective analysis of the Spanish Group of Cutaneous Lymphoma.

BACKGROUND: Reliable prognostic factors for patients with primary cutaneous anaplastic large cell lymphoma (PCALCL) are lacking. OBJECTIVE: To identify prognostic factors for specific survival in patients with PCALCL. METHODS: Using the convenience sampling method, patients with PCALCL diagnosed from May 1986 to August 2017 in 16 University Departments were retrospectively reviewed. RESULTS: One hundred eight patients were included (57 males). Median age at diagnosis was 58 years. All of them showed T1-3N0M0 stages. Seventy per cent of the cases presented with a solitary lesion, mostly at the limbs. Complete response rate after first-line treatment was 87%, and no advantage was observed for any of them (surgery, radiotherapy, chemotherapy or other approaches). Nodal and visceral progression rate was 11% and 2%, respectively. 5-year specific survival (SSV) reached 93%; 97% for T1 patients and 84% for T2/T3 patients (P = 0.031). Five-year SSV for patients developing early cutaneous relapse was 64%; for those with late or no relapse, 96% (P = 0.001). Estimated median SSV for patients showing nodal progression was 103 months (95% CI: 51-155 months); for patients without nodal progression, estimated SSV did not reach the median (P < 0.001). Nodal progression was an independent predictive parameter for shorter survival (P = 0.011). CONCLUSION: Multiple cutaneous lesions at presentation, early skin relapse and nodal progression portrait worse prognosis in patients with PCALCL.

First-line treatment in lymphomatoid papulosis: a retrospective multicentre study.

BACKGROUND: Data regarding response to treatment in lymphomatoid papulosis (LyP) are scarce. AIM: To assess the daily clinical practice approach to LyP and the response to first-line treatments. METHODS: This was a retrospective study enrolling 252 patients with LyP. RESULTS: Topical steroids, methotrexate and phototherapy were the most common first-line treatments, prescribed for 35%, 20% and 14% of the patients, respectively. Complete response (CR) was achieved in 48% of treated patients. Eczematous lesions significantly increased relative risk (RR) of not achieving CR (RR = 1.76; 95% CI 1.16-2.11). Overall median time to CR was 10 months (95% CI 6-13 months), and 78% of complete responders showed cutaneous relapse; both results were similar for all treatment groups (P > 0.05). Overall estimated median disease-free survival (DFS) was 11 months (95% CI 9-13 months) but DFS for patients treated with phototherapy was 23 months (95% CI 10-36 months; P < 0.03). Having the Type A LyP variant (RR = 2.04; 95% CI 0.96-4.30) and receiving a first-line treatment other than phototherapy (RR = 5.33; 95% CI 0.84-33.89) were significantly associated with cutaneous early relapse. Of the 252 patients, 31 (13%) had associated mycosis fungoides unrelated to therapeutic approach, type of LyP or T-cell receptor clonality. CONCLUSIONS: Current epidemiological, clinical and pathological data support previous results. Topical steroids, phototherapy and methotrexate are the most frequently prescribed first-line treatments. Although CR and cutaneous relapse rates do not differ between them, phototherapy achieves a longer DFS. Presence of Type A LyP and use of topical steroid or methotrexate were associated with an increased risk of early relapse.

Coexistence of marginal zone cutaneous B-cell lymphoma and classic Hodgkin disease: does a biological relationship exist?

The coexistence of non-Hodgkin lymphoma (NHL) and Hodgkin disease (HD) in the same patient, although previously reported, is very unusual. This situation is extremely rare when the first diagnosis is a cutaneous B NHL, and exceptional if there is no personal background of cytostatic treatment. We report a 44-year-old man who developed cutaneous nodules over a period of two years. A marginal zone cutaneous B-cell lymphoma was diagnosed. On staging investigation a mass in the lingual tonsil was found and excision biopsy showed a classical Hodgkin lymphoma.

Cutaneous expression of systemic candidiasis.

Skin lesions associated with Candida septicaemia occur only in a minority of patients, who are usually immunocompromised, but they can help to establish a diagnosis rapidly. The lesions form a characteristic maculopapular or nodular rash at the onset of the infection. We report three cases of systemic candidiasis (SC) with cutaneous manifestations in immunocompromised patients. In these patients, the lesions started as asymptomatic or slightly pruriginous macules, papules or nodules localized on the trunk and extremities. The patients' general condition was very poor and they presented a high fever at the onset of the illness. Candida spp. were isolated from blood in all cases, and histology showed yeasts in two of them. Most of the lesions resolved with antifungal treatment. The diagnosis of SC is often delayed or missed because of the absence of useful diagnostic tools, the varying clinical manifestations and the frequent negativity (50-75%) of blood cultures for Candida. Fluconazole is the treatment of choice for Candida albicans, but treatment response is unknown for other Candida spp., which may require treatment with amphotericin B.

Stage IV cutaneous acute graft-versus-host disease. Clinical and histological study of 15 cases.

BACKGROUND: The most severe form of cutaneous acute graft-versus-host disease (aGVHD), stage IV, is characterized by the appearance of vesicles and blisters. OBJECTIVE: To describe the clinicopathological characteristics and evolution of stage IV cutaneous aGVHD presented in our hospital. METHOD: Retrospective study. The following criteria for inclusion were applied: (i) patients subjected to allogeneic stem cell transplantation between 1st January 1984 and 31st of December 2006; (ii) development of vesicles and/or blisters; (iii) extracutaneous coincidental aGVHD manifestations; and (iv) presence of histopathological features consistent with aGVHD. RESULTS: Fifteen cases (10 females and 5 males) were studied. The mean age was 38.1 years. The lesions appeared after a median interval of 19 days, always following a milder stage of GVHD. Two patterns of clinical evolution were found. Mucosal involvement was observed in nine patients. Nikolsky's sign was positive in eight patients. Nine of the patients had biopsies of the vesiculobullous stage which showed a subepidermal blister with epidermal necrosis and basal vacuolar degeneration. Only two patients survived. CONCLUSION: Stage IV cutaneous aGVHD is a severe and unusual complication after haematopoietic stem cell transplantation. Prognosis is poor with a very high mortality rate, although the cause of death is varied and not strictly linked to the cutaneous disease.

Flare and change of psoriasis morphology during the course of treatment with tumour necrosis factor blockers.

Tumour necrosis factor (TNF) blockers represent an exciting advance in the management of psoriasis. However, the safety profile of these drugs is not completely established. We present a review of the literature, and report on eight patients: two with the unexpected appearance of psoriasis, and the remaining six with exacerbation and change in morphology of their existing psoriasis, all of which occurred during treatment with the TNF blockers adalimumab, etanercept and infliximab. The two new cases, neither of whom had any personal or family history of psoriasis, developed pustular psoriasis on the palms and/or soles. The other six patients, previously diagnosed with severe chronic plaque psoriasis (four patients), generalized pustular psoriasis (one) and erythrodermic psoriasis (one), developed eruptive guttate psoriasis between 15 days and 18 months after the beginning of therapy. These patients had never before presented guttate-type psoriatic lesions, and the lesions appeared in areas of the body that were free of psoriatic plaques at baseline.

Unusual clinical presentation of amyloidosis: bilateral stenosis of the external auditory canal, hoarseness and a rapid course of cutaneous lesions.

We report a patient with multiple myeloma associated with primary systemic amyloidosis who had a rapid evolution and a very unusual form of presentation. The association of amyloidosis in patients with multiple myeloma is 15%, and clinically evident mucocutaneous involvement occurs in up to 40% of patients.

[Kaposi's sarcoma treated with topical alitretinoin]. / Sarcoma de Kaposi clásico tratado con alitretinoína tópica.

Kaposi's sarcoma is a multifocal neoplastic process with four clinical variants, all of them induced by human herpes virus 8. Currently there is no treatment of choice and it depends on the extension and location of the lesions as well as on the clinical type of the disease. Alitretinoin gel 0.1 % is approved for the treatment of cutaneous lesions of AIDS-associated Kaposi's sarcoma. The majority of its side effects appear at the site of application and can lead to therapy withdrawal. We report a case of Kaposi's sarcoma treated with topical alitretinoin that had a favourable evolution in spite of an intense local reaction.

[Hidradenitis suppurativa. Response to treatment with infliximab]. / Hidrosadenitis supurativa. Respuesta al tratamiento con infliximab.

INTRODUCTION: Hidradenitis suppurativa is a chronic inflammatory disease that runs in outbreaks with painful lesions, fistulas and scars in axillae, groins, buttocks, and perianal and submammary regions. Among multiple drug therapies available, infliximab, usually employed in dermatology to control psoriasis, has shown its efficacy in the past five years. PATIENTS AND METHOD: It is a prospective, observational study to determine the efficacy and safety of infliximab in the treatment of hidradenitis suppurativa. We selected three women with a history of hidradenitis suppurativa of more than 10 years, with involvement of at least two anatomic locations that was recalcitrant to conventional therapies. Each patient received infliximab at a dose of 5mg/kg/infusion on weeks 0, 2, 6 and every 8 weeks thereafter. RESULTS: Two of the three patients showed mild to moderate improvement of their disease while the third patient did not improve. We can highlight the variability of the results observed in these three patients. Adverse effects were generally mild and well tolerated by the three patients. Despite this, two patients withdrew the therapy due to loss of efficacy in one case and the development of generalized arthalgias in the other case. CONCLUSIONS: Treatment of hidradenitis supurativa with infliximab constitutes a moderately useful alternative in some cases.

[Juvenile multiple xanthogranuloma in a patient with Langerhans cell histiocytosis]. / Xantogranuloma juvenil múltiple en una paciente con histiocitosis de células de Langerhans.

We present the case of a 10-week-old girl who had erythematous papules with a yellowish hue from birth with diagnosis of Langerhans cell histiocytosis, that was accompanied by a lytic lesion in the skull and hepatic involvement. After several months of treatment with prednisone and vinblastine with skin and systemic improvement, several rounded erythematous papules with a yellowish hue appeared in the right cheek. The biopsy showed a histiocytic infiltrate with positivity for CD68 and negative staining for S100 and CD1a, with a final diagnosis of juvenile xanthogranuloma. This association has been previously described in the literature in few cases. Although several hypotheses have been suggested, the causal relationship between both entities has still not been demonstrated.

Sweet's syndrome on the area of postmastectomy lymphoedema.

BACKGROUND: Sweet's syndrome (SS) has been reported in association with many conditions, including malignancy, infections, autoimmune disorders, pregnancy and drugs. MATERIALS AND METHODS: We reviewed patients with SS-like lesions on the lymphoedema area seen in our department. Clinical manifestations, histopathologic characteristics, treatment and outcome data were recorded and analysed. RESULTS: We report seven women with a history of surgery for breast cancer with axillary lymphadenectomy. Six of them were on tamoxifen. All of them had various lesions consistent with SS localized predominantly on the limb affected by the postmastectomy lymphoedema, and on the ipsilateral chest, trunk and back. One of them presented bullous lesions. Three of the cases underwent spontaneous remission, two resolved with antibiotic therapy, one healed with corticosteroids, and one with corticosteroids plus antibiotic. CONCLUSIONS: Erythematous tender plaques on the area of postmastectomy lymphoedema could be considered an unusual manifestation of Sweet's syndrome. We have found only three similar cases in the literature. Although it is difficult to elucidate the pathogenesis of this entity, it has been suggested that it could be due to immune surveillance impairment.