Case of pityriasis rubra pilaris progressed to generalized erythroderma following blockade of interleukin-17A, but improved after blockade of interleukin-12/23 p40.

We report herein a case of a 72-year-old man with pityriasis rubra pilaris (PRP) that was refractory to conventional therapies. His skin lesions progressed to generalized erythroderma despite anti-interleukin (IL)-17A antibody therapy. Topical corticosteroids, emollients, systemic retinoid, methotrexate, cyclosporin and phototherapy yielded no therapeutic response. However, blockade of IL-12/23 p40 dramatically improved his cutaneous lesions. Complete remission was achieved 4 weeks after the first injection of ustekinumab and maintained for more than 48 weeks. Our data indicate that IL-12 was associated with the onset of PRP in this patient, rather than IL-23. IL-12 is critical for the differentiation of T-helper (Th)1 cells. Thus, the Th1 pathway may be associated with the onset of PRP.

Estudio clínico-histopatológico de las eritrodermias en un hospital universitario: 14 años de experiencia / Clinical-histopathological study of erythroderma in a university hospital: 14 years of experience

INTRODUCCIÓN: La eritrodermia es un síndrome inflamatorio cutáneo infrecuente caracterizado por compromiso eritematoso generalizado y descamación, de más del 90% de superficie cutánea total. OBJETIVO: Caracterizar clínica e histopatológicamente a los pacientes con eritrodermia en un hospital universitario chileno. METODOLOGÍA: Estudio retrospectivo, realizado en el Hospital Clínico Universidad de Chile, basado en revisión de fichas clínicas e informes histopatológicos de pacientes con eritrodermia, entre 2005 y 2018. Se evaluó edad, sexo y variables clínicas (co-morbilidades, síntomas, días de evolución, ingreso hospitalario, informe histopatológico, diagnóstico y evolución). RESULTADOS: Total de 28 pacientes, 18 hombres (64%), edad promedio 59 años. Causa más frecuente de eritrodermia fue dermatosis pre-exis-tentes, con 15 casos (54%), que incluyen: psoriasis 9 (32%), dermatitis de contacto 3 (11%), PRP 2 (7%), dermatitis atópica 1 (4%). A estas le siguen: reacción adversa medicamentosa 6 (21%), idiopática 6 (21%) y Síndrome de Sezary 1 (4%). CONCLUSIÓN: El presente estudio corresponde a la primera serie de eritrodermias realizada en Chile. Destacan las dermatosis preexistentes como la principal causa, lo que se correlaciona con la literatura.

INTRODUCTION: Erythroderma is an infrequent cutaneous inflammatory disorder characterized by generalized erythematous compromise and desquamation, of more than 90% of total cutaneous surface. OBJECTIVE: Clinical and histopathological cha-racterization of patients with erythroderma in a Chilean university hospital. METHODOLOGY: Retrospective study, performed at the University of Chile Clinical Hospital, based on review of clinical records and histopatho-logical reports of patients with erythroderma, between 2005 and 2018. Age, sex and clinical variables were evaluated (co-morbidities, symp-toms, days of evolution, hospital admission, histopathological report, diagnosis and evolu-tion). RESULTS: A total of 28 patients, 18 were men (64%), average age 59 years. Most frequent cause of erythroderma was pre-existing dermatosis, with 13 cases (52%), which included: psoriasis 9 (32%), contact dermatitis 3 (11%), PRP 2 (7%), atopic dermatitis 1 (4%). These are followed by adverse drug eruption 6 (21%), idiopathic 6 (21%) and Sezary syndrome 1 (4%). CONCLUSION: The present study corresponds to the first series of erythrodermas performed in Chile. The pre-existing dermatoses were the main cause of erythroderma, which coincides with other reports.

Erythroderma: A clinical-etiological study of 82 cases.

Erythroderma is an uncommon skin disorder characterized by generalized reddening and scaling of over 90% of the skin. It represents a maximal stage of skin irritation induced by several skin diseases such as psoriasis, contact dermatitis, drug reactions, and mycosis fungoides. Data including the clinical symptoms, laboratory examinations and skin biopsies were collected from 82 erythroderma patients admitted to our hospital in the period between Jan.1st, 2003 and Dec.31st, 2008. According to clinical findings, laboratory findings and biopsy results, the most common causative factors were pre-existing dermatoses (72.0%), followed by drug reactions (17.0%), idiopathic causes (6.1%) and malignancies (4.9%). Among the pre-existing dermatoses, psoriasis is the most common etiology (30.5%). We also found hypereosinophilic syndrome, sarcoidosis and dermatomyositis could be causes of erythroderma. In the drug-induced group, Chinese traditional herbal medicines were probably the most frequently implicated drugs in our series, with 9 of the 14 cases (64.3%). Follow-up information was obtained for 65 patients, and most of our patients had improved symptoms after treatment. In our series we found a high percentage of erythroderma secondary to pre-existing dermatoses and a low percentage of erythroderma secondary to malignancy. Among drugs as an etiological group, Chinese traditional herbal medicines were the most frequent drugs. From our follow-up study, the prognosis of most patients with erythroderma is relatively good.

Generalized leukaemia cutis from a small cell variant of T-cell prolymphocytic leukaemia presenting with exfoliative dermatitis.

T-cell prolymphocytic leukaemia (T-PLL) is a rare, aggressive neoplasm of mature T lymphocytes. The small cell variant occurs in approximately 20% of T-PLL patients. The skin findings of leukaemia consist of leukaemia-specific skin lesions, which are infiltrated by leukaemia cells, and non-specific lesions. The former type of lesion signifies leukaemia cutis. Leukaemia cutis presents clinically as tumours, nodules, or patches on the scalp, face and trunk. We report here an 82-year-old Korean male patient who presented with erythema, erosion, vesicles, and scales on his entire body with no clear underlying cause. He had been treated with oral retinoids, steroids, and phototherapy for the diagnoses of drug eruption, pityriasis rubra pilaris, and exfoliative dermatitis at other hospitals. We suspected a hidden malignancy and diagnosed small cell variant T-PLL through blood and bone marrow examination. A skin biopsy specimen showed dense infiltration of small lymphocytes in the dermis. Most of the atypical lymphocytes stained positively with CD markers such as CD2, CD3, CD4, CD5, CD7 and CD8, thereby confirming the presence of leukaemia cells. To our knowledge, this is the first case of generalized leukaemia cutis from small cell variant of T-PLL presenting with exfoliative dermatitis over the whole body.

Generalized mucinosis in a patient with erythroderma.

We describe an 81-year-old Japanese patient with erythroderma overlapping with widespread and symmetrical deposits of mucin in the upper dermis. Clinically, the mucinous lesions on the nape and upper trunk were localized papular mucinosis. Histologically, there was a perivascular infiltrate of lymphohistiocytic cells mingled with plasma cells in the upper dermis but no sclerosis. Immunohistochemical staining revealed that more than 90% of these infiltrating plasma cells produced immunoglobulin lambda-chain. Both the erythroderma and generalized mucinosis responded to topical steroid and PUVA therapy. To the best of our knowledge, this is the first case of erythroderma accompanied by generalized mucinosis.

Mycosis fungoides presenting as Ofuji's papuloerythroderma.

We report three patients presented with clinical features of Ofuji's papuloerythroderma (pruritic erythematous papules and extensive erythema sparing all skin folds), however, showing histopathological findings of mycosis fungoides (Pautrier's microabscess, haloed lymphocytes, disproportionate epidermotropism, and wiry collagen bundles). One case was associated with plaque stage of mycosis fungoides and follicular mucinosis. T-cell receptor (TCR) gene rearrangement analysis in the lesional skin tissue demonstrated rearrangement of the gamma chain in all cases. HTLV-1 serology was negative for two patients who conducted HTLV-1 test. We think that Ofuji's papuloerythroderma might be a variant of early mycosis fungoides rather than secondary skin manifestations to certain cutaneous inflammatory diseases.

[Ofuji's papulo-erythroderma: treatment with cyclosporin A]. / Papulo-érythrodermie d'Ofuji: traitement par ciclosporine-A.

BACKGROUND: Papuloerythroderma of Ofuji is an uncommon condition characterized by diffuse pruriginous eruptions of infiltrating papulae. The large folds are not involved. The eruptions are associated with lymphopenia and eosinophilia. Very few cases have been reported in the literature and because of their heterogeneous nature, there is some debate as to whether this is a single clinical entity or a particular presentation of erythrodermia; Immunomodulation is recommended. CASE REPORT: A 73 year-old man of asian origin living in France presented chronic pruriginous erythrodermia with flat purplish-brown confluent papulae which did not involve the large folds. Eosophilia and lymphopenia were present. The biopsy specimen evidenced dermal infiltration with CD4+CD45+RO+ T cells, eosinophils and DC1a+ dendritic cells. Further explorations did not reveal any sign favoring lymphoma, carcinoma or underlying infection. Dermocorticoids, PUVA-therapy and interferon-alpha were ineffective. Considerable clinical improvement was obtained with cyclosporine A. DISCUSSION: We used ciclosporine A in our patient after repeated failure of other therapies reported to be effective in this dermatosis. Despite the favorable outcome, this therapeutic approach should be used with prudence.

Ofuji's papuloerythroderma: a study of 17 cases.

In this retrospective study the clinical, laboratory and histological features, treatment methods and disease course in 17 patients with papuloerythroderma (PE) are reviewed. The median age at diagnosis was 72 years and the female/male ratio was 1:4:7. The most common abnormal laboratory findings were eosinophilia and an elevated serum IgE level. Psoralen photochemotherapy (PUVA) and oral prednisolone 10-20 mg daily given in combination or alone were very efficient treatments, while UVB phototherapy in combination with topical steroids was also successful. However, potent topical corticosteroids alone or in combination with oral antihistamines were not effective. The patients were observed for a median of 19 months from diagnosis, three being followed for more than 5 years. Five of the patients relapsed, but only one had multiple relapses, two developed cutaneous T-cell lymphoma (CTCL) and two others had histological features suggestive of that disorder. Six of the patients died, cardiovascular disease being the most common cause. PE is a distinct clinical entity with a polymorphous aetiology which frequently includes an association with CTCL or visceral malignancy. PUVA, oral corticosteroids and UVB in combination with topical corticosteroids appear to be effective therapeutic modalities.

Photoaccentuated erythroderma associated with CD4+ T lymphocytopenia: successful treatment with 5-methoxypsoralen and UVA, interferon alfa-2b, and extracorporeal photopheresis.

We describe a 53-year-old HIV-negative white man who had chronic CD4+ T lymphocytopenia and photoaccentuated erythroderma with lymphoma-like histologic changes. The erythroderma completely responded to 5-methoxypsoralen and UVA (PUVA), interferon alfa-2b, and extracorporeal photopheresis. During therapy opportunistic skin infections, including tinea corporis, warts, and disseminated molluscum contagiosum, developed. Although the patient met the current definition of idiopathic CD4+ T lymphocytopenia (ICTL), we cannot rule out the possibility that this peripheral CD4+ T lymphocytopenia resulted from sequestration of CD4+ T lymphocytes in erythrodermic skin.

Ofuji papuloerythroderma in a patient with the acquired immunodeficiency syndrome.

Ofuji papuloerythroderma (OPE) is a distinctive clinical entity of unknown etiology which occasionally may be associated with B-cell and T-cell lymphomas and visceral malignancy. We describe a case of OPE in a male with the acquired immunodeficiency syndrome. To our knowledge, this is the first report of OPE in a patient infected by the human immunodeficiency virus.

Efficacy of ultraviolet B phototherapy for psoriasis in patients infected with human immunodeficiency virus.

To evaluate the efficacy of ultraviolet B (UVB) phototherapy for the treatment of psoriasis in patients infected with human immunodeficiency virus (HIV), the response of 14 patients was compared to that of matched seronegative control individuals. All patients were evaluated prior to treatment (baseline) and after 21 treatments for the extent of total body surface area (TBSA) involvement and the quantification of scale, erythema, and thickness of plaques using a scale of 0 (absent) to 4 (severe). The only concomitant medication allowed was salicylic acid in petrolatum. The cumulative score for scale, erythema, and thickness improved 1.9 +/- 0.5 [mean +/- standard error of mean (SEM)] in the HIV group and 2.4 +/- 0.3 in controls. There was 40.9 +/- 7.3% reduction of TBSA involvement in the former and 38.4 +/- 7.6% reduction in the latter group. None of the differences was statistically significant. There was no statistically significant difference in the response to therapy among various stages of immunosuppression in the HIV group. There was also no deterioration of immune status in this group. UVB phototherapy is an effective treatment for psoriasis in patients infected with HIV. The response is identical to that of matched control individuals.

Cutaneous T-cell lymphoma presenting as papuloerythroderma--a case and review of the literature.

Papuloerythroderma has been described as a distinct clinical entity. It is characterized by pruritus, red flat-topped papules with skin crease sparing, blood eosinophilia and lymphopenia. It has been described almost exclusively in elderly men. We report a case of cutaneous T-cell lymphoma which, at presentation, was indistinguishable from papuloerythroderma, in a 79-year-old male. An excellent clinical response to low dose oral corticosteroids and photochemotherapy was observed. There have only been two previous reports linking papuloerythroderma with T-cell lymphoma. This patient is in keeping with the two previously reported cases and would support the view that papuloerythroderma may predispose to, or in fact represent, an early form of cutaneous T-cell lymphoma.

Papuloerythroderma and cutaneous T cell lymphoma.

An 83-year-old man had the typical cutaneous features of papuloerythroderma of Ofuji. There were reduced numbers of lymphocytes and platelets in his peripheral blood but the eosinophil count was normal. Skin biopsy showed a nondiagnostic infiltrate of T lymphocytes in the dermis. Treatment with topical steroids and UVB phototherapy was ineffective. Twelve months after presentation, a further skin biopsy revealed atypical lymphoid cells invading the epidermis and the skin adnexae. A diagnosis of malignant lymphoma was made; no evidence of extracutaneous spread was found. Photochemotherapy produced rapid resolution of the skin eruption and clearance of the cutaneous infiltrate. Papuloerythroderma may be a manifestation of a cutaneous lymphoma.