Itchy Skin: A Challenging Differential Diagnosis Between Mycosis Fungoides and Sézary Syndrome.

Primary cutaneous lymphomas represent a diverse spectrum of T-cell and B-cell lymphomas with their primary skin manifestation. Among these, mycosis fungoides (MF) and Sézary syndrome (SS) represent classic forms of cutaneous T-cell lymphomas (CTCLs). This report details the case of a 67-year-old female who presented with longstanding pruritic skin lesions, initially misdiagnosed and managed as eczema. The diagnostic process ultimately revealed the presence of Sézary cells in the peripheral blood smear (PBS). The SS diagnosis was confirmed based on CD4 positivity and CD7 negativity as determined by flow cytometry. The disease was staged as IVA1 (T2N0M1B2). The patient exhibited partial improvement with oral corticosteroid therapy. This report underscores the critical importance of integrating clinical evaluation and blood findings to distinguish between MF and SS. The progression of a circulating clone signals a poor prognosis, requiring surveillance and consideration of targeted therapies to enhance patient outcomes and improve their quality of life. Early detection remains paramount in the management of these rare cutaneous lymphomas, which are associated with unique therapeutic challenges.

The Importance of Patient Empowerment: A Clinical Case of Hereditary Angioedem.

Hereditary angioedema (HAE) is a rare condition characterized by recurrent episodes of angioedema without urticaria or pruritus. Untreated angioedema can cause significant work absenteeism and, in rare cases, be lethal due to laryngeal involvement and suffocation. The authors report a case of a patient with laryngeal involvement who was unaware of the severity of their condition. Effective medical training in patient empowerment is essential, and it is an irreplaceable element in healthcare, as it contributes to therapeutic success.

Leg ulcers in antiphospholipid syndrome secondary to systemic lupus erythematosus treated with intravenous immunoglobulin.

BACKGROUND: Despite encouraging reports on the efficacy of intravenous immunoglobulin (IVIg) in antiphospholipid syndrome, the clinical value of this treatment is not well established, and most of the data are based on case reports and small series of patients. OBSERVATION: We describe the significant improvement of leg ulcers with IVIg in a 61-year-old female, with diabetes mellitus, venous peripherical insufficiency and secondary antiphospholipid syndrome to systemic lupus erythematosus. CONCLUSIONS: This case illustrates a rare cause of leg ulcers and documents that IVIg may be an effective adjuvant treatment in the management of selected patients with antiphospholipid syndrome when conventional strategies using subcutaneous heparin and low-dose aspirin are insufficient.

Syndrome in question. Multiple autoimmune syndrome.

Multiple autoimmune syndrome is a rare condition, described by Humbert and Dupond in 1988. It is defined by the association of at least 3 autoimmune diseases in the same patient. Vitiligo is the most common skin condition in this syndrome. This article presents the case of a 31-year-old male with vitiligo, alopecia areata, Crohn's disease, psoriasis vulgaris and oral lichen planus. The rarity of this case is highlighted by the coexistence of four autoimmune skin diseases in association with Crohn's disease, never described in the literature.

Syndrome in Question

Multiple autoimmune syndrome is a rare condition, described by Humbert and Dupond in 1988. It is defined by the association of at least 3 autoimmune diseases in the same patient. Vitiligo is the most common skin condition in this syndrome. This article presents the case of a 31-year-old male with vitiligo, alopecia areata, Crohn's disease, psoriasis vulgaris and oral lichen planus. The rarity of this case is highlighted by the coexistence of four autoimmune skin diseases in association with Crohn's disease, never described in the literature.

Case for diagnosis. Cutaneous involvement associated to multiple myeloma.

Cutaneous involvement associated to multiple myeloma varies from 5 to 10% of cases and is infrequently recognized. Cutaneous metastatic plasmacitomas are rare. We present the case of a 72-year-old man with multiple myeloma in complete remission since 2 years ago with cutaneous tumors on the trunk and face. A cutaneous biopsy was consistent with plasmacytoma. The patient was treated with melphalan, prednisolone and radiotherapy. Despite optimal therapeutic response of the lesions, the disease progressed, with the appearance of new extra-cutaneous plasmocytomas. The cutaneous metastatic plasmocytomas were the first sign of progression of the disease.

Case for diagnosis

Cutaneous involvement associated to multiple myeloma varies from 5 to 10% of cases and is infrequently recognized. Cutaneous metastatic plasmacitomas are rare. We present the case of a 72-year-old man with multiple myeloma in complete remission since 2 years ago with cutaneous tumors on the trunk and face. A cutaneous biopsy was consistent with plasmacytoma. The patient was treated with melphalan, prednisolone and radiotherapy. Despite optimal therapeutic response of the lesions, the disease progressed, with the appearance of new extra-cutaneous plasmocytomas. The cutaneous metastatic plasmocytomas were the first sign of progression of the disease.

One-stage reconstruction of full-thickness lower eyelid using a Tripier flap lining by a septal mucochondral graft.

Basal cell carcinoma (BCC) is the most frequent cutaneous neoplasm of the periorbital region. After tumor excision, the function of the eyelid has to be preserved, keeping the protection of the eyeball with preservation of damp, avoiding epiphora and ectropion. The authors describe the reconstruction, in a single surgical procedure, of a full-thickness defect of the outer half of the free edge of the lower eyelid through free chondromucosal graft of the septum and Tripier flap. There are very few cases that describe the septal chondromucosal flap in eyelid reconstruction. This case also corroborates the fact that the chondromucosal graft is a valuable surgical option for reconstruction of full-thickness lower eyelid defects.

Exacerbation of recalcitrant cutaneous sarcoidosis with adalimumab--a paradoxical effect? A case report.

The paradoxical adverse effects of tumor necrosis factor-alpha (TNF-alpha) antagonists have been described frequently as a result of the widespread use of these drugs. Among the TNF-alpha blocking agents, few reports exist relating the use of adalimumab in cutaneous sarcoidosis, although all of them show good results. More recently, sarcoidosis onsets have been reported with various TNF-alpha inhibitors. The current case is, to our knowledge, the first to describe the exacerbation of cutaneous lesions of sarcoidosis treated with adalimumab.

Do you know this syndrome? / Voce conhece esta sindrome?

POEMS syndrome is a unique clinical entity, the diagnosis of which is made when polyneuropathy and monoclonal gammopathy occur together, associated with other changes such as organomegaly, endocrinopathy, skin changes and papilledema. Cutaneous manifestations are heterogeneous, with diffuse cutaneous hyperpigmentation, hemangiomas and hypertrichosis occurring more frequently. We report the case of a 65- year-old female patient with this syndrome, diagnosed after 15 years of disabling peripheral neuropathy.

A síndrome de POEMS é uma entidade clínica única cujo diagnóstico é colocado quando existe polineuropatia e gamapatia monoclonal associada a outras alterações tais como organomegalia, endocrinopatia, alterações cutâneas e papiledema. As manifestações cutâneas são heterogéneas, sendo as mais frequentes a hiperpigmentação cutânea difusa, os hemangiomas e a hipertricose. Relata-se o caso de uma doente de 65 anos com essa síndrome diagnosticada após 15 anos de neuropatia periférica.

Exacerbation of recalcitrant cutaneous sarcoidosis with adalimumab - a paradoxical effect? A case report / Agravamento de lesoes refratarias de sarcoidose cutanea com adalimumab- um efeito paradoxal? Relato de um caso

The paradoxical adverse effects of tumor necrosis factor-alpha (TNF-alpha) antagonists have been described frequently as a result of the widespread use of these drugs. Among the TNF-alpha blocking agents, few reports exist relating the use of adalimumab in cutaneous sarcoidosis, although all of them show good results. More recently, sarcoidosis onsets have been reported with various TNF-alpha inhibitors. The current case is, to our knowledge, the first to describe the exacerbation of cutaneous lesions of sarcoidosis treated with adalimumab.

Os efeitos paradoxais dos anti-TNF-alpha têm sido cada vez mais descritos com a utilização mais ampla dessas drogas. Entre os TNF-alpha, registam-se poucos casos com a utilização de adalimumab no tratamento da sarcoidose cutânea, sendo que todos eles apresentam bons resultados. Têm sido descritos, mais recentemente, casos de sarcoidose induzidos por vários anti-TNF-alpha. O presente caso é, até à data, o primeiro a descrever a exacerbação de lesões cutâneas de sarcoidose tratadas com adalimumab.

Do you know this syndrome? POEMS syndrome.

POEMS syndrome is a unique clinical entity, the diagnosis of which is made when polyneuropathy and monoclonal gammopathy occur together, associated with other changes such as organomegaly, endocrinopathy, skin changes and papilledema. Cutaneous manifestations are heterogeneous, with diffuse cutaneous hyperpigmentation, hemangiomas and hypertrichosis occurring more frequently. We report the case of a 65- year-old female patient with this syndrome, diagnosed after 15 years of disabling peripheral neuropathy.