Anetoderma after disseminated Mpox.

Anetoderma or macular atrophy is a rare skin condition of unclear pathogenesis, often associated with autoimmune diseases and skin damage from various infections. Human immunodeficiency virus (HIV), syphilis, and poxviruses have been implicated in the development of anetoderma. A 37-year-old male patient with HIV and recent unprotected sexual encounters presented with more than 400 skin lesions, consistent with Mpox. Symptomatic treatment for Mpox resulted in acute symptom resolution. However, 8 months later he developed papular anetoderma lesions in areas previously affected by Mpox. Biopsy confirmed the loss of elastic fibers in the affected skin areas, leading to the diagnosis of Mpox-induced anetoderma. This report presents a unique case of anetoderma following Mpox in an HIV-positive patient.

Brentuximab vedotin in the treatment of cutaneous T-cell lymphomas: Data from the Spanish Primary Cutaneous Lymphoma Registry.

BACKGROUND: Brentuximab vedotin (BV) has been approved for CD30-expressing cutaneous T-cell lymphoma (CTCL) after at least one previous systemic treatment. However, real clinical practice is still limited. OBJECTIVES: To evaluate the response and tolerance of BV in a cohort of patients with CTCL. METHODS: We analysed CTCL patients treated with BV from the Spanish Primary Cutaneous Lymphoma Registry (RELCP). RESULTS: Sixty-seven patients were included. There were 26 females and the mean age at diagnosis was 59 years. Forty-eight were mycosis fungoides (MF), 7 Sézary syndrome (SS) and 12 CD30+ lymphoproliferative disorders (CD30 LPD). Mean follow-up was 18 months. Thirty patients (45%) showed at least 10% of CD30+ cells among the total lymphocytic infiltrate. The median number of BV infusions received was 7. The overall response rate (ORR) was 67% (63% in MF, 71% in SS and 84% in CD30 LPD). Ten of 14 patients with folliculotropic MF (FMF) achieved complete or partial response (ORR 71%). The median time to response was 2.8 months. During follow-up, 36 cases (54%) experienced cutaneous relapse or progression. The median progression free survival (PFS) was 10.3 months. The most frequent adverse event was peripheral neuropathy (PN) (57%), in most patients (85%), grades 1 or 2. CONCLUSIONS: These results confirm the efficacy and safety of BV in patients with advanced-stage MF, and CD30 LPD. In addition, patients with FMF and SS also showed a favourable response. Our data suggest that BV retreatment is effective in a proportion of cases.

Apocrine hidrocystoma on the nipple: the first report in this unusual location.

Apocrine hidrocystoma is a rare, benign, cystic tumor of the apocrine sweat glands. They are most commonly located around the eyes and may also be found on the scalp and neck. However, despite the fact that the nipple and areola contain numerous apocrine sweet glands, apocrine hydrocystomas have not been described previously in this area to the best of our knowledge. We report the first case of this cyst in this unsual location.

Digital ulcerative lichenoid dermatitis in a patient receiving anti-PD-1 therapy.

Programmed cell death receptor 1 inhibitors (anti-PD-1) constitute a form of immunotherapy for the treatment of several cancers. They are associated with cutaneous immune-related adverse events (irAE), occurring in up to 50% of patients. Lichenoid dermatitis is frequent and several presentations have been described. Although attempts have been made to study these reactions, they are yet to be fully characterized and the relationship with tumor response is unclear. We describe a case of digital ulcerative lichenoid dermatitis resembling ulcerative cutaneous lichen planus that occurred during pembrolizumab therapy for oral squamous cell carcinoma. The patient developed a painful ulcer on his index finger 18 months into therapy. Biopsy revealed epidermal ulceration with intense lichenoid dermatitis. Immunohistochemical study revealed intense CD8 positivity at the ulcer's edges and marked CD163 positivity at its base. Although idiopathic forms of this type of lichenoid dermatitis are particularly recalcitrant, our case was successfully managed with topical therapy and oncologic treatment did not require modification. One year after ending treatment the patient remains free of disease progression. It is unclear if this reaction is associated with his favorable oncologic response. This report adds an undescribed reaction to the increasing diversity of cutaneous irAE associated with anti-PD-1 therapy.

Novel PTEN mutation in Cowden syndrome: case report with late diagnosis and non-malignant course.

Cowden syndrome (CS) is an infrequent genodermatosis caused by mutations in the phosphatase and tensin homolog (PTEN) gene in the majority of cases. As such, it belongs to the PTEN hamartoma tumor syndrome spectrum. This disease has a variable clinical expression characterized by the development of multiple hamartomatous tumors in different organs, usually during the second and third decades of life, and a high cumulative risk of several malignancies. We present a case of Cowden syndrome with late diagnosis presenting with a florid dermatological expression and multiple benign tumors, but no malignancies. A novel PTEN mutation was identified.

Coexistence of Pemphigus Foliaceus and Grover Disease After a Radical Surgery for Basal Cell Carcinoma.

The differential diagnosis of Grover disease (GD) includes pemphigus foliaceus. However, the focal nature of the histologic findings and negative direct immunofluorescent point to the diagnosis of GD. It has been associated with a variety of systemic and cutaneous diseases, but its association with other primary acantholytic disorders has been rarely documented. In this article, we describe the first case of GD in association with pemphigus foliaceus.

Paradoxical reversed plantar involvement during ixekizumab therapy for psoriasis.

Palmoplantar psoriasis is a particularly challenging variant of psoriasis. Psoriasis at this location has a significant impact on health-related quality of life and is often recalcitrant. However, difficult cases may respond to biologic therapies. Paradoxical reactions during treatment with biological agents have been described, mostly during anti-tumor necrosis factor therapy. These typically present as a change in morphology or distribution of lesions. We present a patient with palmoplantar psoriasis treated with ixekizumab who achieved a favorable response that was coupled with a rare paradoxical reaction, reversed plantar involvement. The reason for this phenomenon and its clinical course are uncertain, but these new lesions are proving recalcitrant to complementary therapies. Provided the increasingly widespread use of biologic therapies, the incidence and diversity of paradoxical reactions are expected to increase.

Pirfenidone-induced photosensitivity confirmed by pathological phototest.

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is an idiopathic interstitial progressive fibrotic lung disease and the most lethal of all interstitial lung diseases. Pirfenidone is a novel antifibrotic and anti-inflammatory agent which reduces decline in lung function and prolonges progression-free survival. It has a favourable benefit-risk profile and is generally well tolerated. However gastrointestinal events, photosensitivity reactions and rash are the most common adverse events. OBJECTIVE: We report a 71-year-old man with 1 week history of erythematous partially pruritic lesions on both sides of the neck and the back of the hands limited to sun exposed areas. He had been diagnosed with IPF and was being treated with pirfenidone (Esbriet) for 8 months. We suspected a photosensitivity reaction caused by pirfenidone. METHODS: A phototest and a punch biopsy were performed. RESULTS: The phototest had a pathological result. The minimal erythema dose was decreased, presenting with erythema and edema starting from 7 mJ/cm2 of UVB and an aberrant response to UVA starting from 2 J/cm2. Histopathological examination revealed spongiotic acute dermatitis with focal presence of necrotic keratinocytes. The patient was diagnosed with pirfenidone-induced photosensitivity and treated with high potency topical steroid leading to the resolution of the lesions, without the need for discontinuation of the drug. CONCLUSION: To our Knowledge, this is the first pirfenidone-induced photosensitivity reported case confirmed by pathological phototest. Patient education and photoprotection advice are essential for prevention.

Incidence of non-melanoma skin cancer in patients treated with psoralen and ultraviolet A therapy. / Incidencia de cáncer cutáneo distinto del melanoma en pacientes tratados con PUVA oral.

INTRODUCTION: Studies reporting incidences of non-melanoma skin cancer (NMSC) are heterogeneous, depend on the geographic area of the studied population and are often short-term. The aim of this study is to determine the incidence of NMSC in patients treated with oral PUVA therapy in the Mediterranean area. MATERIAL AND METHODS: A retrospective, observational study was carried out with a sample of 234 patients treated with systemic PUVA between 1982 and 1996, carrying out a historical follow-up until May 2017. The incidencedensity rate of CCNM (crude and adjusted) was calculated by direct standardisation. The incidence of CCNM was compared with that reported in the general population in a similar geographical area. RESULTS: 50 neoplasms were diagnosed in 22 patients. The prevalence of CCNM in patients treated with phototherapy was 10.3%. The mean follow-up time was 21 years. The crude-adjusted incidence density rate of CCNM was 554.4-183.9 cases/100,000 treated patients per year. The crude-adjusted incidence density rate of basal cell carcinoma was 352.3-111.2 cases/100.000 patients and of squamous cell carcinoma was 229-77.7 cases /100,000 patients. CONCLUSION: PUVA therapy is associated with an increased risk of CCNM inthe Mediterranean population.