We report a case of aggressive amelanotic melanoma in a patient with genetically confirmed oculocutaneous albinism type 1A. The tumor showed poor response to targeted and immune therapies, and RNA-seq analysis suggested a link between pigment loss and PD-L1 upregulation.

Generalized pustular psoriasis (GPP) is a rare, potentially life-threatening inflammatory disease characterized by neutrophilic pustules and systemic inflammation. We report a case of severe GPP triggered by SARS-CoV-2 infection in a 46-year-old woman with a long history of psoriasis. Eleven days after recovery from COVID-19 pneumonia, she developed widespread pustules and fever. Histopathology revealed subcorneal spongiform pustules and dermal neutrophilic infiltration consistent with GPP. Systemic corticosteroids followed by etretinate and deucravacitinib achieved complete remission. A literature review identified 11 infection- and 10 vaccine-related GPP cases. Compared with vaccine-associated cases, infection-related flares showed longer latency and higher corticosteroid use. Mechanistically, both SARS-CoV-2 infection and vaccination may be associated with IL-36 axis activation, potentially via spike protein-driven, Toll-like receptor-mediated innate immune signaling. This case highlights that distinct immune kinetics may underlie infection- and vaccine-related GPP, while supporting a putative role of IL-36-driven inflammation in COVID-19-associated disease exacerbation.

Extranodal natural killer (NK)/T-cell lymphoma, nasal type (ENKTCL), is a rare and aggressive Epstein-Barr virus (EBV)-associated malignancy, typically affecting middle-aged individuals. Cases in patients over 80 are extremely rare. We report an 88-year-old woman, the oldest known case, successfully treated with radiotherapy alone. She presented with erythema and swelling of the right cheek and nasal cavity, initially misdiagnosed as facial cellulitis. A skin biopsy revealed atypical CD3⁺/CD56⁺ lymphoid cells, and EBER positivity confirmed EBV involvement. Imaging showed localized disease without systemic spread. Due to her advanced age and comorbidities, chemotherapy was not feasible, and she received radiotherapy alone, resulting in complete remission without recurrence. This case highlights the effectiveness of radiotherapy in localized ENKTCL and suggests that even very elderly patients can achieve favorable outcomes with appropriate treatment.

Subcutaneous fat necrosis of the newborn (SCFN) is a rare panniculitis that typically presents within the first few weeks of life. We report a unique case of SCFN diagnosed as early as day three of life in a large-for-gestational-age neonate born to a diabetic mother. Notably, the lesion exhibited a central purpuric area with an irregular, serrated erythematous border, which was clearly visualized using dermoscopy. Histological confirmation was limited due to sampling constraints, but the clinical features and evolution were consistent with SCFN. This case highlights the utility of early dermatologic assessment and dermoscopy in identifying SCFN with atypical hemorrhagic features, expanding the clinical spectrum of this condition.

A 78-year-old woman with a history of follicular lymphoma treated with obinutuzumab and bendamustine developed Merkel cell carcinoma (MCC) on her left forearm. After surgical excision, in-transit and lymph node metastases appeared within three weeks. Avelumab and radiotherapy were initiated, but severe neutropenia occurred after two doses, requiring G-CSF support and treatment discontinuation. Despite early cessation, metastatic lesions regressed. This case suggests that prior B-cell-directed therapy may increase the risk of hematologic toxicity with immune checkpoint inhibitors and that even short-term avelumab may induce a durable response in MCC.

BackgroundAlopecia areata (AA) is a chronic immune-mediated disorder causing non-scarring hair loss. Severe forms like alopecia totalis (AT) and alopecia universalis (AU) pose therapeutic difficult situations. Topical immunotherapy with squaric acid dibutylester (SADBE) and diphenylcyclopropenone (DPCP) is widely used but has variable efficacy.ObjectivesTo evaluate the efficacy of topical immunotherapy in severe AA, identify factors influencing outcomes, and assess adjunctive antihistamine therapy.MethodsA retrospective analysis of 106 severe AA cases (26 AT, 80 AU) among 1,098 patients diagnosed between 2007 and 2016. Treatment efficacy was assessed using the Alopecia Areata Investigational Assessment Guidelines (AAIAG), and statistical analyses included chi-square tests and Kaplan-Meier analysis.ResultsAmong the 106 patients included in this study, 43% exhibited excellent or good responses to topical immunotherapy, while 75% experienced at least partial hair regrowth. Patients with alopecia totalis demonstrated slightly better outcomes than those with alopecia universalis (50% vs. 40% achieving excellent or good responses). Atopic dermatitis was significantly associated with lower treatment efficacy (54% vs. 80%, p = 0.0157). Although antihistamine use showed a trend toward improved responses (78% vs. 38%), the difference did not reach statistical significance (p = 0.0991), and multivariate analysis did not confirm its efficacy (p = 0.649). Hair regrowth was observed within 4 months in 90% of cases, while peak therapeutic effects were achieved within 3 years. Long-term treatment adherence correlated with improved outcomes, highlighting the importance of sustained therapy.ConclusionTopical immunotherapy remains an effective treatment for severe alopecia areata, although response rates vary among patients. Individualized treatment approaches, including prolonged therapy and consideration of patient-specific factors, are essential for optimizing clinical outcomes.

PURPOSEMalignant apocrine and eccrine tumors (MAETs) are extremely rare cutaneous adnexal malignancies, accounting for only 0.005%-0.01% of all skin tumors. These tumors are highly metastatic, and evidence regarding optimal chemotherapy and prognostic outcomes remains limited. This study aimed to evaluate the efficacy of systemic chemotherapy and overall prognosis in Japanese patients with unresectable MAETs.PATIENTS AND METHODSWe conducted a retrospective, multicenter study involving 81 patients with unresectable MAETs treated at 27 institutions across Japan. Patients received one of three primary chemotherapy regimens: platinum-based (cisplatin or carboplatin), taxane-based (docetaxel or paclitaxel), or TS-1 (tegafur/gimeracil/oteracil). Patient demographics, objective response rates (ORRs), overall survival (OS), and progression-free survival were assessed. Survival curves were estimated using the Kaplan-Meier method.RESULTSThe estimated ORRs for the platinum-based, taxane-based, and TS-1 groups were 37.0%, 25.0%, and 22.2%, respectively, with no statistically significant differences among them (P = .527). The median OS and 5-year OS rate for the entire cohort were 29.0 months and 34.0%, respectively. The median OS and 5-year OS rates by regimen were as follows: platinum-based, 29.0 months and 36.2%; taxane-based, 22.0 months and 39.7%; and TS-1, 30.0 months and 13.9%, with no significant differences observed (P = .907). In addition, there were no significant differences in ORR or OS between patients receiving combined chemoradiotherapy and those receiving chemotherapy alone.CONCLUSIONNo single chemotherapeutic regimen demonstrated superior efficacy in patients with unresectable MAETs. These findings highlight the need for further investigations using larger, prospective cohorts and multidisciplinary approaches to establish optimal therapeutic strategies for this rare malignancy.

Extramammary Paget disease (EMPD) is a rare skin cancer with an estimated incidence rate of 0.13 per 100 000 population/year in Caucasians and 0.28 in Asians. Although distant metastases have been reported in 10%-20% of EMPD cases, standardized systemic chemotherapy has not been established. Prospective clinical trials are essential to establish standard treatments for advanced EMPD. Therefore, this retrospective study examined a substantial number of patients with EMPD to assess the efficacy of systemic chemotherapy. This study included 164 patients with advanced EMPD who underwent treatment at 16 Japanese institutions. Treatment efficacy was evaluated in a cohort of 138 patients, after excluding 26 patients without lesions outside the radical irradiation field from the 164 patients. The efficacy of each treatment was evaluated by determining the objective response rate (ORR), progression-free survival (PFS), and overall survival (OS) using Kaplan-Meier analysis. Multivariate analysis was performed to account for potential confounding factors, such as age, sex, and performance status. The patients received the following treatments: docetaxel hydrate (DOC) (65.9%); tegafur/gimeracil/oteracil potassium, DOC (S-1/DOC) (9.8%); fluorouracil and cisplatin (FP) (15.9%); and other drugs (8.5%). DOC is the most commonly used in Japan. The ORRs in the DOC, S-1/DOC, and FP groups were 51.6%, 78.6%, and 27.8%, respectively. Logistic regression analysis revealed that, compared with the DOC group, the odds ratio for the ORR of the S-1/DOC group was 3.29 (95% CI: 1.49-7.25, p = 0.003). However, no significant differences in OS or PFS were observed between the treatment groups (p = 0.122 and p = 0.422, respectively). This study provides valuable information on EMPD and may serve as a useful historical control for the future evaluation of new treatments for EMPD.

Primary cutaneous anaplastic large cell lymphoma (pcALCL) is a CD30-positive cutaneous T-cell lymphoma with a generally favorable prognosis. While radiotherapy is effective, recurrence is not uncommon. Herein, we report the case of an 87-year-old man with recurrent pcALCL who achieved complete remission after bexarotene treatment, despite resistance to radiotherapy and topical steroids. This case highlights bexarotene as a promising therapeutic option for refractory pcALCL. Our experience reinforces the potential of bexarotene as a viable treatment for pcALCL, particularly in recurrent or refractory cases where conventional therapies are contraindicated or ineffective.

Cutaneous nocardiosis is an uncommon but clinically significant opportunistic infection, primarily affecting immunocompromised individuals, including those with underlying malignancies, organ transplants, or chronic corticosteroid use. Although it can also occur in elderly patients, this is often due to age-associated immunosenescence or comorbid conditions that impair immune function. We describe a case of lymphocutaneous Nocardia brasiliensis infection in an 87-year-old man with no history of trauma or corticosteroid use. The patient had been receiving long-term low-dose methotrexate (MTX) for rheumatoid arthritis. He presented with painful, erythematous nodules arranged along the lymphatic vessels of the right forearm. Histopathological analysis and culture confirmed the diagnosis. Oral trimethoprim-sulfamethoxazole (TMP-SMX) and minocycline were initiated; minocycline was discontinued after three weeks due to gastrointestinal symptoms. MTX was also stopped in response to clinical progression. The lesions resolved completely with continued TMP-SMX monotherapy over a three-month course. A brief review of the literature revealed only a few reported cases of cutaneous nocardiosis in individuals over 85 years of age. Some of these lacked clear immunosuppressive backgrounds or trauma history. While TMP-SMX remains the standard therapy, its use in older adults may be constrained by tolerability. This case serves as a reminder that Nocardia infection should be considered in the differential diagnosis of nodular skin lesions in very elderly patients, even in the absence of typical risk factors.

Condyloma acuminatum (CA) is thought to be a sexually transmitted disease caused by human papillomavirus, most commonly types 6 and 11. Pediatric cases are relatively rare, and the routes of transmission remain controversial. We report two cases of refractory pediatric CA involving the perianal and genital areas. Both cases were resistant to topical therapies, including imiquimod and cryotherapy, and eventually required multiple surgical interventions. Neither case presented any clinical or historical evidence suggestive of sexual abuse, and the mode of transmission remained unclear.

An 84-year-old female experienced progressive erythema on her limbs and chest over the past year. Initially managed with topical steroids, the erythema eventually spread throughout her body, forming erosions. A biopsy confirmed the diagnosis of mycosis fungoides (MF) (Stage IIB, T2bN0M0B0). Treatment with oral bexarotene (300 mg/day) and narrow-band UVB therapy showed limited improvement. Electron beam therapy (30 Gy in 10 fractions) applied to facial and plantar tumors resulted in a reduction of the tumors. This case highlights the treatment of tumors of MF on the face showing the effectiveness of combining electron beam therapy with bexarotene.

Malignant melanoma is a melanocyte-derived tumor known for its aggressive clinical behavior. Melanocytes originate from the neural crest, which also gives rise to neural tissues. Malignant melanoma can occasionally exhibit neural differentiation. We report a case of a 70-year-old male with malignant melanoma exhibiting neural marker positivity in the absence of typical melanoma markers. The patient initially presented with a dark nodule on his left heel, which was confirmed as malignant melanoma through cytology. Surgical resection and lymph node dissection were performed, revealing atypical melanocytes. Despite postoperative nivolumab treatment, metastases in the brain and lungs were observed. Histological examination of the brain tumor showed neural differentiation markers (thyroid transcription factor 1 (TTF-1), cytokeratin 7 (CK7), AE1/AE3, and epidermal growth factor receptor (EGFR)) with negative melanoma markers. The patient eventually succumbed to the disease despite multiple treatments. An autopsy revealed multiple organ tumors (brain, duodenum, stomach, liver, and bile duct) negative for melanoma markers but positive for neuroendocrine markers (CD56, synaptophysin, and chromogranin A). This case suggests two possibilities: the coexistence of malignant melanoma with neuroendocrine tumors or a transformation of melanoma into a neuroendocrine phenotype. This case highlights the need for clinicians to consider the potential for melanoma to lose typical markers and transform into neuroendocrine cancer.

We present the case of a 71-year-old man who, after undergoing postoperative radiotherapy for epithelial carcinoma, developed progressively enlarging erythema. Initially, the condition resembled radiation dermatitis or erysipelas, and topical steroids and antibacterial agents were administered without success. A biopsy was performed for further evaluation, revealing a cutaneous invasion of parotid carcinoma. The lesion continued to enlarge, leading to dysphagia and ultimately necessitating a tracheostomy.

Dermatofibrosarcoma protuberans (DFSP) is a rare and infiltrative soft tissue tumor. Our report details a distinctive case of DFSP with pan-TRK positivity in the right nasal dorsum of a 46-year-old female. Histological analysis identified NTRK fusion gene involvement in this patient, detectable through pan-TRK immunostaining. The case underscores the significance of comprehensive management for pan-TRK-positive DFSP in challenging facial locations, indicating the potential efficacy of TRK inhibitors.

Myoepithelial carcinoma is an exceedingly rare malignancy, particularly when originating from the skin. It frequently arises from malignant transformations of pleomorphic adenomas in various locations such as the parotid gland, breast, soft tissues, and lungs. Primary cutaneous myoepithelial carcinoma is exceptionally rare, often leading to delayed diagnosis. We report a case of giant primary cutaneous myoepithelial carcinoma of the left thigh, initially misdiagnosed as squamous cell carcinoma (SCC). The patient, a 64year-old male, presented with a rapidly enlarging, ulcerated, and necrotic skin lesion. The initial presentation mimicked SCC. Due to the large tumor size and anemia caused by the tumor, the patient underwent a reduced-dose chemotherapy regimen (cytarabine plus aclarubicin chemotherapy) to shrink the tumor, enabling successful local surgical resection. Post-surgery, the patient received radiotherapy and tegafur gimeracil oteracil potassium, resulting in disease control without progression for two years. This case highlights the diagnostic challenges of myoepithelial carcinoma, which can mimic SCC among numerous other tumors. Accurate diagnosis relies on immunohistochemical staining and careful pathological evaluation. The case underscores the importance of considering myoepithelial carcinoma in the differential diagnosis of ulcerative tumors.

Mycosis fungoides (MF) is a type of primary cutaneous T-cell lymphoma. The anti-cluster of differentiation (CD) 30 antibody agent, brentuximab vedotin (BV), has recently been developed for specific targets against CD30-expressed tumor cells with high efficacy against various lymphomas. Herein, we present a case of marginally CD30-expressed MF successfully treated with BV rechallenge.