Primary Extramammary Paget's Disease Following Previously Treated Secondary Extramammary Paget's Disease Over the Scrotum: A Rare Case Successfully Treated With Mohs Micrographic Surgery.

Extramammary Paget's disease (EMPD) is a rare cutaneous neoplasm that can be classified as either primary or secondary, depending on the presence or absence of an associated internal malignancy. Primary EMPD arises as an intraepithelial adenocarcinoma, while secondary EMPD results from the extension of an underlying visceral malignancy. This case report presents a unique instance of primary EMPD developing 10 years after a diagnosis of secondary EMPD in the same anatomical location, a phenomenon not previously documented in the literature. The patient, initially treated for secondary EMPD with wide local excision, later developed primary EMPD, as confirmed through histopathological and immunohistochemical analysis. This rare occurrence raises questions about the potential mechanisms, including field cancerization, persistent risk factors, or a coincidental event. The case underscores the importance of long-term follow-up and surveillance for EMPD patients. Mohs micrographic surgery remains the gold standard for treating EMPD due to its high precision in margin control and lower recurrence rates compared to conventional surgical methods. This case highlights the need for meticulous diagnostic approaches and continuous monitoring to manage and understand the complexities of EMPD effectively.

Vitamin C Deficiency Masquerading as Vasculitis in a Patient With Crohn's Disease.

This case study features a 40-year-old male with Crohn's disease (CD) who was initially misdiagnosed with vasculitis but was later shown to have scurvy owing to vitamin C deficiency. The patient's diet was nearly exclusively made up of highly processed fast food, with no fresh fruits or vegetables. A mildly sensitive, violaceous rash on his lower legs, mild gingival hemorrhage and enlargement, and muscle soreness were among his symptoms. Anemia and undetectable vitamin C levels were discovered in laboratory studies. A skin sample revealed follicular hyperkeratosis, coiled hairs, and perifollicular bleeding, eliminating the possibility of vasculitis. Scurvy was confirmed by undetectable vitamin C levels and intramuscular bleeding discovered during a muscle biopsy. After one month of vitamin C administration, the patient's skin was entirely clear. This instance emphasizes the significance of taking vitamin C insufficiency into account in patients with CD and other disorders that can cause malabsorption. Misdiagnosis might result in unneeded treatments and medical expenses. Scurvy must be diagnosed as soon as possible because it might cause gastrointestinal/intracerebral hemorrhage and death.

Adverse cutaneous reaction to hydroxychloroquine in a patient with anti-SAE-1-positive dermatomyositis and a history of diffuse large B-cell lymphoma.

This case report details the cutaneous findings of a patient with a history of diffuse B-cell lymphoma and SAE-1-positive dermatomyositis who developed an adverse cutaneous reaction after initiation of treatment with hydroxychloroquine. This adds to the sparse literature available detailing the correlation between anti-SAE-1 autoantibodies in dermatomyositis and the unique adverse cutaneous reactions in patients taking hydroxychloroquine. Additionally, our patient developed dermatomyositis years after a diagnosis of lymphoma. This report highlights the utility of the myositis-specific antibody panel to guide diagnosis and management, as well as the potential for developing dermatomyositis years after a lymphoma diagnosis.

Cutaneous Intravascular CD-30-Positive Anaplastic Large Cell Lymphoma: A Case Report and Literature Review.

Intravascular cutaneous anaplastic large cell lymphoma (ALCL) is an extremely rare non-Hodgkin lymphoma that proliferates in the lumen of small blood vessels and has a propensity to manifest in the skin. Most reported cases of intravascular lymphoma described in the literature are of large B-cell lymphomas, making T-cell lymphomas incredibly rare. As such, we present the case of an 87-year-old male with primary cutaneous intravascular anaplastic large T-cell lymphoma that initially presented with an erythematous, subcutaneous nodule on the right mid-abdomen. We report the immunohistochemical results showing lymphoma cells staining positively for CD3 and CD30 and lacking expression of anaplastic lymphoma kinase, pan-cytokeratin, CD10, CD20, and SOX10. We also review and compare previously reported cases of intravascular ALCL with primary cutaneous involvement.

Cutaneous carcinosarcoma: a rare diagnosis.

Carcinosarcoma is a rare malignant tumor that is a combination of carcinoma (cancer of epithelial tissue) and sarcoma (cancer of mesenchymal tissue). In practice, it is more common to have either carcinoma or sarcoma individually, but it is novel to have a combination of the two; as a result, few cases have been reported. Carcinosarcoma typically occurs in visceral organs and is rare in the skin. We present the case of a 68-year-old man with a lesion on his left zygomatic cheek that was revealed to be a biphasic malignant neoplasm. Multiple stains provided evidence of both epithelial and mesenchymal components within the lesion. Treatment for such a diagnosis typically involves a combination of wide local excision or plastic surgery for reconstruction purposes, both of which were used with this patient. This case describes the identification of cutaneous carcinosarcoma using immunohistochemical tests and emphasizes the uniqueness of such a diagnosis that requires early attention and appropriate selective treatment.

Cutaneous mastocytoma obscured by allergic contact dermatitis in an infant.

The diagnosis of solitary mastocytoma is usually made clinically, however, atypical presentations may obscure the diagnosis. We present a unique case of solitary cutaneous mastocytoma in an 11-month-old male initially misdiagnosed as atopic dermatitis; the diagnosis was obscured due to the development of an allergic contact dermatitis most likely secondary to topical medications that were being applied to the lesion. The diagnosis of solitary cutaneous mastocytoma is made based on lesion morphology, Darier's sign, and lack of systemic involvement. Most solitary cutaneous mastocytomas resolve before puberty; symptomatic treatment and avoidance of triggers are mainstay therapy.

Drug-induced neutrophilic lobular panniculitis secondary to BRAF and MEK inhibitor used for treatment of low-grade glioma and its management.

An 8-year-old boy presented with his mother for evaluation of an erythematous rash 3 weeks after the start of dual BRAF-MEK inhibition with dabrafenib and trametinib for treatment of progression of low-grade glioma. Panniculitis has been reported as a rare adverse cutaneous event induced by BRAF inhibitors, MEK inhibitors, and the combined dual BRAF-MEK therapy. Based on the patient's history, clinical presentation, and histopathological findings, a diagnosis of drug-induced neutrophilic panniculitis was made. This case describes neutrophilic panniculitis as a potential cutaneous manifestation of dual BRAF-MEK inhibitor therapy and describes the management of such side effects. Neutrophilic panniculitis is a relatively rare manifestation, characterized by neutrophilic inflammation in the subcutaneous tissue. Additionally, this case serves as a reminder to consider the cutaneous side effects of such therapies, given that MEK and BRAF inhibitors are increasingly used to treat primary brain tumors in the pediatric population. Routine inspection and early management may improve patients' quality of life and enable continuation of anticancer therapy.

Acute Onset of Bullous Skin Rash and Acute Kidney Injury after Exposure to Radiocontrast: Sweet's Syndrome.

Sweet's syndrome or acute febrile neutrophilic dermatosis is characterized by an acute inflammatory skin eruption of oedematous and erythematous papules, plaques or nodules, accompanied by fever, and leucocytosis with possible extracutaneous involvement. Aetiologies include infections, inflammatory bowel disease, pregnancy or malignancy, or the syndrome may be drug-induced by many classes of medications or very rarely, radiocontrast exposure. Herein, the authors report a case of radiocontrast-induced bullous Sweet's syndrome and contrast-induced acute kidney injury in a woman in her 60s with a complex medical history. LEARNING POINTS: Patients with Sweet's syndrome (SS) typically present with acute-onset fever, leucocytosis, and erythematous, tender plaques with dense neutrophilic infiltration in the dermis. The condition is classified into three subtypes: classic SS, malignancy-associated SS, and drug-induced SS.Drug-induced SS is characterized by an abrupt onset of a painful erythematous rash, dense neutrophilic dermal infiltrate without vasculitis, a temporal relationship between exposure and onset, and resolution of symptoms after drug discontinuation and/or corticosteroid therapy.Treatment options include systemic corticosteroids as first-line therapy, while colchicine, dapsone, indomethacin, naproxen, clofazimine, ciclosporin, α-interferon, and potassium iodide may be considered as second-line therapies in cases resistant to corticosteroids.

​​Longitudinal Erythronychia Secondary to a Wooden Splinter.

Longitudinal erythronychia (LE) is a term for red streaks in the nail which can be caused by a range of diseases. The specific type of longitudinal erythronychia can correlate with certain associated conditions making it important to properly categorize when discovered. A 71-year-old Hispanic male presented to the clinic with a type 1A LE associated with subungual keratosis that had been asymptomatic for approximately 12 months. The patient denied injury, pain, cold sensitivity, or cosmetic distress. The working diagnosis was squamous cell carcinoma in situ (SCCIS) vs. onychopapilloma or glomus tumor. A 4mm punch biopsy from the distal nail matrix was performed, and dermatopathology revealed that the LE was secondary to a wooden splinter. After a literature review, it was discovered that this is the first confirmed case of LE secondary to a splinter. Future providers should keep splinters as a potential differential diagnosis, especially as they evaluate LE, but ultimately all suspicious type IA lesions should be biopsied to rule out potential insidious pathologies, such as SCCIS and malignant melanoma.

Neurofibroma Within a Nevus Sebaceus: A Case Report.

Nevus sebaceus most commonly presents as a yellow, alopecic plaque on the head or neck in childhood and evolves into a verrucous plaque at puberty. Numerous secondary tumors may arise within nevus sebaceus lesions. Tumors of mesenchymal origin have been rarely documented. We present a unique case of a patient who presented with a nevus sebaceus on the scalp. Excision of the tumor and subsequent histopathology of the lesion revealed a nevus sebaceus with a desmoplastic trichilemmoma, a tumor of follicular infundibulum, and a neurofibroma. This case highlights a rare finding of a mesenchymal tumor, and the first reported neurofibroma, arising in association with a nevus sebaceus.

Adnexal iceberg: pruritic papule on top of a nodule on the back.

Basal cell carcinoma (BCC), one of the most common malignancies, is rarely associated with follicular and apocrine differentiation patterns. This case presents a case of BCC with atypical gross presentation and features of aberrant differentiation. Clinicians should maintain a high level of clinical suspicion for BCC in atypical lesions. Click here for the corresponding questions to this CME article.

A tale of three common nipple diseases.

Paget's disease of the nipple is a rare breast carcinoma of the nipple-areola complex and is often associated with an underlying in situ or invasive carcinoma. It clinically presents as a thickened, erythematous, weeping or crusted lesion with irregular borders. Paget's cells invading the epidermis are the hallmark histopathologic feature, and cytokeratin 7 staining is positive in nearly all cases. However, the diagnosis of Paget's disease may be delayed or missed in favor of clinically similar benign nipple processes such as nipple adenoma and nipple dermatitis. We present a case of Paget's disease of the nipple in a 73-year-old woman with a brief review of other common nipple diseases.

The clinical evolution of lichen planus pemphigoides.

Lichen planus pemphigoides (LPP) is a rare autoimmune subepidermal blistering disease characterized by lichenoid and bullous lesions. LPP is generally thought to be idiopathic, possibly related to medication or malignancy, or potentially the result of long-standing lichenoid inflammation damaging the basement membrane zone leading to epitope spreading. The histological appearance of lichenoid and bullous lesions in LPP resembles findings of lichen planus and bullous pemphigoid, respectively. We present a case of LPP in a 64-year-old woman with a brief historical review of the establishment of LPP as a separate disease entity and a discussion of similarities and differences of LPP with other lichenoid and blistering dermatoses.

Multiple painful plaques and the Sweet's syndrome.

We present a rare case of Sweet's syndrome. A 39-year-old woman with subjective fevers, polyarthralgia, and malaise presented with worsening painful erythematous plaques on the trunk, arms, and legs. Further examination with biopsy revealed a diagnosis of acute febrile neutrophilic dermatosis, or Sweet's syndrome. Diagnosis by skin biopsy is crucial, and onset requires prompt evaluation for serious associated disorders such as leukemias, inflammatory bowel disease, thyroid disease, sarcoidosis, and infectious etiologies. In general, symptoms and cutaneous manifestations of Sweet syndrome respond rapidly to treatment with systemic corticosteroids or potassium iodide.

Basal cell carcinoma arising within a longstanding hemangioma.

Basal cell carcinomas have been reported to arise within several different tumors. Without careful attention to detail, basal cell carcinomas may go unnoticed when presenting within or directly adjacent to another distinct tumor. The increased use and accessibility of dermoscopy has allowed dermatologists to detect skin cancers that might otherwise be undetected by the naked eye. This case report details a collision, detected by dermoscopy, of a basal cell carcinoma developing within a long-standing hemangioma (cherry angioma) on the left lower eyelid of a 75-year-old man.

Familial cold autoinflammatory syndrome with rheumatoid arthritis.

Familial cold autoinflammatory syndrome (FCAS) is a cryopyrin-associated periodic syndrome that presents with episodic fever, skin rash, and joint pain after exposure to cold temperatures. Although the diagnosis is often singular, there are several instances of concurrent underlying autoimmune pathologies with either rheumatoid arthritis (RA) or amyloidosis. Because symptoms of the two entities overlap, it can be difficult to address a potential dual diagnosis of FCAS and an autoimmune disorder. We found seven previously reported cases of FCAS and amyloidosis and five cases of FCAS and RA and present another case of an FCAS-RA dual diagnosis.