Increased Doses of Adalimumab are Associated With Clinical Improvement of Hidradenitis Suppurativa.

BACKGROUND: TNF-inhibitor adalimumab 40 mg/week (ADA40) is the only approved treatment for hidradenitis suppurativa (HS); however, it is not uniformly effective. Despite a high prevalence of comorbid obesity in HS patients, adalimumab dosing is not weight-based, unlike other TNF-inhibitors. OBJECTIVE: To evaluate the effectiveness of adalimumab 80 mg/week (ADA80) compared with ADA40 in overweight and obese patients with moderate to severe HS. METHODS: We conducted a dual-center retrospective chart review of HS patients treated with ADA80 between August 2016 and December 2021. We collected data on demographics, comorbidities, treatments, and disease severity that are presented as descriptive statistics and compared with Wilcoxon signed-rank test. RESULTS: Eight patients with median body mass index of 36.6 (IQR 32.5–40.7) and no improvements in HS severity on ADA40 were prescribed ADA80. Patients experienced improved HS-Physician Global Assessment (ADA40: median 3.0 (3.0-3.8); ADA80: (2.0 (1.8, 2.0)) (P=0.01)), all 5 patients who had lesion counts documented achieved HS Clinical Response, and all 8 patients reported symptom improvements. CONCLUSIONS: Increased adalimumab dose may be associated with improved outcomes for overweight and obese patients with moderate to severe HS. J Drugs Dermatol. 2023;22(6) doi:10.36849/JDD.6868.

Thymoma-associated multiorgan autoimmunity: a graft-versus-host-like disease.

Graft-versus-host disease (GVHD) is a T cell-mediated disease seen most commonly after hematopoietic stem cell transplantation. Rarely, a GVHD-like disease can be seen in patients with malignant thymoma. We describe a 50-year-old man with malignant thymoma who developed skin, liver, and intestinal manifestations similar to that seen in GVHD. We also review other reported cases of GVHD-like manifestations in the setting of thymoma and propose "thymoma-associated multiorgan autoimmunity" as a name for this novel disease. Specifically, thymoma-associated multiorgan autoimmunity is defined as a disease of the liver, intestine, or skin, which on histopathology resembles GVHD but is seen in the setting of malignant thymoma and not after hematopoietic stem cell transplantation.

Muir-Torre syndrome.

A 65-year-old man with a history of multiple neoplastic and pre-neoplastic gastrointestinal lesions as well as urogenital carcinoma presented for evaluation of two asymptomatic skin-colored papules in the head and neck region. Biopsy revealed sebaceous neoplasms and immunohistochemical staining was negative for the presence of hMSH-2 protein in both specimens. These findings were consistent with a diagnosis of Muir-Torre syndrome in the setting of a prior history of visceral malignancies. Muir-Torre Syndrome is a rare autosomal dominant genodermatosis associated with mutations in mismatch repair proteins, hMSH-2 and hMLH-1, which predispose affected patients to visceral malignancies as well as sebaceous gland neoplasms.

Metastatic basal cell carcinoma: a case report and literature review. How accurate is our incidence data?

A 55-year-old man presented to Skin Cancer Surgery Center of Sacramento in 1995 for Mohs micrographic surgery of a 1.5-cm nodular basal cell carcinoma located on the right superior antihelix and scaphoid fossa. The tumor was excised by Mohs micrographic surgery. A recurrent basal cell carcinoma developed at the same site 5 years later and was treated also by Mohs micrographic surgery. One year later (approximately 6 years after the diagnosis of the initial basal cell carcinoma), a right parotid mass was noted on routine physical exam. CT scan and fine needle aspiration of the mass revealed metastatic basal cell carcinoma. The patient underwent excision of the parotid mass followed by radiation therapy. He has done well for the past 2 years since the diagnosis of his metastatic lesion. Basal cell carcinoma is the most common malignancy in the world. The metastatic potential of this tumor has been a cause for concern to many of our patients. We report a case of basal cell carcinoma metastatic to the parotid gland and critically review the incidence data reported in the literature. We recommended the collection of more current and accurate incidence data for basal cell carcinoma and metastatic basal cell carcinoma.

Chondroid syringoma: case report and review of the literature.

An 84-year-old man presented with an enlarging bluish, painless subcutaneous nodule on the glabella. The lesion had been excised 4 years prior and was diagnosed as chondroid syringoma, but had gradually regrown. The recurrent lesion was treated by surgical re-excision. Histopathological examination was again consistent with chondroid syringoma, and showed the following: 1) a chondroid matrix, 2) tubuloalveolar structures lined by a double epithelium, 3) ductal structures lined by a single epithelium, 4) nests of polygonal cells, and 5) the presence of keratinous cysts. Chondroid syringoma is a rare mixed tumor of the skin that was first described by Hirsch and Helwig. Characteristically, it is composed of a proliferation of epithelial cells set in a myxoid and chondroid matrix. Although chondroid syringomas are predominantly benign, malignant forms have been reported.