Bullous systemic lupus erythematosus (BSLE) is a rare cutaneous complication of systemic lupus erythematosus (SLE). It is a heterogeneous disease that is caused by autoantibodies to the dermoepidermal junction, mainly type VII collagen. Similarities in histology and immunopathology exist between BSLE and other primary bullous dermatoses, namely dermatitis herpetiformis (DH) and epidermolysis bullosa acquisita (EBA), respectively. EBA and BSLE commonly share the same autoantibody to type VII collagen and heterogeneous clinical presentations, creating a diagnostic challenge. However, clinical presentation combined with histology, immunological testing, and concomitant diagnosis of SLE distinguish this entity from other similar dermatoses. Diagnosis of this disease is important given its coexistence with SLE and its many complications. New developments in IgG subtyping have shown subtle variations in IgG subtypes between EBA and BSLE. In addition, rituximab was recently found to be efficacious in recalcitrant cases of BSLE that do not respond to dapsone and immunosuppressants. We review the topic of BSLE with emphasis on clinical, histologic, and immunopathologic features, as well as new methods of diagnosis and treatment.

Drug-induced hypersensitivity syndrome/drug reaction with eosinophilia and systemic symptoms (DiHS/DRESS) is a potentially fatal multi-organ inflammatory disease associated with herpesvirus reactivation and subsequent onset of autoimmune diseases 1 – 4 . Pathophysiology remains elusive and therapeutic options are limited. Cases refractory to corticosteroid therapy pose a clinical challenge 1 , 5 , and approximately 30% of DiHS/DRESS patients develop complications including infections and inflammatory/autoimmune diseases 1 , 2 , 5 . Progress in single-cell RNA sequencing (scRNAseq) provides an opportunity to dissect human disease pathophysiology at unprecedented resolutions 6 , particularly in diseases lacking animal models, such as DiHS/DRESS. We performed scRNAseq on skin and blood from a refractory DiHS/DRESS case, found JAK-STAT signaling pathway as potentially targetable, and further identified that central memory CD4 + T cells were enriched with HHV6b DNA. Intervention via tofacitinib enabled disease control and tapering of other immunosuppressive agents. Furthermore, tofacitinib, as well as anti-viral agents, suppressed culprit-induced T cell proliferation in vitro , identifying the JAK-STAT pathway and herpesviruses as potential therapeutic targets in DiHS/DRESS. Thus, scRNAseq analyses guided therapeutic decisions that enabled successful therapeutic intervention in this refractory DiHS/DRESS case. Employing scRNAseq analyses in human diseases may facilitate our understanding of complicated disease pathophysiology and further provide an alternative approach in personalized medicine.

No abstract

Dermal fibroblasts are mesenchymal cells found between the skin epidermis and subcutaneous tissue. They are primarily responsible for synthesizing collagen and glycosaminoglycans; components of extracellular matrix supporting the structural integrity of the skin. Dermal fibroblasts play a pivotal role in cutaneous wound healing and skin repair. Preclinical studies suggest wider applications of dermal fibroblasts ranging from skin based indications to non-skin tissue regeneration in tendon repair. One clinical application for autologous dermal fibroblasts has been approved by the Food and Drug Administration (FDA) while others are in preclinical development or various stages of regulatory approval. In this context, we outline the role of fibroblasts in wound healing and discuss recent advances and the current development pipeline for cellular therapies using autologous dermal fibroblasts. The microanatomic and phenotypic differences of fibroblasts occupying particular locations within the skin are reviewed, emphasizing the therapeutic relevance of attributes exhibited by subpopulations of fibroblasts. Special focus is provided to fibroblast characteristics that define regional differences in skin, including the thick and hairless skin of the palms and soles as compared to hair-bearing skin. This regional specificity and functional identity of fibroblasts provides another platform for developing regional skin applications such as the induction of hair follicles in bald scalp or alteration of the phenotype of stump skin in amputees to better support their prosthetic devices.

IVIG may be an effective adjuvant in the treatment of PG and has an acceptable side-effect profile. Randomized, placebo-controlled, double-blinded trials are needed to confirm this hypothesis.

No abstract

Telemedicine in the U.S. military has provided valuable dermatology support to providers in remote locations by delivering appropriate and timely consultation for military service members and coalition partners. In addition to avoiding unnecessary medical evacuations, the program facilitated appropriate evacuations that may otherwise have been delayed.

Psoriasis is a chronic immune-mediated dermatologic disorder affecting approximately 2% of the general population. Under current U.S. Army regulation, the diagnosis of psoriasis is a bar to enlistment or appointment and, if poorly controlled, is grounds for referral to a Medical Evaluation Board and potential discharge from military service, according to Army Regulation 40-501. However, between 2008 and 2015, over 3,600 soldiers sought care for psoriasis while deployed to combat theaters, indicating that psoriasis remains a significant medical concern in the U.S. military. Although mild psoriasis is treatable with topical agents, moderate to severe psoriasis may require systemic treatment. Prior to Food and Drug Administration approval of apremilast (Otezla) in 2014, the standard systemic treatment options were methotrexate and biologic agents such as anti-Tumor Necrosis Factor-alpha medications. Active use of methotrexate or biologic immunomodulatory medications automatically disqualifies soldiers from deployment due to monitoring requirements, the need for refrigeration, and the risk for immune compromise. Apremilast offers treatment efficacy similar to that of methotrexate, and it may be taken while deployed because it does not require monitoring or refrigeration. However, efficacy must be evaluated in the context of its much higher unit cost. Nonetheless, we argue that apremilast may be a useful treatment option for psoriasis in a select group of soldiers who must deploy despite suffering from moderate to severe psoriasis.