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Dermatology Online Journal

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About

Dermatology Online Journal is an open-access, refereed publication intended to meet reference and education needs of the international dermatology community since 1995. Dermatology Online Journal is supported by the Department of Dermatology UC Davis, and by the Northern California Veterans Administration.

Volume 28, Issue 4, 2022

Review

Treatment modalities in brachioradial pruritis: a systematic review

Introduction: Brachioradial pruritis is a rare dysesthesia syndrome that is known to negatively impact quality of life. No consensus exists regarding optimal treatment strategies. Methods: We searched MEDLINE, EMBASE, and the Cochrane Collaboration Clinical Trials Registry from 1966 to 2021 for studies using the title word "brachioradial pruritis" with no language restriction. One author (A.Z.) screened and performed full article reviews of all randomized clinical trials, cohort studies, case-control studies, case reports, and case series describing treatment outcomes among patients with brachioradial pruritis. Results: We identified 239 potential articles with a final set of 45 articles meeting inclusion criteria. Only a single randomized clinical trial was identified, finding no significant benefit of topical capsaicin cream. Treatment modalities with the greatest number of reported successful therapeutic trials include gabapentin and tricyclic antidepressants. In patients with confirmed cervical spine disease, spine-directed therapies such as epidural injections were found to be beneficial. Case reports and small case series describing less-common treatments were also identified. Discussion: The literature is overall limited with the greatest support for gabapentin, pregabalin, tricyclic antidepressants, and spine-directed therapies in appropriate patients with brachioradial pruritis. Future randomized clinical trials are needed to compare the relative effectiveness of available treatments.

Original

Characteristics of non-melanoma skin cancers in Native American patients treated with Mohs micrographic surgery

Despite the lower incidence of non-melanoma skin cancers in skin of color populations, greater morbidity and mortality have been reported. Literature describing non-melanoma skin cancers in Native Americans is scarce. We designed a retrospective review study aimed to evaluate the characteristics of non-melanoma skin cancers (basal cell carcinoma and squamous cell carcinoma) in Native American patients treated with Mohs micrographic surgery between January 2015 and August 2020, at a single academic center. Twenty-six patients with 28 tumors were identified; 12 squamous cell carcinomas (92% well-differentiated) and 16 basal cell carcinomas (94% nodular). Most tumors were on the head and neck, with mean size of 563mm2 (squamous cell carcinomas) and 350mm2 (basal cell carcinomas). Tumor clearance was achieved in one stage for 75% of tumors. Recurrence was seen in two patients with squamous cell carcinoma. No mortality reported, although follow up was limited. Few Native Americans patients underwent Mohs micrographic surgery for non-melanoma skin cancers. Squamous cell cancers were larger, lower risk while basal cell carcinomas were predominantly nodular. Average time from biopsy to Mohs micrographic surgery was three months. Further studies are needed to better characterize non-melanoma skin cancers in Native Americans and to identify barriers to prompt care.

Case Report

A unique case of subcutaneous panniculitis-like T-cell lymphoma presenting as an abscess following an arthropod bite

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare form of low-grade cutaneous lymphoma that usually presents with multifocal non-tender subcutaneous nodules over the trunk and extremities. We present an exceptional case of SPTCL masquerading as a solitary abscess which developed following an antecedent arthropod bite. Unique histological features encountered include foci of neutrophilic aggregation and admixed eosinophils within the neoplastic lymphomatous subcuticular infiltrate. As SPTCL rarely presents as an abscess, the authors wish to highlight this diagnostic pitfall and suggest excluding SPTCL as a cause of pseudo-pyoderma in patients who are afebrile with a discordant inflammatory marker profile. In addition, this condition should be suspected in non-diabetic patients who experience a rapid clinical course with suppuration and demonstrate a poor response to appropriate antibiotics. As such, we recommend sending tissue for histopathological examination for patients with atypical presentations.

Noninflammatory presentation of cutaneous breast cancer: a retrospective case series at a single academic institution with review of the literature

Breast cancer with skin involvement is an uncommon clinical presentation of this malignancy. Breast cancer overall has a relatively high mortality rate and wide variety of presentations, making skin involvement by breast cancer a necessary consideration in the differential diagnosis for many types of breast lesions. A retrospective review of our own small academic dermatology outpatient clinic, between August 2006 and January 2020, found four cases of noninflammatory breast cancer with skin involvement diagnosed through biopsy by our dermatologists. This review was approved by the local Institutional Review Board. Of the four patients identified, three were female and one was male. One patient had prior history of invasive ductal carcinoma in remission before recurrence was diagnosed. Another patient had a history of melanoma in situ before diagnosis with breast cancer. Patients were treated with various combinations of surgery, radiation, and hormone therapy. These four cases are presented here in detail, which emphasize the role of the dermatologist in recognizing various cutaneous manifestations of noninflammatory breast cancer in order to make a timely diagnosis.

Anti-laminin 332 mucous membrane pemphigoid in a young woman treated with rituximab

Mucous membrane pemphigoid, formerly known as cicatricial pemphigoid, is a rare and difficult-to-treat bullous disorder that occurs most commonly in older adults. We describe a 32-year-old woman who was diagnosed with anti-laminin 332 mucous membrane pemphigoid through indirect immunofluorescence for laminin 332 following nonspecific histologic and direct immunofluorescence findings. At 16 weeks following completion of her first cycle of with rituximab 375mg/m2 weekly for four weeks, her mucosal erosions had resolved. Although not widely available, this case highlights the utility of anti-laminin 332 immunofluorescence for diagnostic confirmation of this entity and the efficacy of rituximab in obtaining disease control.

Pityriasis rubra pilaris heralding diagnosis of urothelial carcinoma: a case report

Pityriasis rubra pilaris is a papulosquamous inflammatory dermatosis that can be associated with HIV, autoimmunity, infections, certain medications, and neoplasms. Paraneoplastic pityriasis rubra pilaris has previously been reported in association with solid organ malignancies and once with leukemia. Herein, we present an elderly man with paraneoplastic pityriasis rubra pilaris, heralding the diagnosis of low-grade papillary urothelial carcinoma. Our patient's pityriasis rubra pilaris resolved after surgical resection of the tumor.

Werner syndrome associated with acroosteolysis

Werner syndrome (WS) is an autosomal recessive syndrome characterized by genomic instability that affects multiple body systems. The characteristic features of the disease include growth retardation, short stature, alopecia, scleroderma, atrophic skin with ulcerations, infertility, cataracts, premature arteriolosclerosis, diabetes, osteoporosis, and increased risk of malignancies. Werner syndrome protein (WRN) protein deficiency in this disease causes changes in gene expression, similar to those observed in normal aging. As the median age of death in WS is the fourth or fifth decade of life, early diagnosis leads to a better screening opportunity for malignancies. Herein, we present a 28-year-old woman who presented with growth arrest, dyspigmentation, and acroosteolysis and was later diagnosed with Werner syndrome.

Annular elastolytic giant cell granuloma in a woman with metabolic syndrome

Annular elastolytic giant cell granuloma (AEGCG) is a rare granulomatous skin condition. It belongs to a group of skin and elastic fiber disorders. When it affects sun-exposed skin, it is also called actinic granuloma. The etiology and pathogenesis are still debated. However, sun-induced actinic damage to elastic fibers is acknowledged as the primary triggering factor, though the pathogenesis of instances in sun-covered areas is unknown. The most commonly linked systemic illness is diabetes mellitus. Different case reports show an association of this disease with hematological conditions, infections, sarcoidosis, and protoporphyria. Multisystemic involvement was also reported in a case. The disease is clinically recognized by erythematous non-scaly annular patches and plaques with raised borders and hypopigmented or skin-colored centers, sometimes atrophic. It is usually asymptomatic or mildly itchy. The presence of an inflammatory infiltration with non-palisading granulomas, multinucleate large cells, elastin degradation, and elastophagocytosis, as well as the absence of necrobiosis and mucin, are histopathological characteristics. We report a 5-year history of annular elastolytic giant cell granuloma in a 66-year-old woman with a history of type two diabetes mellitus, hypertension, and fatty liver disease (steatosis). She presented with asymptomatic polymorphic erythematous skin lesions mainly in sun-exposed areas.

Case Presentation

Blastic plasmacytoid dendritic cell neoplasm presenting as violaceous forehead plaque

A 72-year-old man with a history of squamous cell carcinoma presented to the Portland VA with forehead discoloration. He was initially diagnosed with actinic damage and prescribed topical treatment. However, he returned to clinic months later with a large, violaceous forehead plaque. Upon biopsy, he was diagnosed with blastic plasmacytoid dendritic cell neoplasm (BPDCN), a rare hematological malignancy. This case report illustrates the importance of keeping BPDCN in the differential diagnosis for ecchymotic plaques that fail to respond to first line therapy.

Angioimmunoblastic T-cell lymphoma with elevated serum IgA and infiltration of IgA-positive plasma cells into a skin lesion

Angioimmunoblastic T-cell lymphoma (AITL) is one of the most common types of peripheral T-cell lymphoma. Laboratory examination exhibits immunological abnormalities, such as polyclonal hypergammaglobulinemia and hemolytic anemia. Skin lesions are also observed in approximately half of AITL cases. However, the relationship of skin involvement with the clinical course and prognosis is unknown. Herein, we report the case of a patient with AITL with elevated serum immunoglobulin A (IgA) level, which was a predictive element of poor prognosis, and infiltration of IgA-positive plasma cells into the skin lesions. Based on this case, we believe that skin manifestations could be used to identify the characteristics of immune disorders and prognosis of AITL.

A new eruption of bullous pemphigoid following mRNA COVID-19 vaccination

The rapid development and implementation of COVID-19 vaccines throughout the global population has given rise to unique, rare, adverse skin reactions. This case report describes an elderly man with new-onset bullous pemphigoid following the second dose of the Pfizer-BioNTech (mRNA) COVID-19 vaccine.

Imiquimod-induced hypertrophic lupus erythematosus-like reaction.

Imiquimod is a topical immunomodulator that acts as an inducer of interferon (IFN)-a expression through Toll-like receptor (TLR)7 signaling with indications for the treatment of non-hyperkeratotic actinic keratosis of the face or scalp, superficial basal cell carcinoma (BCC), and external genital and perianal warts. Imiquimod is also used off-label for nodular BCC, cutaneous T-cell lymphoma, pyogenic granuloma, and melanoma. Imiquimod-induced lupus-like reactions have been reported. However, hypertrophic lupus erythematosus (HLE) is a rare variant of cutaneous lupus and imiquimod-induced hypertrophic lupus has not been reported to date. We report a case of local induction of a plaque that resembled HLE clinically and histologically in an 82-year old woman following topical treatment with imiquimod.

Successful treatment of exanthematous lichen planus in a young adult with low dose oral corticosteroid and isotretinoin.

Lichen planus is an inflammatory disease affecting the skin and mucosal membranes often with a chronic course lasting months to years with episodes of relapses. Classically it presents as flat topped, purple, polygonal, pruritic papules on the volar aspect of wrists and forearms, ankles, lower legs, and lumbo-sacral spine. We report a young woman with an exanthematous/eruptive variant of lichen planus who had a sudden outbreak of multiple papules and plaques all over the body with relative sparing of head and neck region. Eruptive lichen planus is rarely reported in adults and effective treatments are not well documented. We prescribed a short course of oral corticosteroid to which the patient did not respond. This was followed by oral isotretinoin and there was dramatic improvement in her symptoms and cutaneous lesions. A short course of oral corticosteroid followed with oral isotretinoin may be considered as a valuable management plan for exanthematous lichen planus. This combination may avoid serious adverse effects of both drugs when prescribed in high doses.

"Knife-cut" intertriginous ulcers related to herpes simplex virus in three patients

Linear intertriginous erosions and ulcerations related to herpes simplex virus (HSV) infection have been reported in patients with underlying immunosuppression. This rare presentation of HSV seems to occur predominantly in patients undergoing treatment of hematologic malignancies and rheumatologic conditions. Herein, we report three cases of linear "knife-cut" ulcerations in patients who were not undergoing active pharmacologic immunosuppressive therapy and lacked coexisting malignancy or autoimmune disease. Close examination of the skin folds for HSV infection is warranted to rule out disseminated infection as early intervention can be lifesaving.

Photo Vignette

Modified keystone flap used to repair nose defect after Mohs micrographic surgery

Mohs micrographic surgery (MMS) is a surgical technique used to remove skin tumors with a complete evaluation of the margins. The keystone flap technique is generally used to repair large surgical defects on limbs. We present a case where a modified keystone flap technique was used to close a large defect after Mohs micrographic surgery in a patient with a basal cell carcinoma on the nose. An excellent functional and aesthetic result was obtained with no complications during or after the procedure. We offer a novel indication for this technique for surgical defects in this area.