United States

Background
Harlequin sign is a rare disorder of the sympathetic nervous system with a noteworthy clinical presentation. Disruption of T2-T3 sudomotor and vasomotor innervation manifests as a unilateral facial paleness and anhidrosis with compensatory flushing and hypersudation contralateral to the site of injury. Horner’s syndrome is commonly associated, which involves interruptions to the T1 oculomotor sympathetic neurons resulting in ptosis, pupil constriction, and lack of sweating ipsilaterally. Recognition of these clinical presentations is essential in determining appropriate workup. 

Case Presentation
An 81-year-old female with a history of metastatic lung cancer presented with right-sided flushing predominantly in a V1 and some V2 dermatomal distribution, sharply demarcated at the midline. Notably, the patient also had left pupillary miosis and left partial ptosis, although no asymmetric anhidrosis. Her review of systems was negative for pruritus, pain, tingling, burning, blistering of the skin, which argues against zoster. She denied any neuropathic symptoms, changes in vision, flushing when eating, or prior procedures to the face that might otherwise suggest Frey syndrome. Given the clinical findings, this condition was likely secondary to a mass effect from a left upper lobe lung tumor, which measured 11.0 cm with encasement of the left common carotid artery and posterior elements of T1 and T2 segments on MRI. The patient continued treatment for her underlying malignancy and her symptoms improved.

Discussion
Diagnosis relies on a thorough medical history and physical exam. A brief exercise test or heat exposure can also induce symptoms in the clinic. Cervical MRI scans may further elucidate the primary cause, which can be idiopathic, congenital, iatrogenic, or organic, such as surgical procedures or a mass in the cervical region. Thus, the underlying primary cause should be the focus for treatment management and symptoms may subsequently resolve on their own. Interventions such as botulinum toxin injections or sympathectomy can be recommended if flushing or sweating negatively affects social life. Although this patient already had a known history of malignancy, such cutaneous signs may manifest as the first presenting sign of internal disease and should prompt physicians to assess for the underlying etiology and consider imaging to rule out malignancy.


AMA Research Challenge 2022

Harlequin Sign Associated with Horner&#39;s Syndrome

Harlequin Sign Associated with Horner's Syndrome

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Histopathologic analysis of debulk specimens in Mohs micrographic surgery (MMS) may augment high-risk factor identification in cutaneous squamous cell carcinoma (cSCC), leading to tumor upstaging. Existing studies have yet to comment on the location of high-risk factors within tumors. Herein we report four cSCCs initially categorized as well-differentiated that were reclassified to moderate to poorly differentiated on debulk analysis and upstaged accordingly. In all cases, the deep tumor margins displayed morphological differences from other areas, exhibiting less differentiation and/or perineural invasion. Thorough inspection of the deep tumor margins may be required for accurate tumor staging and evaluation of metastatic risk. 

Case Series
Patient 1: An 80-year-old male presented with a 2 cm plaque on the frontal scalp. Biopsy revealed well-differentiated cSCC staged at T2a with Brigham and Women’s Hospital Staging System (BWH), subsequently upgraded to T2b during MMS due to galea involvement. Debulk analysis revealed moderate to poorly differentiated cSCC with the least differentiated cells at the deep margin.
 
Patient 2: A 75-year-old male presented with a 2 cm plaque on the left vertex scalp. Biopsy revealed well-differentiated cSCC staged at T2a (BWH), subsequently upgraded to T2b during MMS due to extension beyond subcutaneous fat and PNI. Examination of the second stage revealed moderately differentiated cSCC with the least differentiated cells at the deep margin.
 
Patient 3: An 86-year-old female presented with a 2.4 cm nodule on the right lower leg. Biopsy revealed well-differentiated invasive cSCC. Debulk analysis revealed moderately differentiated cSSC with the least differentiated cells at the deep margin.

Patient 4: An 82-year-old male presented with a 2.7 cm nodule on the vertex scalp. Biopsy revealed well-differentiated cSCC staged at T2a (BWH), subsequently upgraded to T2b during MMS due to extension beyond subcutaneous fat. Debulk analysis revealed moderate to poorly differentiated cSSC with the least differentiated cells at the deep margin in the galea.

cSCCs may exhibit intratumor heterogeneity in which predominantly well-differentiated tumors contain focal areas of poorer differentiation. Intratumor heterogeneity complicates tumor risk estimation as a well-differentiated tumor on biopsy may exhibit poor differentiation at a deeper margin. Thus, the most clinically relevant cells for prognosticating risk may not be visible on biopsy, underscoring the utility of close examination of the debulk specimen and deep margin of the Mohs layer. Despite this, most Mohs surgeons endorse immediately discarding debulk specimens without evaluation. These cases reinforce that tumor staging is incomplete until the deep margins are assessed to find the most dysplastic cells.

Inspection of Deep Tumor Margins in Mohs Micrographic Surgery for Accurate Cutaneous Squamous Cell Carcinoma Staging

Pretibial myxedema (PTM), more generally thyroid dermopathy (TD), results from mucopolysaccharide accumulation in the dermis, typically between the knee and dorsal foot. TD presents in Graves disease, but can occur in Hashimoto’s thyroiditis, primary hypothyroidism, and euthyroid patients. Treatment of thyroid eye disease (TED) with teprotumumab is established in the literature, with few case reports also showing improvement in PTM. Reported is a 76-year-old male with TED and PTM treated with teprotumumab with improvement in both conditions. He developed “muffled” hearing as an adverse effect, a complication not widely published in the dermatology literature. At 13 months post-treatment, his symptoms are stable without recurrence, but hypoacusis persists. Given the long-term efficacy and side-effects, dermatologists should recognize the potential benefits and risks of using teprotumumab for TD. A baseline audiogram may be considered prior to therapy. Additionally, longitudinal data is needed to document the benefits and risks of this novel therapy.

Novel teprotumumab treatment of severe thyroid dermopathy; ototoxicity as an adverse effect

Here we describe the case of a Black adolescent patient who was found to have widely metastatic melanoma originating from a primary vulvar lesion. The lesion presented as a pink, vegetative nodule with asymmetric borders of the clitoral hood which had grown gradually in size over several years and was confirmed to be melanoma on shave biopsy. This patient’s amelanotic presentation in conjunction with the rare incidence of vulvar melanoma contributed to the delay in identification of the malignancy. This case exemplifies the challenge of early recognition of potentially malignant vulvovaginal lesions for primary care providers, especially in pediatric patients where melanomas often present with atypical features.

Primary vulvar melanoma in an adolescent patient

Background
Since 2000 the incidence of syphilis in the United States has increased by over 400%. Classically, secondary syphilis presents as a generalized, non-pruritic, maculopapular rash, often including the palms and soles. However, due to the wide range of mucocutaneous presentations, secondary syphilis is often called ‘The Great Imitator.’ Herein, we report an unusual presentation of secondary syphilis to remind physicians to consider this diagnosis. 

Case Presentation
A 39-year-old female with a history of Hepatitis B presented with a two-month history of numerous tender, crusted, erythematous papules on the face and neck, as well as superficial ulcerations on the tongue, posterior oropharynx, and lower mucosal lip. The lesions first appeared around her nose and mouth after wearing a new cloth mask and then spread more broadly across her face and neck. She had previously been treated with courses of acyclovir and methylprednisolone with no improvement. A seven-day course of sulfamethoxazole-trimethoprim 800mg-160mg resulted in partial healing of some of the lesions. She also endorsed hoarseness and a sore throat but denied additional systemic symptoms, sick contacts, or recent travel. Our differential diagnoses included: sarcoidosis, deep fungal infection, bullous impetigo, Majocchi's granuloma, cutaneous tuberculosis, cutaneous lymphoma, and lupus.
Punch biopsies revealed superficial and deep dense lymphoplasmacytic infiltrate with spirochetes seen in the epidermis on H&E. The rapid plasma reagin titer was reactive (1:4), and anti-Treponema pallidum IgG was positive, confirming the diagnosis of secondary syphilis. The patient was treated with an intramuscular injection of Penicillin G benzathine resulting in complete resolution of the lesions.

Discussion
The incidence of syphilis infection has been increasing in the United States in recent years. The case described herein demonstrates the importance of including secondary syphilis, an infection that often masquerades as others, in the differential when sexually active patients present with mucocutaneous lesions. Rapid identification is essential to combat this increasingly prevalent infection in the United States, through expeditious treatment and effective contact tracing. 


The Great Imitator: an unusual presentation of secondary syphilis

A case of Serotonin syndrome due to the concurrent use of Sertraline, Trazodone, and 
Quetiapine

Open globe ruptures are a common sight-threatening trauma consisting of decreased vision, loss of anterior chamber depth, irregularly shaped pupil, 360-degree subconjunctival hemorrhage, and prolapse of uveal tissue. The delayed presentation further complicates the return of vision with infectivity, and autoimmune pathology, and increases the likelihood of enucleation.

A Man with Traumatic Vision Loss: A Case Report

Abstract Title: Myocarditis preceded by hemolysis secondary to a Loxosceles reclusa bite
Background: Envenomation from the brown recluse spider (Loxosceles reclusa) can cause significant tissue damage or severe systemic illness, primarily potentially fatal hemolysis and renal failure. Few cases demonstrate loxoscelism-induced myocarditis. We present a case of loxoscelism with hemolytic anemia and myocarditis leading to cardiogenic shock and respiratory failure requiring mechanical ventilation.
Case Presentation: A previously healthy 18-year-old man presented to the emergency department with a one-day history of pleuritic chest pain and fever and was discharged with a diagnosis of viral syndrome after an unremarkable workup. He returned to the ED three days with a new morbilliform rash, dark urine, and painful right scapular lesion which reportedly began 5 days earlier. He was initially febrile and hypotensive to 59/49 and was admitted to the intensive care unit. On hospital day two, he had worsening tachycardia and hypotension. Electrocardiogram showed diffuse ST-segment elevation. Troponin peaked at 2.51 ng/mL. Transthoracic echocardiogram showed a maximally depressed ejection fraction of 28%. Respiratory failure ensued requiring intubation and mechanical ventilation. His Coombs positive hemolytic anemia had a hemoglobin nadir of 7.6 g/dL. An ELISA test of the scapular wound (SpiderTek, Rolla, MO) confirmed Loxosceles reclusa venom. By hospital day five, the patient was weaned from inotropes and extubated. He did not receive IV steroids, IVIG, or plasmapheresis. A repeat echocardiogram was normal. The patient was discharged on hospital day nine with a diagnosis of myocarditis.
Discussion: This is the third report of myocarditis associated with loxoscelism, the second case confirmed with a positive test for Loxosceles venom, and the first with an acute course requiring ventilator support. These studies include two cases of cardiac magnetic resonance-confirmed myocarditis. A direct cardiotoxic Loxosceles species venom has been previously demonstrated. Additionally, Interleukin 6, known to be elevated in loxoscelism, is associated with severe myocarditis. The abrupt decrease in cardiac function in an otherwise young, healthy patient, followed by rapid recovery, is consistent with toxin-mediated myocarditis caused by L. reclusa envenomation. Providers should be aware that recluse spider bites can cause life-threatening myocarditis and should tell all patients with recluse bites should seek immediate medical attention for dark urine.

Myocarditis Preceded by Hemolysis Secondary to a Loxosceles Reclusa Bite

This case illustrates a rare presentation of hypothalamic lipoma in a patient who presents with hyperprolactinemia causing galactorrhea in a patient with a Family History of Birt-Hogg-Dubé syndrome. The Birt Hogg Dubé Syndrome (BHD) is a rare autosomal dominant disorder caused by a mutation in the FLCNgene and is only found in approximately 200 families worldwide to date. This condition manifests clinically as fibrofolliculomas, acrochordons, angiofibroma, and pulmonary cysts developing into spontaneous pneumothorax and renal tumors. A few cases of BHD syndrome have recently been reported to have lipomas, angiomyolipomas, and lesions containing abnormal connective tissue. Intracranial lipomas, however, have not been reported. Further genetic analysis in our case may link the possible presentation of intracranial lipomas to BHD syndrome.

A Case of Hypothalamic Lipoma causing Hyperprolactinemia in a Patient with Family History of Birt-Hogg-Dubé syndrome.​

Background
Hemorrhage of the adrenal glands is uncommon and can present with a variety of symptoms. A high index of suspicion based on awareness of risk factors associated with the condition is vital as the condition’s mortality rate is estimated at 15%. 

Case Presentation
CH is a 43-year-old male with past medical history of lupus anticoagulant disorder who presented to his PCP with muscle aches, abdominal pain, confusion, poor appetite and tachycardia. One week prior, he had a deep tissue massage where the therapist walked on his back. He also switched from Coumadin to Xarelto approximately one month ago. Two days prior to presenting to his primary care office, he was discharged from the hospital after being admitted with symptoms of back pain, abdominal pain, constipation and dark urine. A CT during that admission revealed a right-sided adrenal hemorrhage. However, with normalization of his urine color and improvement of his abdominal pain following constipation treatments, he was discharged home. At his PCP’s office, a CMP was ordered that revealed a serum Na level of 120 mg/dl. This result cued his readmission. Further workup revealed elevated ACTH and undetectable serum cortisol levels supporting adrenal insufficiency. He was started on IV normal saline and a hydrocortisone regimen. His symptoms gradually improved, and two days later his serum Na was 132 mg/dl. He was then discharged home with a hydrocortisone taper and a follow-up with endocrinology.

Discussion
Risk factors for adrenal hemorrhage include physical trauma, anticoagulants, hypercoagulable states and severe stressors. Anticoagulants are implicated in one third of patients with adrenal hemorrhage. Additionally, it has been found that there is an increased incidence of hemorrhage within the first weeks of initiating anticoagulants. Imaging of the adrenals, particularly CT of the abdomen, can confirm the diagnosis of adrenal hemorrhage. Serum sodium, ACTH and cortisol levels are also vital to obtain as they provide valuable insight on the presence of adrenal insufficiency. Results supporting the diagnosis of adrenal hemorrhage with adrenal insufficiency should prompt treatment with surgical intervention and/or non-invasive management with glucocorticoids as appropriate. These treatments could be lifesaving.


A Deep Tissue Massage and a Factor Xa Inhibitor: The Recipe for a Life-Threatening Crisis?

Abstract Title:
Flatbush diabetes – ketosis prone atypical diabetes mellitus: A case report

Background:
Flatbush diabetes, also termed as atypical or Ketosis-prone type 2 diabetes mellitus is being increasingly reported. It is characterized by sudden onset ketoacidosis, absence of pancreatic beta cell autoimmunity and dysfunction. Though cases commonly seen amongst African-Americans and Afro-Caribbean individuals, it is also seen in Indian populations. The aetiology is unknown but predisposition to glucose desensitization is suspected.
These patients are typically obese, middle-aged men who has strong family history of type 2 diabetes. Here we report an atypical case of Flatbush diabetes in a young female patient who has no family H/O DM.



Case Presentation:
A 31-year-old female presented to emergency room with complaints of grade IV dyspnea, abdominal distension and 5 episodes of bilious vomiting’s since morning. She has history of recurrent diffuse abdominal pain, vomiting’s, polyuria, polydipsia, weight loss, hair loss for 3 months. Her vitals at presentation are 110/80 mmHg of BP, pulse rate is 122/min and oxygen saturation is 99% on room air.
On examination she is diaphoretic, with severe tachypnea. Capillary blood glucose levels are high (>599). ABG showed PH of 6.99, pCO2 of 7 with no derived values of bicarbonate. urine ketones tested positive with dip stick (3+). She is immediately started on IV fluids and subsequently managed as DKA according to protocols. Her HbA1C is 12%. Her C-peptide levels are with in normal limits. Autoantibodies against GAD, tyrosine phosphatase, insulin are absent. CECT is normal. No family H/O diabetes. She is put on glargine U-100 along with ADA’s and is reviewed after 2 weeks. In these two weeks she didn’t have hypoglycemic attacks and symptoms of DKA, and Urine ketones tested negative. Insulin dose is adjusted and reviewed after 1 month.



Discussion:
This case report sheds light on the atypicality of presentation and summarizes the main diagnostic features of Flatbush diabetes. Absence of autoantibodies to GAD, insulin, tyrosine phosphatase and presence of normal levels of C-peptide rules out type 1 DM. 

This condition may improve after temporary insulin therapy and many patients become insulin-independent eventually. Increased awareness of this atypical form of DM can facilitate early diagnosis and management.



Next from AMA Research Challenge 2022

Inspection of Deep Tumor Margins in Mohs Micrographic Surgery for Accurate Cutaneous Squamous Cell Carcinoma Staging

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