SAEM Clinical Images Series: More Than Skin Deep

skin

A 57-year-old female college counselor living in the northeastern United States with no PMH presented for evaluation of rash, joint pain, and dyspnea for the past three weeks. The patient first noticed the rash on her upper back, describing it as being itchy. The rash then spread to her face, scalp, and thighs. Two weeks ago, she noticed swelling in her hands and had a gradual onset of dyspnea on exertion. The patient has pain in her hands and when moving her fingers. She denied fever, cough, chills, chest pain, headache, vision changes, focal weakness, abdominal pain, nausea, vomiting, and diarrhea. She denied recent travel, sick contacts, significant time spent outdoors, known tick bites, new medications, and changes in her diet. She has never had a rash like this before.

Vitals: BP 110/70; HR 86 BPM; RR 18 breaths/min; T 37°C; SpO2 96% on RA

Skin: Warm and dry. There is a macular, violaceous rash to the upper back and upper thighs. The patient’s hands are slightly edematous with nontender papules on the palmar aspect of the hands.

CV: Heart sounds are normal. No jugular venous distention or lower extremity edema.

Lungs: There are faint bibasilar rales heard on auscultation of the chest.

Extremities: Full ROM of the joints and there are no bony deformities. The patient does not have muscular tenderness.

Neuro: Within normal limits; muscle strength 5/5 in all four extremities.

CBC w/ differential: Normal

BMP: Na 142, K 3.8, Cl 106, HCO3 24, BUN 16, Cr 0.44, Glu 112, Ca 8.5, Mg 2.2, Phos 3.4

LFT’s: AST 105, ALT 76, ALP 68, Tbili 0.2 Alb 3.7

PT/PTT/INR: 12.0s/32.2/1.04

D-dimer: 347

ESR: 62

CRP: 0.75

Ferritin: 625

CK: 195

LDH: 276

Procalcitonin: undetectable

Amyopathic dermatomyositis – specifically anti-melanoma differentiation-associated gene 5 (anti-MDA5) positive dermatomyositis as determined by subsequent inpatient auto-immunological workup. Compared to other dermatomyositides, anti-MDA5 positive dermatomyositis is characterized by an absence of traditional muscular involvement. Additionally, patients can present with respiratory symptoms related to interstitial lung disease (ILD). One phenotype of this condition is associated with a rapidly progressive ILD, but respiratory involvement may be delayed years after the initial symptoms are noticed. The patient’s clinical images demonstrate a macular, violaceous rash in the “shawl sign” and “holster sign” distribution patterns typical of dermatomyositides. Palmar papules (not to be confused with Gottron’s papules which are found on the dorsal surface of the metacarpophalangeal and interphalangeal joints) are fairly specific for anti-MDA5 positive dermatomyositis

There are no specific guidelines for treating anti-MDA5 positive dermatomyositis. Patients are typically started on a high-dose steroid regimen. A rheumatology consult should be obtained to determine if the patient would benefit from treatment with immunosuppressants. Given her complaints of dyspnea, the patient should undergo a non-contrast CT of the chest to evaluate for evidence of scarring or pulmonary fibrosis.

Take-Home Points

  • Anti-MDA5 positive dermatomyositis is associated with rapidly progressive ILD has a poor prognosis.
  • This rare form of dermatomyositis should be suspected if the patient has respiratory complaints in addition to the hallmark cutaneous findings commonly observed in all types of dermatomyositides. Palmar papules are fairly specific for anti-MDA5 positive dermatomyositis. It often lacks typical historical and physical features of muscular weakness.
  • Treatment involves high-dose corticosteroids and consideration of immunomodulator therapy.

  • Allenbach Y, Uzunhan Y, Toquet S, Leroux G, Gallay L, Marquet A, Meyer A, Guillaud C, Limal N, Gagnadoux F, Hervier B, Borie R, Deligny C, Terrier B, Berezne A, Audia S, Champtiaux N, Devilliers H, Voermans N, Diot E, Servettaz A, Marhadour T, Castelain V, Humbert S, Blanchard-Delaunay C, Tieulie N, Charles P, Gerin M, Mekinian A, Priou P, Meurice JC, Tazi A, Cottin V, Miyara M, Grange B, Israël-Biet D, Phin-Huynh S, Bron C, De Saint Martin L, Fabien N, Mariampillai K, Nunes H, Benveniste O; French Myositis Network. Different phenotypes in dermatomyositis associated with anti-MDA5 antibody: Study of 121 cases. Neurology. 2020 Jul 7;95(1):e70-e78. doi: 10.1212/WNL.0000000000009727. Epub 2020 Jun 2. PMID: 32487712; PMCID: PMC7371381.
  • Nombel A, Fabien N, Coutant F. Dermatomyositis With Anti-MDA5 Antibodies: Bioclinical Features, Pathogenesis and Emerging Therapies. Front Immunol. 2021 Oct 20;12:773352. doi: 10.3389/fimmu.2021.773352. PMID: 34745149; PMCID: PMC8564476.

SAEM Clinical Images Series: Man with a Recurrent Rash

rash

A 33-year-old male presented to the emergency department with a diffuse pruritic rash that appeared several days after starting Trimethoprim/Sulfamethoxazole (TMP-SMX) for a dental infection. Initially beginning on the torso and low back, the rash spread to the palms, soles, and genitalia. Progression stopped after discontinuing TMP-SMX. He conveyed a remote history of a similar rash following use of an unknown medication, and noted that several of the current lesions arose at the same location as previous.

Skin: Widely distributed violaceous, non-blanching patches with a dusky center. Lesions ranged from 3 cm to 10 cm, and included palms and soles. There was no mucosal involvement.

Non-contributory

Fixed drug eruption (FDE). FDE is an uncommon, potentially life-threatening CD8+ T-helper cell-mediated hypersensitivity reaction to certain drugs, commonly NSAIDs, antibiotics, and antiepileptic [1].

Skin findings typically arise within two days of exposure and then more rapidly with subsequent exposures [2]. Characteristically, recurrent lesions appear at the same sites as prior lesions (hence “fixed”) but may arise in additional locations. The rash is classically divided into two phases: an acute phase of pruritic violaceous patches and plaques with central duskiness, followed by a residual phase of hyperpigmentation that can last several months. The sulfonamide moiety of TMP-SMX is a common cause of FDE [3]. Management of FDE anchors on identification and discontinuation of the causative agent. The majority of cases involve five or fewer lesions, however generalized or bullous cases (> 10% total body surface area, or involvement of 3 or more anatomic sites) [1], may require aggressive wound care and carry a mortality rate up to 22% [4]. Topical or systemic steroids are common adjuncts and there is limited evidence suggesting the utility of systemic cyclosporine for severe cases [1]. Patients need to be carefully advised on the risks of specific medication use and can expect a gradual resolution of lesions over the coming months.

Take-Home Points

  • FDE is a potentially life-threatening hypersensitivity reaction to certain drugs.
  • Recurrent lesions in similar distribution is a hallmark of FDE. Avoidance of the causative agent is the mainstay of management.
  1. Anderson HJ, Lee JB. A Review of Fixed Drug Eruption with a Special Focus on Generalized Bullous Fixed Drug Eruption. Medicina (Kaunas). 2021 Sep 1;57(9):925. doi: 10.3390/medicina57090925. PMID: 34577848; PMCID: PMC8468217.
  2. Flowers H, Brodell R, Brents M, Wyatt JP. Fixed drug eruptions: presentation, diagnosis, and management. South Med J. 2014 Nov;107(11):724-7. doi: 10.14423/SMJ.0000000000000195. PMID: 25365443.
  3. Chow TG, Khan DA. Sulfonamide Hypersensitivity. Clin Rev Allergy Immunol. 2022 Jun;62(3):400-412. doi: 10.1007/s12016-021-08872-3. Epub 2021 Jul 1. PMID: 34212341
  4. Lipowicz S, Sekula P, Ingen-Housz-Oro S, Liss Y, Sassolas B, Dunant A, Roujeau JC, Mockenhaupt M. Prognosis of generalized bullous fixed drug eruption: comparison with Stevens-Johnson syndrome and toxic epidermal necrolysis. Br J Dermatol. 2013 Apr;168(4):726-32. doi: 10.1111/bjd.12133. Epub 2013 Feb 16. PMID: 23413807.

SAEM Clinical Images Series: A Case of Painful Skin Lesions

necrobiosis

A 50-year-old Caucasian female with a history of hypertension, coronary artery disease, and insulin-dependent diabetes mellitus presents to the emergency department with a complaint of painful sores on the top of her left foot. She notes that ulcerations have formed over the past two weeks and reports a history of multiple recurrent usually non-tender skin lesions to her lower extremities, forearms, and hands over the past twenty years. She is homeless and medically non-compliant secondary to financial issues.

Vitals: T 37.2°C; BP 149/77; HR 94; RR 20

Skin: Multiple yellow-brown and violaceous plaques on the pretibial lower extremities and feet, some exhibiting ulceration with central necrosis and surrounding erythema. Raised reddish-brown well-demarcated plaques with waxy centers were also noted on the dorsal forearms and hands.

Glucose: 539 (with a normal anion gap)

Hemoglobin A1C: 10.9

Necrobiosis Lipoidica – This patient had a previous skin biopsy with histopathologic changes demonstrating a granulomatous dermatitis involving the dermis and subcutaneous tissues with necrobiosis of collagen and inflammatory infiltrates of lymphocytes and plasma cells consistent with a diagnosis of necrobiosis lipoidica.

Necrobiosis lipoidica is a rare, chronic, idiopathic, granulomatous disease of collagen degeneration classically associated with type 1 diabetes (with a prevalence of 0.3 to 1.2%). It may present as the first clinical finding of or a precursor to diabetes, although its course is unaffected by glycemic control and it is unrelated to other diabetic complications including renal, ocular, and vascular problems. It has been associated with thyroid disease, inflammatory bowel disease, rheumatoid arthritis, and sarcoidosis. It may be equally common in patients without diabetes, hence was renamed without the term “diabeticorum”.

Necrobiosis lipoidica typically is asymptomatic and presents in females (average onset at age of 30) as small, well-demarcated papules that expand into waxy-centered plaques with indurated borders that may resolve spontaneously (up to 17%) or may be complicated by ulceration, infection, and occasionally transformation to squamous cell carcinoma. The differential diagnosis includes other granulomatous and inflammatory diseases such as granuloma annulare, sarcoidosis, rheumatoid arthritis, and necrobiotic xanthogranuloma. The diagnosis is suggested by clinical presentation and is proven by biopsy.

Complications of necrobiosis lipoidica include long-term scarring, ulceration (more common in males), infection, and when lesions are chronic they may rarely transform into squamous cell carcinoma. There is no cure for necrobiosis lipoidica, and some skin lesions may resolve spontaneously, therefore, treatment is focused on addressing any complications. Multiple medical and surgical interventions have been tried. Topical and intra-lesional corticosteroids have been used to stabilize rapidly enlarging lesions with limited success, however, have the potential to cause further skin atrophy. Surgical interventions including debridement and skin grafting are discouraged as in necrobiosis lipoidica trauma tends to induce the Koebner phenomenon.

Take-Home Points

  • Necrobiosis lipoidica is an idiopathic rare skin disease classically associated with insulin-dependent diabetes mellitus but may affect otherwise healthy individuals.
  • More common in females but more severe in males, necrobiosis lipoidica usually affects the pretibial lower extremities, may present in various stages, and has no known cure.
  • Non-diabetic patients presenting with necrobiosis lipoidica should be monitored for the development of diabetes mellitus, thyroid and inflammatory diseases, and squamous cell carcinoma.

  • Lepe K, Riley CA, Salazar FJ. Necrobiosis Lipoidica. [Updated 2022 Dec 1]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK459318/ PMID:29083569.
  • Kota SK, Jammula S, Kota SK, Meher LK, Modi KD. Necrobiosis lipoidica diabeticorum: A case-based review of literature. Indian J Endocrinol Metab. 2012 Jul;16(4):614-20. doi: 10.4103/2230-8210.98023. PMID: 22837927; PMCID: PMC3401767.

SAEM Clinical Images Series: Unusual Scalp Lesions

scalp

A 6-year-old male presented to the pediatric emergency department (PED) for scalp lesions. He was seen by his pediatrician 2 weeks prior and prescribed antibiotics and a delousing shampoo for suspected cellulitis versus lice infestation. Symptoms did not improve despite completion of treatment. An outpatient ultrasound was performed showing “multiple scalp echogenic nodular lesions measuring from 0.5 cm to 1.2 cm in the long axis diameter.” The following differential diagnosis was entertained: lymphadenitis, benign avascular mass, epidermal inclusion cyst, or pilomatricoma, and the patient was started on clindamycin. Due to concern for an oncologic process, a surgery consultation was placed to arrange for a biopsy. Four days after the ultrasound and before the biopsy could be performed, the patient and his mother presented to the PED due to worsening symptoms. Multiple new lesions developed across the patient’s scalp which bled when pressure was applied. The patient denied fever and reported intermittent pruritus and pain over the lesion sites. The mother reported a history of travel to Ecuador one month prior to symptom onset.

Vitals: BP 98/61; Pulse 73; Temp 36.3°C (97.3°F) temporal; Resp 18; SpO2 99%, RA

Skin: Large, 3 x 3cm indurated, erythematous lesion located over the patient’s right temporal scalp (Image 1). Five additional lesions noted across the entirety of the scalp. No lesions identified below the neck. Lesions are mildly tender to palpation; no fluid able to be expressed. A small centrally located pore is noted on each lesion with appearance of pulsatile fluid level. No associated lymphadenopathy. A point-of-care ultrasound (POCUS) using a high-frequency, linear transducer was performed during the PED visit (Image 2).

Non-contributory

In short axis, there is an echogenic lesion with surrounding fluid (halo sign) suggesting a foreign body that also exhibits posterior acoustic shadowing. With the transducer held still, independent movement is visualized within the center of the lesion (Image 3).

Cutaneous furuncular myiasis due to Dermatobia hominis (botfly infestation).

Take-Home Points

  • Native to Central and South America, botfly infestation is facilitated through an infected female mosquito which deposits its eggs on the skin of a mammal on which it feeds.
  • Cutaneous furuncular myiasis is important to consider for unexplained head, neck, and extremity lesions when there is suspected travel to endemic areas and is unlikely to be recognized in the continental United States due to low prevalence.
  • Consider pertinent physical exam findings and utility of POCUS in confirming the diagnosis.
  • Harris AT, Bhatti I, Bajaj Y, Smelt GJ. An unusual cause of pre-auricular swelling. J Laryngol Otol. 2010 Mar;124(3):339-40. doi: 10.1017/S002221510999082X. Epub 2009 Aug 11. PMID: 19664319.
  • Minakova E, Doniger SJ. Botfly larva masquerading as periorbital cellulitis: identification by point-of-care ultrasonography. Pediatr Emerg Care. 2014 Jun;30(6):437-9. doi: 10.1097/PEC.0000000000000156. PMID: 24892687.

SAEM Clinical Images Series: A Rare Pediatric Scalp Rash

rash

The patient is a 3-month-old, full-term male who presents with a rash on his head. The rash started one day prior to presentation on his forehead and spread to the rest of his head. Today, it developed a central clearing with surrounding redness. He has a history of sensitive skin since birth with patches of eczema and cradle cap. He treats these with Aquaphor and Honest Co. Cream; he has never been prescribed topical steroids for his rashes. Denies fever, cough, rhinorrhea, congestion, decreased appetite, diarrhea, and decreased urination. He had an uncomplicated birth history.

General: Well appearing, no distress.

Skin: Large, serpiginous rash on the left forehead and scalp with central clearing and peripheral erythema as well as areas of erythematous plaques. He has some erythema of the left medial epicanthus. He also has a large erythematous patch at the base of his skull. The remainder of his skin is clear.

CV: Normal rate and rhythm, no murmur.

White blood cell (WBC) count: 8.4

Hemoglobin: 12.4

Hematocrit: 37.1

Platelet Count: 468

Complete metabolic panel (CMP): ALT 30, AST 60, Alk phos 266, Tbili 0.7, Total Protein 6.4

The image is of the cutaneous manifestation of neonatal lupus erythematosus. Neonatal lupus erythematosus is an autoimmune disease caused by transplacental passage of maternal autoantibodies to Sjögren’s syndrome A or B autoantigens (SS-A/SS-B). It can present with reversible changes including cutaneous lesions (most common, in up to 40% of patients), hepatobiliary disease, and cytopenias, which resolve once maternal autoantibodies have been cleared.

All infants that present with concern for neonatal lupus erythematosus should have screening labs performed to evaluate for hematologic, cardiac, and hepatobiliary involvement including a CBC with differential, liver enzymes, and antibody testing. In addition, an EKG is essential given that neonates can present with irreversible total atrioventricular heart block, which can present in utero or after birth.

The rash typically presents in the first few weeks of life but can present as late as 2-3 months of life (usually within 1-2 days of first sun exposure). Eighty percent of cases are not clear at birth and present in the first month of life. The rash appears as a coalescing rash with raised margins, with annular and discoid erythema involving the head in 95% of cases. It is often misdiagnosed as skin infections or eczema if the mom is asymptomatic. Fifty percent resolve by four months of life and 100% by one year.

Any neonate with a slow fetal heart rate or the postnatal diagnosis of atrioventricular heart block warrants immediate maternal testing for these autoantibodies. Most cardiac changes from neonatal lupus are diagnosed before 26 weeks gestation, with <20% later in pregnancy and 2% detected postnatally.

Take-Home Points

  • While cutaneous findings of neonatal lupus most commonly present in the first month of life, they can present as late as 2-3 months.
  • The cutaneous findings associated with neonatal lupus most of the time resolve in 4-6 months (when maternal antibodies are cleared from the infant’s circulation).
  • Any baby with findings concerning for neonatal lupus should have an EKG performed. Around 2% of infants present with heart block postnatally within the first month.

  • Diaz-Frias J, Badri T. Neonatal Lupus Erythematosus. [Updated 2021 Jun 30]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK526061/
  • Lee LA. Neonatal lupus erythematosus: clinical findings and pathogenesis. J Investig Dermatol Symp Proc. 2004 Jan;9(1):52-6. doi: 10.1111/j.1087-0024.2004.00827.x. PMID: 14870986.

By |2023-04-05T14:00:29-07:00Apr 10, 2023|Dermatology, Pediatrics, SAEM Clinical Images|
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