Dermatologic Emergencies CME Part V: Abuse-Related skin manifestations, dermatologic surgical emergency and other rare miscellaneous emergencies
Department of Dermatology, Basildon University Hospital NHS Foundation Trust, UK
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The last part of this continual medical education series reviews the cutaneous manifestations of physical and sexual abuse and their differential diagnosis, dermatologic surgical emergencies and miscellaneous dermatologic emergencies such as acute graft-versus-host disease, hypereosinophilic syndromes and vascular Ehlers-Danlos syndrome.
Key words: Physical abuse; Sexual abuse; Dermatologic surgeries; Acute graft-versus-host disease; Hypereosinophilic Syndromes
Abuse can take many different forms, including physical and emotional abuse. Child abuse, elder abuse, and domestic violence are common and affect patients of all socioeconomic classes and races. The diagnosis of physical abuse requires a high level of suspicion. A thorough history and physical examination are the cornerstones of diagnosis.
ABUSE-RELATED SKIN MANIFESTATIONS
Over 55 million children in the WHO European Region are affected by child maltreatment. A high prevalence of child abuse was documented in the 2013 European report on the prevention of child abuse, from 9.6 percent for sexual abuse, 16.3% for physical neglect, 18.4% for emotional neglect, 22.9% for physical abuse, and 29.6% for emotional abuse .
Cutaneous manifestations of physical abuse may include unexplained bruises, lacerations and abrasions, bite marks, curvilinear marks, burns and traumatic alopecia . Signs of sexual abuse may include attenuation, fresh tears, or scars of the hymen and the anal margin extending out onto the perianal skin (Fig. 1).
|Figure 1: Abuse-related skin manifestations Dermatologic conditions that can mimic abuse should be excluded before making the diagnosis of physical/sexual abuse.|
The diagnosis of physical abuse requires a high index of suspicion. A thorough history and physical examination are the mainstays of diagnosis . Dermatologists may be the first physicians to encounter the abused patient, so it is important to be alert for signs of abuse, perform the appropriate documentation and testing, and report it to the proper authorities .
DERMATOLOGIC SURGICAL EMERGENCY
Even for healthy patients, surgical procedures include some risk and can result in unpredictable complications. Although uncommon, recognition of dermatologic surgical emergencies while operating in outpatient setting is vital to avoid undesirable outcomes (Table 1).
OTHER RARE MISCELLANEOUS EMERGENCIES
Acute Graft-versus-host disease
Acute Graft-versus-host disease (GvHD) is caused by an immunological reaction between donor T lymphoid cells and the host tissue. Around 35 to 50 percent of recipients of hematopoietic stem cell transplant (HSCT) will experience acute GVHD typically before day 100 after the HSCT. The risk of aGvHD depends on the patient’s age, source of the stem cell, prophylaxis and conditioning methods .
Skin involvement is often the first indicator of acute GVHD (81%), followed by gastrointestinal (54%) and liver disease (50%). Skin manifestations range from a mild, asymptomatic morbilliform eruption to erythroderma with TEN-like bullae and desquamation (Table 2). Gastrointestinal involvement is usually the most severe and challenging to treat. It manifests as abdominal pain, nausea/vomiting, and secretory diarrhea. Hepatic disease is characterized by jaundice, elevated total bilirubin, and alkaline phosphatase levels . Diagnosis is usually based on clinical features after the exclusion of similar conditions . Skin biopsy, upper and lower GI endoscopy, and cholestatic pattern liver function derangement may aid in the diagnosis [27-29].
patients with mild (grade I) skin involvement without significant hepatic or GI symptoms usually respond to high-potency topical steroids. More severe disease (stage II-IV) is treated with systemic steroids (Fig. 2). Other modalities for steroid-unresponsive aGvHD include mycophenolate mofetil, extracorporeal photopheresis, biologics, pentostatin, methotrexate, and mesenchymal stem cells- .
|Figure 2: Graft-versus-host disease Management of acute Graft-versus-host disease according to its severity.|
Hypereosinophilic Syndromes (HES)
Hypereosinophilic Syndrome is a rare heterogeneous group of disorders characterized by significant eosinophilia (>1500 eosinophils/mm3 on at least two separate determinations or evidence of prominent tissue eosinophilia) associated with signs and symptoms of eosinophil-related end-organ damage after exclusion of secondary causes of eosinophilia, such as parasitic infections, allergic reactions, drug or chemical-induced eosinophilia, and neoplasms .
Common skin manifestations of HES include pruritic eczematous lesions, urticaria with or without angioedema, mucosal (oral and genital) ulcers, vasculitis, and erythroderma [35,36]. The two major clinical subtypes of HES are myeloproliferative and lymphocytic-variant HES. Myeloproliferative HES (M-HES) is usually associated with the tyrosine kinase fusion gene FIP1-like 1/Platelet-derived growth factor receptor (PDGFR) or other molecular mutations associated with eosinophil clonality. It is characterized by splenomegaly, thrombocytopenia, anemia, severely debilitating mucosal ulcers, and endomyocardial disease [37,38].
In lymphocytic-variant HES (L-HES), eosinophilia results from T-cell clones producing eosinophilopoietic cytokines such as IL-5. Compared to M-HES, this variant is more organ-restricted with a more protracted course and lower tissue fibrosis incidence .
The diagnosis of HES is based mainly on clinical features with the exclusion of other causes of eosinophilia.
Corticosteroids are the first-line therapy for most patients with HES . a tyrosine kinase inhibitor, Imatinib mesylate, is FDA approved for treatment of HES M-HES with a dramatic, quick response [41,42].
Vascular Ehlers-Danlos syndrome (vEDS)
Vascular Ehlers-Danlos syndrome is a rare inherited connective tissue disorder caused by mutated type III collagen. The condition is characterized by thin translucent skin with extensive bruising and hypermobility of the small joints. Patients with vEDS usually have a characteristic facial appearance, including pinched nose, thin lips, micrognathia, hollow cheeks, and prominent eyes due to decreased periorbital adipose tissue [44,45]. vEDS is associated with an increased risk of spontaneous arterial aneurysms, dissection, or rupture involving the medium-sized arteries, intestinal and uterine perforations, pneumothorax, and sudden death [46,47].
Diagnosis can be made using a combination of clinical features and molecular genetic testing to identify the mutated gene.
There are currently no specific treatments for vEDS; the aim is to alleviate the symptoms, prevent fatal complications, and provide genetic counseling [48,49].
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