In part 2 of this review, more vulvovaginal diseases are examined.
EDITOR’S NOTE: This is part 2 of a 2-part series. Part 1 was published in the July issue of Contemporary OB/GYN.
Skin disease is common in hospitalized patients. This article focuses on the recognition and management of common dermatologic and systemic diseases that may present with vulvovaginal involvement. In Part I, we reviewed red flags for severe skin disease. Then we reviewed drug-induced conditions where particular care should be taken to manage vulvovaginal involvement in the inpatient setting. Here, we continue our discussion and cover vulvovaginal involvement in inflammatory and ulcerative conditions in the inpatient setting.
Pustular psoriasis of pregnancy
Pustular psoriasis of pregnancy (PPP) is a life-threatening condition that typically occurs in the third trimester of pregnancy, though it may present during any trimester.
Patients present with symmetric erythematous plaques with overlying sterile cutaneous pustules at plaque margins in a circinate pattern (Figure 1). Skin changes are often accompanied by systemic signs and symptoms such as fatigue, fever, elevated inflammatory markers, diarrhea, and delirium.1-3 In severe cases, disease can progress to erythroderma and be complicated by dangerous fluid and electrolyte imbalances, loss of thermoregulation in the skin, and secondary infection and sepsis.1,4
Evidence for treatment of patients with PPP is limited and depends on severity of presentation. First-line treatments include high-dose systemic corticosteroids vs low-dose cyclosporine.1 This condition is associated with placental insufficiency, so systemic signs and symptoms need close fetal monitoring because of an increased risk of fetal anomalies and fetal demise.1,5 Although pustular psoriasis of pregnancy often resolves after parturition, cases have been reported in the postpartum period, and recurrence in subsequent pregnancies is common.1,6
Hidradenitis suppurativa (HS) is a chronic follicular occlusive disease that affects any skin area containing folliculopilosebaceous units. Usual symptom onset is in the second or third decade of life, and females are more likely to be affected.7-9 Factors associated with the disease include a family history of HS, obesity, and smoking.
The primary lesion of HS is the recurrent, painful inflammatory nodule, which predominantly affects intertriginous areas. Distribution is influenced by sex, and females are more likely to have involvement of the axillae, upper anterior torso including breast and inframammary regions, buttocks, and gluteal cleft.10,11
HS may also affect the vulva (Figure 2). Vulvar HS can often be mistaken for other vulvar diseases such as abscesses, folliculitis, or Bartholin cyst.12 Compared with patients with HS without vulvar involvement, patients with vulvar HS tend to present at a later onset, with lower body mass index, and higher incidence of psychiatric disease.12 Clinical manifestations include the presence of fistulas with minimal axillary involvement.12 Chronic disease may result in subcutaneous extension with suppuration, sinus tract formation, or hypertrophic scarring. In addition, an association with vulvar cancer has been demonstrated in patients with disease involving the groin.13
Treatment depends on disease stage. Acute management includes education, topical field therapy, anti-inflammatory antibiotics, intralesional triamcinolone (Kenalog), or prednisone as a bridge to biologics. Most patients with vulvar HS have been shown to respond well to medical treatment and not require surgery.12 HS may significantly impact sexual function and quality of life. In cases of anogenital involvement, impairment can be especially significant.14 Patient support is available at the HS Foundation website.
Genital herpes is caused by primary and recurrent infection involving herpes simplex virus type 1 (HSV-1) or type 2 (HSV-2). Most cases of recurrent disease are caused by HSV-2, but increasingly HSV-1 is associated with genital herpes, especially in younger women.15 Genital herpes is underdiagnosed because infection is often subclinical.16
In women, genital herpes commonly involves the vulva and vagina (Figure 3). Primary infection presents with highly variable symptoms, including painful genital ulcers, systemic symptoms such as fever and headache, local pain and pruritus, tender lymphadenopathy, and dysuria.17-19 Physical exam in primary disease reveals characteristic grouped 2- to 4-mm vesicles, erosions, or ulcers, which may be angulated or linear. Other patients have milder symptoms or may be asymptomatic. Recurrent infection is characterized by less severe symptoms, including smaller vesicular or ulcerative lesions that are often unilateral.18 Of note, rate of recurrent infection is positively associated with initial infection duration and severity, but unrelated to treatment with acyclovir.20
Clinical diagnosis is neither sensitive nor specific, and suspicions should be followed with routine use of virological and type-specific serology. Viral culture and polymerase chain reaction testing are preferred methods in cases of active lesions. Most patients experiencing their first episode of genital HSV infection should be treated with an antiviral to decrease duration and severity of disease. Patients with severe HSV disease or complications, such as disseminated infection, pneumonitis, or hepatitis, should be treated with intravenous acyclovir until clinical improvement, with subsequent oral antivirals to total at least 10 days of therapy.21 Treatment of recurrent infection should be individualized; chronic suppressive therapy is generally recommended in patients with severe or frequent recurrences. In addition, all patients with genital herpes should be counseled on the natural course of the disease, including identifying any symptomatic prodrome prior to recurrence and understanding the potential for asymptomatic transmission.
Acute vulvar aphthous ulcers
Acute vulvar aphthous ulcers (also known as Lipschütz acute vulvar ulceration) are similar to the more common oral aphthous ulcer, though typically much larger in size.22 The condition mainly affects patients 20 years or younger, who present with sudden onset of necrotic and painful ulcers. Exact disease pathogenesis is not certain but may be related to a hypersensitivity reaction in the context of bacterial or viral infection.23
On skin examination, patients most commonly present with 1 to 3 large (more than 10 mm), well-delimited lesions with a fibrinous necrotic center and symmetric distribution (Figure 4). Lesions are often described as “kissing.”22 Associated symptoms include a flulike illness or mononucleosislike syndrome.
This is a diagnosis of exclusion. A full review of systems should be obtained, and the differential should initially include other noninfectious causes of vulvar ulcers. Patients should be screened for underlying inflammatory bowel disease with fecal calprotectin.
Treatment depends on ulcer size. Small ulcers are treated with topical steroids, whereas larger lesions warrant a short course of oral prednisone until pain improves. Of note, systemic corticosteroids have not been shown to reduce disease duration.22 Pain control is another important component of treatment. Burning pain can be improved with copious amounts of zinc paste; patients also can be instructed to urinate while in a tub of water. Ulcers tend to resolve within 3 weeks without recurrence. Patient education should emphasize the natural course of disease and the fact that disease is not sexually transmitted.22
Pyoderma gangrenosum (PG) is an idiopathic, inflammatory, ulcerative neutrophilic dermatosis that most commonly occurs on the lower extremities. The average age of onset is 40 to 60 years, and females are more commonly affected than males.24 Pyoderma gangrenosum is often associated with systemic disease such as inflammatory bowel disease, arthritis, malignancy, and hematological disease.25 Disease pathogenesis is unclear.
Pyoderma gangrenosum is a diagnosis of exclusion aided by evaluation of ulcer morphology, histopathology, and rapid response to systemic corticosteroids.26 Other entities important to exclude include infectious disease, Behçet disease, metastatic Crohn disease, and pemphigus vegetans.27 Pyoderma gangrenosum most commonly presents with 1 or more painful ulcers with violaceous, undermined borders. Most lesions affect the lower extremities or peristomal areas but may involve the upper extremities or genitalia, including the vulva. Genital PG is rare, but recognition is important. It should be considered in cases of genital ulcerations that are nonhealing and painful.26 Genital PG may be misdiagnosed as malignancy, leading to unnecessary radical surgery with associated morbidity and mortality. Studies has shown an inconsistent association between genital PG and systemic diseases.27,28
Treatment includes topical or systemic corticosteroids, minocycline, tacrolimus, cyclosporine, dapsone (Aczone), or surgical resection.26,29,30 With treatment, more than two-thirds of patients have been shown to achieve complete remission within 6 months, and 95% were in remission within 3 years.31
Cutaneous Crohn disease
Crohn disease (CD) is a relapsing-remitting full-thickness inflammatory disease of the gastrointestinal (GI) tract that can involve anywhere from the oral cavity to the anus. Crohn disease most often presents in early adulthood.32
Cardinal symptoms include abdominal pain, diarrhea, intermittent diarrhea, fatigue, and weight loss. Extraintestinal manifestations may involve musculoskeletal, ocular, skin, and hepatobiliary systems. Skin manifestations occur in up to one-third of patients and may be divided on an etiopathological basis into disease-specific lesions that share histopathology with underlying GI disease, reactive lesions secondary to inflammation, and associated conditions secondary to chronic inflammation or human leukocyte antigen linkages.33
Disease-specific lesions may involve the genital region and include perianal fissures and fistulae, and metastatic (cutaneous) lesions. Chronically, perianal fissures and fistulae may form abscesses and acrochordae, and form the majority of genital lesions in Crohn disease. Metastatic (cutaneous) lesions refer to inflammatory lesions involving areas not typically considered part of the underling Crohn disease. They can involve extremities, intertriginous areas, the face, and genitalia, and they are generally recognized as a rarer cutaneous manifestation of Crohn disease.33,34
Metastatic lesions involving the vulva are an underrecognized condition.35,36 Vulvoperineal Crohn disease (Figure 5) may occur in the absence of abdominal complaints, preceding gastrointestinal manifestations by months to years.33 Initial presentation may include vulvar or perineal pain.37 Physical exams can vary widely and may reveal vulvar ulcers in approximately half of vulvar cases, labial edema, or hypertrophic lesions.35,36
Definitive diagnosis requires biopsy because clinical appearance is not specific. Surgical outcomes are poor in cases of vulvoperineal Crohn disease, and treatment includes oral metronidazole, corticosteroid, or corticosteroid sparing agents such as azathioprine (Azasan) or infliximab.35 Some patients with vulvoperineal Crohn disease experience spontaneous healing, whereas others experience lesions that are refractory to medical therapy and require partial or total vulvectomy.38-40
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