From ATLANTA Medicine, Vol. 85, No. 5
While primarily thought of as an outpatient specialty, the practice of dermatology also plays a vital role in certain hospital settings. By providing assistance for an efficient assessment, application of diagnostic studies and suggestion of treatment plans for cutaneous disease, the dermatologist can be a valuable asset to medical and surgical teams. Recognition of cutaneous manifestations of systemic disease is central to the consultant dermatologist’s role and adds invaluable insight into perplexing diagnostic cases.
The following are clinical pearls relating to common dermatologic manifestations found in the inpatient setting. (For a discussion of serious skin eruptions secondary to medication reactions, see page 10.)
Erythema, or redness of the skin, can have various presentations. Examples of causes leading to erythema include toxin-mediated erythema (bacterial/viral infectious etiology vs medication), Graft vs Host Disease (GvHD) or Kawasaki disease. Morbilliform eruptions (measles-like eruptions) are commonly due to drug eruptions, viral exanthems and GvHD. However, disseminated deep fungal infections such as histoplasmosis, cryptococcosis, and coccidiomycosis can also mimic the morbilliform pattern.
Erythroderma is defined as full-body erythema associated with skin scaling, also known as exfoliative dermatitis. There are numerous common and rare causes for erythroderma, such as drug reactions, psoriasis, cutaneous T-cell lymphoma (CTCL), Sézary syndrome, atopic dermatitis, pityriasis rubra pilaris (PRP), systemic lupus erythematosus, pemphigus foliaceus, pemphigus vulgaris, seborrheic dermatitis, cutaneous manifestations of reactive arthritis, atypical pityriasis rosea, lichen planus, GvHD, diffuse histoplasmosis and nutritional disorders. Though literature reports suggest approximately 25 percent of erythroderma may also be idiopathic, some of these patients go on to develop CTCL and therefore should be monitored closely.
Management that is usually warranted in these cases includes a full body skin examination and diagnostic skin biopsies by a dermatologist for the underlying etiology. Diagnostic laboratory workup may be necessary on a case-by-case basis, but baseline labs – such as a complete blood count with differential, comprehensive metabolic panel and urine studies – are usually warranted at the time of the dermatology consultation.
Erythroderma is best managed in the inpatient setting, as these patients are prone to life-threatening systemic disorders such as thermodysregulation from insensible water and protein loss, peripheral edema and tachycardia. Meticulous nursing care is of the utmost importance, as patients are also prone to skin breakdown and sepsis. Patients may also benefit from occlusion suits or extremity wraps over application of topical steroids. Communication between the hospitalist team, nursing team and the consultant dermatologist should be clear because of the complexity of care required.
A great mimic of bilateral lower extremity “cellulitis” is acute venous congestion and venous stasis dermatitis. Patients with congestive heart failure, kidney dysfunction, hepatic disease, vascular disease and/or diabetes are more prone to this noninfectious cause of bilateral lower extremity erythema and edema. Supportive care, leg elevation, compression and treatment of the underlying systemic disease are recommended. Vascular surgery input may be necessary depending upon the clinical picture.
Cellulitic-like plaques that are a cause for concern include carcinoma erysipeloides, deep fungal infections (i.e. cryptococcus), and acute neutrophilic dermatoses (aka Sweet syndrome). If plaques are ulcerating and/or not responding to standard antibiotic treatment, especially in the immunocompromised patient, these other etiologies need be considered, which would require skin biopsies.
Necrotizing fasciitis, commonly known as flesh-eating bacteria, is obviously a life-threatening emergency and classically described as pain out of proportion to clinical exam with rapidly progressing edema, erythema, overlying bullae, cyanosis and eventually gangrene. Emergent surgical consultation for evaluation and treatment is necessary, with diagnostic blood cultures and tissue cultures at time of surgical debridement.
Other differential diagnoses that may appear to be similar to a “deep cellulitis” include panniculitis, diabetic muscle infarction and pyomyositis. A low threshold for radiologic imaging must be used as these can be quite serious and painful. Panniculitides may also need additional biopsies to elicit an etiology, based on the clinical exam.
A presentation of generalized vesicles and bullae can be quite alarming, as this can represent serious infections or autoimmune blistering conditions. Varicella is one of the most common infectious causes of generalized vesicles, with the appearance of a classic “dew drop on a rose petal” appearance. Although this is classically seen in pediatrics, with the advent of varicella immunization, it is now common to see cases in adults that were once vaccinated. In patients who were vaccinated, it is common to see “abortive” cases of varicella, a milder presentation with shorter duration. Patients who are immunocompromised are also at risk for generalized varicella, even if they have already had primary varicella. Furthermore in pediatric patients, aspirin is an absolute contraindication in varicella cases as this may lead to Reye Syndrome.
Herpes simplex virus can also have increase morbidity and mortality in certain situations. Lesions around the eye can lead to herpetic keratoconjunctivitis, which may lead to scarring and vision loss. In these cases, an urgent ophthalmology consult is warranted. Eczema herpeticum is a condition in which herpes simplex disseminates in a generalized distribution on compromised skin such as atopic dermatitis, pemphigus, Darier’s disease (DAR) or on burn patients. Treatment with oral or IV antiviral medication, depending on the extent of surface area involved and immune status, with meticulous wound care is necessary in these patients. Herpetic encephalitis is a severe complication of herpes when it affects the temporal lobes, presenting as decreased level of consciousness, seizures and fevers.
Autoimmune blistering conditions usually present as numerous and larger bullae and commonly require special diagnostic biopsies for confirmation of diagnosis, such as a direct immunofluorescence (DIF) skin biopsy. Bullous pemphigoid (BP) is the most common autoimmune blistering condition with large tense bullae and typically presents in the elderly. Occasionally, urticarial plaques may precede the bullous stage of BP.
Components of the junctional adhesion complex within the skin and mucosa are targeted by specific circulating autoantibodies. There can be significant morbidity due to skin breakdown and resultant infection. Treatment is usually a combination of topical and systemic medications; these cases may necessitate a variety of immunosuppression, from corticosteroids to long-term steroid sparing agents.
Pemphigus vulgaris and pemphigus foliaceous are other autoimmune blistering conditions in which the autoantibodies are directed at cell adhesion proteins in the skin and sometimes the mucosa. These are more superficial than BP. Therefore, these bullae are more flaccid and may not even be clinically present; widespread erosions may be the only evidence of pemphigus.
These patients are also at risk for significant morbidity due to skin breakdown and resultant infection, and a treatment plan may be similar to a BP patient. However, as pemphigus can have debilitating mucosal findings, otolaryngology and ophthalmology colleagues may need to be involved to prevent long-term mucosal scarring and strictures.
Linear IgA bullous disease is another blistering condition that may occur in the inpatient setting, as the adult form is essentially drug-induced, particularly in patients exposed to vancomycin. Penicillin, cephalosporins, ACE-inhibitors, and NSAIDs are also some well-known culprits.
It is thought that these medications stimulate a patient’s predisposed immune system to create IgA antibodies against specific proteins in the skin. Supportive care and withdrawal of culprit medications are key in management, with remission of eruption within two to six weeks of drug termination.
Cutaneous pustules are a manifestation of a spectrum of dermatologic disease from drug eruptions, psoriasis, insect bites, contact dermatitis and various infections. Generalized pustules may herald a case of generalized pustular psoriasis (von Zumbusch variant). This is considered a dermatologic emergency and may require inpatient monitoring and systemic immunosuppression with cyclosporine, acitretin, or methotrexate. Intravenous steroids must be avoided, as this can exacerbate pustular psoriasis.
Generalized pustules in an immunocompromised patient may also be caused by disseminated candidiasis. These lesions may first appear as numerous erythematous papules with pale centers, but typical pustular lesions may present later. The patient should also be evaluated by an ophthalmologist as eye findings, including candida endophthalmitis, can be present.
Disseminated gonococcal infection may also present as pustules in a febrile patient, but more classically localized over affected joints (i.e. knees, elbows, wrists, ankles). The pustules are larger, surrounded by erythema, and may be hemorrhagic. Gram stain cultures from the urethra, endocervical canal or posterior pharynx is usually the gold standard for diagnosis.
Papules on the skin are common and have an innumerable list of differential diagnoses; most can be handled appropriately in the outpatient setting. However, if there is either a papule or nodule of a deep violaceous color (known as a “purple plum”), the differential is more of an urgent matter as diagnoses such as cutaneous metastases, lymphomas, melanoma, sarcomas, vascular tumors and vascular infections are more concerning. A skin biopsy is diagnostic, but in cases of unusual tumors it may take special staining and outside dermatopathologic consultation for a confirmatory diagnosis.
Immunocompromised inpatients may also be at risk for scabies; classic lesions are typically pruritic pink to skin-colored small papules on volar wrists, finger webspaces, peri-areolar and peri-umbilical skin. Scrotal papules are pathognomonic for scabies and can sometimes be the only finding.
Crusted scabies is typical of the immunosuppressed and appears as marked hyperkeratosis, particularly of acral sites. If a patient is found to have crusted scabies, hospital infection control may need to be involved as it is likely to have spread to hospital staff and patients.
Purpura (palpable and retiform)
Palpable purpura is typically a small vessel vasculitis issue, which has an array of etiologies such as infection, medications, systemic inflammatory conditions and malignancy. Histology is important to confirm the diagnosis, but a DIF skin biopsy may also be obtained for further etiology.
Laboratory testing, such as complete blood count with differential, comprehensive metabolic panel, urine studies, fecal occult blood test and a hepatitis panel, may be necessary for a thorough systemic work-up. Other testing such as a RPR, ANA, HIV, SPEP, UPEP, RF, Total complement/C3/C4, ANCAs, cryoglobulins and an up-to-date age appropriate malignancy screening may also be necessary to tease out the etiology of the vasculitis if necessary.
Retiform purupra is more disturbing as it can quickly lead to necrosis of overlying skin. Embolization or thrombosis of vasculature can cause the distinct retiform (netlike) pattern via intraluminal occlusion (extraluminal occlusion may also occur). Diagnoses to strongly consider are calciphylaxis, cryoglobulinemia or cryofibrinogenemia, septic vasculitis, severe acute meningococcemia, levamisole exposure or a hypercoagulable state such as catastrophic antiphospholipid syndrome (CAPS). If one comes across periumbilical “thumbprint purpura” in an intensive care unit patient (usually on a respiratory vent), one must strongly consider hyperinfection of strongyloidiasis, which can quickly disseminate and lead to increased mortality. These cases are urgent and require quick diagnosis so treatment may be instituted.
Ulcers are commonly found in the inpatient setting and can be from a variety of conditions such as chronic venous insufficiency, a range of infectious etiologies and inflammatory conditions such as lichen planus or pyoderma gangrenosum. Chronic herpes simplex virus infections on the buttocks are very common in bedridden immunocompromised patients and should be cultured by the primary team. Ecthyma gangrenosum is another necrotic type of ulcer with raised erythematous borders classically associated with Pseudomonas aeruginosa bacteremia; these patients are critically ill and generally immunocompromised.
Pyoderma gangrenosum is a rare but chronic ulcerative disease that is usually associated with a variety of underlying systemic diseases. These can become large and painful with dusky borders and cribiform scarring; this requires a multi-disciplinary approach with the involvement of a wound care nurse for management. Chronic non-healing ulcers, especially in venous stasis wounds or old burn scars, may need to be evaluated for a Marjolin’s ulcer, a squamous cell carcinoma that arises in previously traumatized and/or chronically inflamed skin.
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