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Uncommon manifestations of erysipelas
Clinics in Dermatology (2005) 23, 515 – 518
Uncommon manifestations of erysipelas La´szlo´ To¨ro¨k, MD* Department of Dermatology, County Hospital, 6000 Kecskeme´t, Hungary Abstract Erysipelas is a common infection of soft tissues by streptococci or Staphylococcus aureus. Uncommon manifestations of erysipelas include atypical localizations, unusual spreading of lesions, and atypical clinical morphology or course of the disease including the absence of general signs. D 2005 Elsevier Inc. All rights reserved.
Introduction Erysipelas is the most frequent infection of the soft tissues caused by group A (occasionally group C or G) b-hemolytic streptococci or Staphylococcus aureus. Erysipelas is a superficial dermal infection that also affects the regional lymph nodes. High amounts of streptococci penetrate into the interstitium and into the subcutaneous lymphatics; the spreading inflammatory reaction is mediated by neutrophil leukocytes. The disease has been known since the time of Hippocrates, whose accurate description is still valid. Erysipelas is a bright-red, flamelike, spreading, superficial, edematous lesion with sharply demarcated edges. These signs, as well as fever and general symptoms, confirm the diagnosis. Erysipelas has a particular predilection for the lower extremities and the face. The process is typically unilateral. Clinical manifestations of erysipelas include vesiculous, bullous, pustulous, hemorrhagic, absceding, and phlegmonous (necrotizing fasciitis) forms.1,2 The manifestation, course, and complications of erysipelas depend on the immune status of the patient, the factors localizing infection, as well as the virulence of the pathogens. Severe and unusual forms of erysipelas commonly occur in immunocompromised patients. Diabetes
T Corresponding author. Tel.: +36 76 485 511; fax: +36 76 485 887. E-mail address: [email protected] (L. To¨ro¨k). 0738-081X/$ – see front matter D 2005 Elsevier Inc. All rights reserved. doi:10.1016/j.clindermatol.2005.01.005
mellitus, renal insufficiency, nephritic syndrome, malnutrition, and alcohol and drug abuse account for the severe course of the disease. Furthermore, carcinomas and chemotherapy, HIV infection, and immunosuppressive therapy are also noteworthy. Rapid progression and life-threatening complications of erysipelas are characteristic of the elderly. Mitigated (afebrile) forms of the disease, however, are documented in the elderly as well. Among localizing factors impaired regional circulation (eg, venous edema, lymphostasis, lymphedema), lipoedema, surgical interventions and scars, and active or passive inflammatory foci (locus minoris resistentiae) should be stressed. Uncommon manifestations of erysipelas include atypical localization, unusual large spreading of the lesions, and atypical morphology or course of the disease, as well as the absence of general symptoms.
Atypical localizations Erysipelas of the ear occurs relatively rarely. Bilateral erysipelas can develop as a sequel of ear-picking in patients with otitis externa. Another rare form, erysipelas of the hands, can be caused by interdigital mycotic erosions or fissures (Fig. 1). Mucosal erysipelas primarily affecting the pharynx (erysipelatous angina) and the larynx (larynx erysipelas) are very rare forms of the disease and can cause laryngeal edema. This form of erysipelas occurred in
L. To¨ro¨k
516
Fig. 1
Hemorrhagic erysipelas of the hand.
patients during the preantibiotic era. A typical ipsilateral erysipelas of the arm can develop after mastectomy even without lymphedema and intact regional lymph nodes. Scarring after saphenous venectomy for the coronary artery bypass surgery is another interesting new localizing factor (Fig. 2). Erysipelas of the limb and thigh surrounding surgical scars occurs in 4% to 6% of patients operated for coronary artery bypass. As a rule, infection develops within 8 months (range, 3-84 months) after surgery. Impaired postoperative lymphatic drainage and streptococci penetrating into the skin (interdigital fissures), which are accumulated in the scar, all contribute to the pathogenesis of erysipelas after bypass surgery.3
Fig. 2 Erysipelas in the scar after saphenous venectomy after coronary artery bypass surgery.
superficial and deeper structures, and is associated with a high risk of necrotizing fasciitis (Fig. 6). Erysipelas with atypical localization with relapses at the same site suggests persisting focus of the infection
Extended spreading These forms of erysipelas can develop after block dissection following mastectomy, especially in patients with bilateral breast cancer. The infection affects the whole trunk and the arms of the patient (Fig. 3). Bilateral vulvectomy and bilateral block dissection in patients with vulvar carcinoma can be in the background of a clinically typical erysipelas spreading on both gluteal areas and lower extremities (Fig. 4). In chronic infections of the major joints and the adjacent bones (hip joint osteomyelitis), erysipelas can spread to the whole gluteal area and the thigh (Fig. 5). Because of intertriginous maceration, erysipelas of the proximal parts of the abdomen and the thigh, localized to the flexures, occurs among obese patients with diabetes mellitus. The infection rapidly spreads, affecting both
Fig. 3 Erysipelas on the left side of the trunk after mastectomy and axillary block dissection.
Uncommon manifestations of erysipelas
517
Fig. 5
Erysipelas after hip join osteomyelitis.
newborn, the poorer is the prognosis and the outcome. The above statement is especially noteworthy in cases of perianal erysipelas and infection of areas above major joints.
Coexistence with other dermatoses
Fig. 4 A, B, Erysipelas after bilateral vulvectomy and bilateral inguinal block dissection.
(eg, impaired lymphatic drainage, old scars, torpid infectious foci).
Atypical clinical picture and course of the infection
Comorbidity might alter the individual clinical picture of each or both diseases. Ipsilateral occurrence of herpes zoster in patients after mastectomy is a triggering factor for extensive ipsilateral erysipelas. In one case, we have observed a unilateral extensive erysipelas associated with a segmental herpes zoster developing in the area of erysipelas (Fig. 7). Erysipelas is common among patients with leg ulcer. It is well-known that after erysipelas the ulcers heal more rapidly. The role of lymphedema has been stressed earlier.
At sites with loose subcutis susceptible of marked edema (predominantly periorbital and genital areas), an extensive distorting edema is the leading clinical feature of the infection. As mentioned above, elderly enfeebled patients fail to develop dramatic general symptoms (ie, fever) and inflammatory reaction typical of erysipelas.
Atypical age Because of its rapid progression and spreading, erysipelas among infants and young children is dangerous and its prognosis is poor (erysipelas migrans). The younger is the
Fig. 6 Erysipelas of the inguinal fossa with abdominal spreading in a patient with diabetes.
L. To¨ro¨k
518
doerysipelas usually occurs in patients with breast cancer7 and, rarely, in malignancies of the skin (melanoma: melanoma erysipelatoides8) or the internal organs (uterus, colon, rectum, bladder, lung, stomach, pancreas).9 Via the lymphatics, this pseudoerysipelas shows cutaneous spreading (per continuitatem), and its clinical picture resembles erysipelas. Differentiation is based on the histological findings that reveal typical signs of tumor propagation (inflammatory carcinoma). Afebrility, lack of general symptoms, and no improvement upon administration of antibiotics are helpful in differentiation. The diagnosis is further confirmed by biopsy from the lesion, that is, by identification of the tumor cells in the lymphatics. Recently, an erysipeloid inflammation of the extremities (elbow and leg) lasting several days has been documented after administration of a nucleoside analogue gemcitabine. This reaction might be mediated by an assumed cutaneous toxicity of gemcitabine.10
Conclusions Fig. 7 Relapsing erysipelas with extensive spreading after mastectomy associated with herpes zoster.
Uncommon complications If not treated adequately, erysipelas caused by chronic edema of the limbs can either relapse or turn into multifocal necrotizing cellulitis affecting the subcutis; as a rule, the latter complication develops in the lower extremities. High tissue pressure and congestion both enhance bacterial sequestration in the deeper tissues; bacterial accumulation can lead to a circumscribed necrosis of the adipose tissue, which gives rise to new proximal metastatic foci of necrosis.4 Necrotizing fasciitis can be a sequel of the above process. Apart from the well-known complications of erysipelas (lymphedema), mucinosis can sometimes develop at the site of erysipelas (erysipelas-associated mucinosis). This recently observed complication results from the local damages of the lymphatic circulation. Recognition of erysipelas associated with mucinosis is important in terms of therapy, as it mimics erysipelas with long course.5
Differential diagnosis Uncommon manifestations of erysipelas should be differentiated from facial and periorbital cellulitis, contact eczema, herpes zoster, and relapsing chondritis and perichondritis of the ear. Because cellulitis is an infection of the deeper layers of the skin, the lesions are more compact at palpation and their edges are blurred.6 Carcinoma erysipeloids (inflammatory carcinoma) can mimic the macromorphological picture of erysipelas. Pseu-
In conclusion, recognition of diseases with atypical uncommon course and manifestation is crucial for an early diagnosis and onset of adequate therapy. Furthermore, it helps to better understand pathology in the background of atypical manifestation. Diagnostic approach to uncommon clinical forms expands and enriches our understanding of the disease, helps to prevent complications, and is also a useful tool to test our knowledge.
References 1. Chiller K, Selkin BA, Murakawa GJ. Skin microflora and bacterial infections of the skin. J Investig Dermatol Symp Proc 2001;3:170 - 4. 2. Bonnetblanc JM, Bedane C. Erysipelas: recognition and management. Am J Clin Dermatol 2003;3:157 - 63. 3. Karakas M, Baba M, Aksungur VL, et al. Manifestation of cellulitis after saphenous venectomy for coronary bypass surgery. J Eur Acad Dermatol Venereol 2002;16:438 - 40. 4. Vocks E, Ring J. Multifokale nekrotisierende Zellulitis als Komplikation eines Erysipels bei chronischem Lymphfdem. Akt Dermatol 1999;25:190 - 4. 5. Fischer M, Benndorf K, Drunkenmflle E, et al. Localized mucinosis subsequent to erysipelas. J Eur Acad Dermatol Venereol 2004; 18:99 - 117. 6. Reis MD, Freitas JP, Coutinho VS, et al. Facial and periorbital cellulitis with orbital involvement. J Eur Acad Dermatol Venereol 2002;16:156 - 8. 7. Meadows KP, Egan CA. Vesicular carcinoma erysipelatodes. J Am Acad Dermatol 1999;40:805 - 7. 8. Tfrfk L, Ta´pai M. Entzqndungsreaktion beim Melanoma. Z Hautkr 1993;7:459 - 62. 9. Ellen S, Cruz PD. A spectrum of inflammatory metastasis to skin via lymphatics. Three cases of carcinoma erysipeloides. J Am Acad Dermatol 1994;30:304 - 7. 10. Kuku I, Kaya E, Servinc A, et al. Gemcitabine-induced erysipeloid skin lesions in a patient with malignant mesothelioma. J Eur Acad Dermatol Venereol 2002;16:271 - 2.
Uncommon manifestations of erysipelas La´szlo´ To¨ro¨k, MD* Department of Dermatology, County Hospital, 6000 Kecskeme´t, Hungary Abstract Erysipelas is a common infection of soft tissues by streptococci or Staphylococcus aureus. Uncommon manifestations of erysipelas include atypical localizations, unusual spreading of lesions, and atypical clinical morphology or course of the disease including the absence of general signs. D 2005 Elsevier Inc. All rights reserved.
Introduction Erysipelas is the most frequent infection of the soft tissues caused by group A (occasionally group C or G) b-hemolytic streptococci or Staphylococcus aureus. Erysipelas is a superficial dermal infection that also affects the regional lymph nodes. High amounts of streptococci penetrate into the interstitium and into the subcutaneous lymphatics; the spreading inflammatory reaction is mediated by neutrophil leukocytes. The disease has been known since the time of Hippocrates, whose accurate description is still valid. Erysipelas is a bright-red, flamelike, spreading, superficial, edematous lesion with sharply demarcated edges. These signs, as well as fever and general symptoms, confirm the diagnosis. Erysipelas has a particular predilection for the lower extremities and the face. The process is typically unilateral. Clinical manifestations of erysipelas include vesiculous, bullous, pustulous, hemorrhagic, absceding, and phlegmonous (necrotizing fasciitis) forms.1,2 The manifestation, course, and complications of erysipelas depend on the immune status of the patient, the factors localizing infection, as well as the virulence of the pathogens. Severe and unusual forms of erysipelas commonly occur in immunocompromised patients. Diabetes
T Corresponding author. Tel.: +36 76 485 511; fax: +36 76 485 887. E-mail address: [email protected] (L. To¨ro¨k). 0738-081X/$ – see front matter D 2005 Elsevier Inc. All rights reserved. doi:10.1016/j.clindermatol.2005.01.005
mellitus, renal insufficiency, nephritic syndrome, malnutrition, and alcohol and drug abuse account for the severe course of the disease. Furthermore, carcinomas and chemotherapy, HIV infection, and immunosuppressive therapy are also noteworthy. Rapid progression and life-threatening complications of erysipelas are characteristic of the elderly. Mitigated (afebrile) forms of the disease, however, are documented in the elderly as well. Among localizing factors impaired regional circulation (eg, venous edema, lymphostasis, lymphedema), lipoedema, surgical interventions and scars, and active or passive inflammatory foci (locus minoris resistentiae) should be stressed. Uncommon manifestations of erysipelas include atypical localization, unusual large spreading of the lesions, and atypical morphology or course of the disease, as well as the absence of general symptoms.
Atypical localizations Erysipelas of the ear occurs relatively rarely. Bilateral erysipelas can develop as a sequel of ear-picking in patients with otitis externa. Another rare form, erysipelas of the hands, can be caused by interdigital mycotic erosions or fissures (Fig. 1). Mucosal erysipelas primarily affecting the pharynx (erysipelatous angina) and the larynx (larynx erysipelas) are very rare forms of the disease and can cause laryngeal edema. This form of erysipelas occurred in
L. To¨ro¨k
516
Fig. 1
Hemorrhagic erysipelas of the hand.
patients during the preantibiotic era. A typical ipsilateral erysipelas of the arm can develop after mastectomy even without lymphedema and intact regional lymph nodes. Scarring after saphenous venectomy for the coronary artery bypass surgery is another interesting new localizing factor (Fig. 2). Erysipelas of the limb and thigh surrounding surgical scars occurs in 4% to 6% of patients operated for coronary artery bypass. As a rule, infection develops within 8 months (range, 3-84 months) after surgery. Impaired postoperative lymphatic drainage and streptococci penetrating into the skin (interdigital fissures), which are accumulated in the scar, all contribute to the pathogenesis of erysipelas after bypass surgery.3
Fig. 2 Erysipelas in the scar after saphenous venectomy after coronary artery bypass surgery.
superficial and deeper structures, and is associated with a high risk of necrotizing fasciitis (Fig. 6). Erysipelas with atypical localization with relapses at the same site suggests persisting focus of the infection
Extended spreading These forms of erysipelas can develop after block dissection following mastectomy, especially in patients with bilateral breast cancer. The infection affects the whole trunk and the arms of the patient (Fig. 3). Bilateral vulvectomy and bilateral block dissection in patients with vulvar carcinoma can be in the background of a clinically typical erysipelas spreading on both gluteal areas and lower extremities (Fig. 4). In chronic infections of the major joints and the adjacent bones (hip joint osteomyelitis), erysipelas can spread to the whole gluteal area and the thigh (Fig. 5). Because of intertriginous maceration, erysipelas of the proximal parts of the abdomen and the thigh, localized to the flexures, occurs among obese patients with diabetes mellitus. The infection rapidly spreads, affecting both
Fig. 3 Erysipelas on the left side of the trunk after mastectomy and axillary block dissection.
Uncommon manifestations of erysipelas
517
Fig. 5
Erysipelas after hip join osteomyelitis.
newborn, the poorer is the prognosis and the outcome. The above statement is especially noteworthy in cases of perianal erysipelas and infection of areas above major joints.
Coexistence with other dermatoses
Fig. 4 A, B, Erysipelas after bilateral vulvectomy and bilateral inguinal block dissection.
(eg, impaired lymphatic drainage, old scars, torpid infectious foci).
Atypical clinical picture and course of the infection
Comorbidity might alter the individual clinical picture of each or both diseases. Ipsilateral occurrence of herpes zoster in patients after mastectomy is a triggering factor for extensive ipsilateral erysipelas. In one case, we have observed a unilateral extensive erysipelas associated with a segmental herpes zoster developing in the area of erysipelas (Fig. 7). Erysipelas is common among patients with leg ulcer. It is well-known that after erysipelas the ulcers heal more rapidly. The role of lymphedema has been stressed earlier.
At sites with loose subcutis susceptible of marked edema (predominantly periorbital and genital areas), an extensive distorting edema is the leading clinical feature of the infection. As mentioned above, elderly enfeebled patients fail to develop dramatic general symptoms (ie, fever) and inflammatory reaction typical of erysipelas.
Atypical age Because of its rapid progression and spreading, erysipelas among infants and young children is dangerous and its prognosis is poor (erysipelas migrans). The younger is the
Fig. 6 Erysipelas of the inguinal fossa with abdominal spreading in a patient with diabetes.
L. To¨ro¨k
518
doerysipelas usually occurs in patients with breast cancer7 and, rarely, in malignancies of the skin (melanoma: melanoma erysipelatoides8) or the internal organs (uterus, colon, rectum, bladder, lung, stomach, pancreas).9 Via the lymphatics, this pseudoerysipelas shows cutaneous spreading (per continuitatem), and its clinical picture resembles erysipelas. Differentiation is based on the histological findings that reveal typical signs of tumor propagation (inflammatory carcinoma). Afebrility, lack of general symptoms, and no improvement upon administration of antibiotics are helpful in differentiation. The diagnosis is further confirmed by biopsy from the lesion, that is, by identification of the tumor cells in the lymphatics. Recently, an erysipeloid inflammation of the extremities (elbow and leg) lasting several days has been documented after administration of a nucleoside analogue gemcitabine. This reaction might be mediated by an assumed cutaneous toxicity of gemcitabine.10
Conclusions Fig. 7 Relapsing erysipelas with extensive spreading after mastectomy associated with herpes zoster.
Uncommon complications If not treated adequately, erysipelas caused by chronic edema of the limbs can either relapse or turn into multifocal necrotizing cellulitis affecting the subcutis; as a rule, the latter complication develops in the lower extremities. High tissue pressure and congestion both enhance bacterial sequestration in the deeper tissues; bacterial accumulation can lead to a circumscribed necrosis of the adipose tissue, which gives rise to new proximal metastatic foci of necrosis.4 Necrotizing fasciitis can be a sequel of the above process. Apart from the well-known complications of erysipelas (lymphedema), mucinosis can sometimes develop at the site of erysipelas (erysipelas-associated mucinosis). This recently observed complication results from the local damages of the lymphatic circulation. Recognition of erysipelas associated with mucinosis is important in terms of therapy, as it mimics erysipelas with long course.5
Differential diagnosis Uncommon manifestations of erysipelas should be differentiated from facial and periorbital cellulitis, contact eczema, herpes zoster, and relapsing chondritis and perichondritis of the ear. Because cellulitis is an infection of the deeper layers of the skin, the lesions are more compact at palpation and their edges are blurred.6 Carcinoma erysipeloids (inflammatory carcinoma) can mimic the macromorphological picture of erysipelas. Pseu-
In conclusion, recognition of diseases with atypical uncommon course and manifestation is crucial for an early diagnosis and onset of adequate therapy. Furthermore, it helps to better understand pathology in the background of atypical manifestation. Diagnostic approach to uncommon clinical forms expands and enriches our understanding of the disease, helps to prevent complications, and is also a useful tool to test our knowledge.
References 1. Chiller K, Selkin BA, Murakawa GJ. Skin microflora and bacterial infections of the skin. J Investig Dermatol Symp Proc 2001;3:170 - 4. 2. Bonnetblanc JM, Bedane C. Erysipelas: recognition and management. Am J Clin Dermatol 2003;3:157 - 63. 3. Karakas M, Baba M, Aksungur VL, et al. Manifestation of cellulitis after saphenous venectomy for coronary bypass surgery. J Eur Acad Dermatol Venereol 2002;16:438 - 40. 4. Vocks E, Ring J. Multifokale nekrotisierende Zellulitis als Komplikation eines Erysipels bei chronischem Lymphfdem. Akt Dermatol 1999;25:190 - 4. 5. Fischer M, Benndorf K, Drunkenmflle E, et al. Localized mucinosis subsequent to erysipelas. J Eur Acad Dermatol Venereol 2004; 18:99 - 117. 6. Reis MD, Freitas JP, Coutinho VS, et al. Facial and periorbital cellulitis with orbital involvement. J Eur Acad Dermatol Venereol 2002;16:156 - 8. 7. Meadows KP, Egan CA. Vesicular carcinoma erysipelatodes. J Am Acad Dermatol 1999;40:805 - 7. 8. Tfrfk L, Ta´pai M. Entzqndungsreaktion beim Melanoma. Z Hautkr 1993;7:459 - 62. 9. Ellen S, Cruz PD. A spectrum of inflammatory metastasis to skin via lymphatics. Three cases of carcinoma erysipeloides. J Am Acad Dermatol 1994;30:304 - 7. 10. Kuku I, Kaya E, Servinc A, et al. Gemcitabine-induced erysipeloid skin lesions in a patient with malignant mesothelioma. J Eur Acad Dermatol Venereol 2002;16:271 - 2.