Clinicopathologic challenge
Multiple erythematous, shiny papules in a child Fernanda Ventin de Oliveira Prates, MD, Bruno de Oliveira Rocha, MD, and Juliana Dum^et Fernandes, MD, PhD
Department of Dermatology, Federal University of Bahia, Salvador, Bahia, Brazil
What is your diagnosis?
Correspondence ^t Fernandes, MD, PHD Juliana Dume ~o Garcez Fro es Street, 122 Dr. Joa Room 101 Ondina, Salvador Bahia Brazil E-mail: [email protected] Conflicts of interest: None.
History A 9-year-old boy presented with a 1-year history of lesions on the abdomen. Initially, there was only one lesion, which was excised, and after three months, a large number of lesions appeared, scattered around the original. There was no history of local trauma. Physical examination revealed bright erythematous, violaceous papule nodules distributed around a central cicatricial lesion on the abdomen (Fig. 1a,b). Histopathologic examination showed lobules of dilated and congested capillaries in an edematous stroma covered by an epithelial collarette (Fig. 2a–c). Immunohistochemistry was positive for CD31 and CD34 (Fig. 2d,e) and negative for GLUT-1. Complete blood count, urinalysis, and abdominal ultrasonography were normal. Serology for hepatitis viruses and retroviruses were negative.
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(a)
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Figure 1 (a) Bright erythematous–violaceous papule–nodules
distributed around a central cicatricial lesion on the abdomen. (b) A close view of the lesions ª 2014 The International Society of Dermatology
Figure 2 (a) Fragments of ulcerated skin showing a lesion with a multilobulated pattern of growth, consisting of cells without atypia, outlining vascular spaces. Hematoxylin and eosin stain; original magnification 940. (b,c) show a close view of the histological sample. Hematoxylin and eosin stain; original magnification: (a) 9100, (b) 9250. Immunohistochemical study reveals expression of CD31 (d) and CD34 (e). Original magnification 9250 International Journal of Dermatology 2014, 53, 1423–1424
1423
1424
Clinicopathologic challenge
Erythematous-shiny papules
Diagnosis Disseminated eruptive pyogenic granuloma (PG). Discussion Pyogenic granuloma (PG) is considered a hyperproliferative vascular response to a variety of stimuli.1–5 The first report of PG was made by Poncet and Dor in 1897,2,3 and later, Warner and Wilson-Jones in 1968 reported a series of patients who developed a localized cluster of PGs around the site of an original PG after its surgical removal (localized eruptive PG).1 Approximately one-third develop following local trauma,1,4,5 but infectious/hormonal stimuli, microscopic arteriovenous anastomoses, and angiogenic growth factors are all hypothetical etiological factors.1,3,4 There are reported cases of multiple PG developing after exfoliative dermatitis and during therapy with gefitinib, systemic 5-fluorouracil and capecitabine, systemic retinoids, indinavir, and anti-epidermal growth factor receptor antibodies.1,3–5 Eruptive PGs have also been reported in hematopoietic malignancies.1 In the present case, we could not identify any of these causative factors. The development of multiple satellite lesions at and around the site of a previously destroyed PG (disseminated eruptive PG), as in the present case, is considered an extremely rare event.2,5 This variant is not well-documented in the literature,3 but it is known that it usually occurs on the shoulders or upper trunk of children and, sometimes, in association with visceral disease.2,5 Radiological screening in our patient did not find any visceral disease. As classic PGs, disseminated eruptive PG presents as lobulated, pedunculated, or sessile benign vascular tumors but with the sudden appearance of multiple widespread lesions.1,5 Histopathologic features are capillary lobules separated by fibromyxoid stroma containing scattered spindle- and stellate-shaped connective tissue cells.2,4 A superficial lymphocyte-predominant inflammatory reaction can occur in ulcerated lesions,2,3 but inflammation does not seem to be an intrinsic feature.4 Collarette of acanthotic epidermis constricting the base of the lesion is a typical finding.2,5 Immunohistochemical studies demonstrate positive labeling of endothelial cells for factor VIII-related antigen, CD31, CD34, and of perithelial cells for muscle-specific actin and type IV collagen.3–5 The present case shows characteristic histopathology, with positive
International Journal of Dermatology 2014, 53, 1423–1424
Prates et al.
staining for CD31 and CD34. GLUT-1 negativity distinguished it from infantile hemangioma.6 Particularly for patients with multiple lesions, Kaposi’s sarcoma and angiosarcoma should be considered as differential diagnoses.3,5 In our patient, clinicopathological aspects allowed us to differentiate it. Elevated polypoid lesions are rare in Kaposi’s sarcoma, and a much greater degree of atypia is found in well-differentiated angiosarcoma.3 Bacillary angiomatosis is more frequent in HIV-infected patients with a history of contact with cats,1,5 which is not consistent with the present report. Treatment of PG can be done with surgical excision, curettage, sclerotherapy, cryotherapy, cautery, or laser.2–4 Lesions do not rarely recur and, if not removed, can persist indefinitely.2 The initial approach in our patient resulted in recurrence and the appearance of satellite lesions; however, excision with subsequent cauterization was successful. Finally, we emphasize that, although it is extremely rare, the eruptive PG should be considered as a diagnostic possibility in cases similar to which we report herein. Despite the benign nature, patient and family should be counseled about the possibility of relapse. References 1 Palmero ML, Pope E. Eruptive pyogenic granulomas developing after drug hypersensitivity reaction. J Am Acad Dermatol 2009; 60: 855–857. 2 Baselga E, Wassef M, Lopez S, et al. Agminated, eruptive pyogenic granuloma-like lesions developing over congenital vascular stains. Pediatr Dermatol 2012; 29: 186–190. 3 Ceyhan AM, Basak PY, Akkaya VB, et al. A case of multiple, eruptive pyogenic granuloma developed on a region of the burned skin: can erythromycin be a treatment option? J Burn Care Res 2007; 28: 754–757. 4 Wollina U. Multiple eruptive periungual pyogenic granulomas during anti-CD20 monoclonal antibody therapy for rheumatoid arthritis. J Dermatol Case Rep 2010; 19: 44–46. 5 Wilson BB, Greer KE, Cooper PH. Eruptive disseminated lobular capillary hemangioma (pyogenic granuloma). J Am Acad Dermatol 1989; 21: 391–394. 6 Villapalos JL, Wolfe K, Kangesu L. GLUT-1: an extra diagnostic tool to differentiate between haemangiomas and vascular malformations. Br Assoc Plast Surg 2005; 58: 348–352.
ª 2014 The International Society of Dermatology
Multiple erythematous, shiny papules in a child Fernanda Ventin de Oliveira Prates, MD, Bruno de Oliveira Rocha, MD, and Juliana Dum^et Fernandes, MD, PhD
Department of Dermatology, Federal University of Bahia, Salvador, Bahia, Brazil
What is your diagnosis?
Correspondence ^t Fernandes, MD, PHD Juliana Dume ~o Garcez Fro es Street, 122 Dr. Joa Room 101 Ondina, Salvador Bahia Brazil E-mail: [email protected] Conflicts of interest: None.
History A 9-year-old boy presented with a 1-year history of lesions on the abdomen. Initially, there was only one lesion, which was excised, and after three months, a large number of lesions appeared, scattered around the original. There was no history of local trauma. Physical examination revealed bright erythematous, violaceous papule nodules distributed around a central cicatricial lesion on the abdomen (Fig. 1a,b). Histopathologic examination showed lobules of dilated and congested capillaries in an edematous stroma covered by an epithelial collarette (Fig. 2a–c). Immunohistochemistry was positive for CD31 and CD34 (Fig. 2d,e) and negative for GLUT-1. Complete blood count, urinalysis, and abdominal ultrasonography were normal. Serology for hepatitis viruses and retroviruses were negative.
(a)
(a)
(b)
(c)
(d)
(e)
(b)
Figure 1 (a) Bright erythematous–violaceous papule–nodules
distributed around a central cicatricial lesion on the abdomen. (b) A close view of the lesions ª 2014 The International Society of Dermatology
Figure 2 (a) Fragments of ulcerated skin showing a lesion with a multilobulated pattern of growth, consisting of cells without atypia, outlining vascular spaces. Hematoxylin and eosin stain; original magnification 940. (b,c) show a close view of the histological sample. Hematoxylin and eosin stain; original magnification: (a) 9100, (b) 9250. Immunohistochemical study reveals expression of CD31 (d) and CD34 (e). Original magnification 9250 International Journal of Dermatology 2014, 53, 1423–1424
1423
1424
Clinicopathologic challenge
Erythematous-shiny papules
Diagnosis Disseminated eruptive pyogenic granuloma (PG). Discussion Pyogenic granuloma (PG) is considered a hyperproliferative vascular response to a variety of stimuli.1–5 The first report of PG was made by Poncet and Dor in 1897,2,3 and later, Warner and Wilson-Jones in 1968 reported a series of patients who developed a localized cluster of PGs around the site of an original PG after its surgical removal (localized eruptive PG).1 Approximately one-third develop following local trauma,1,4,5 but infectious/hormonal stimuli, microscopic arteriovenous anastomoses, and angiogenic growth factors are all hypothetical etiological factors.1,3,4 There are reported cases of multiple PG developing after exfoliative dermatitis and during therapy with gefitinib, systemic 5-fluorouracil and capecitabine, systemic retinoids, indinavir, and anti-epidermal growth factor receptor antibodies.1,3–5 Eruptive PGs have also been reported in hematopoietic malignancies.1 In the present case, we could not identify any of these causative factors. The development of multiple satellite lesions at and around the site of a previously destroyed PG (disseminated eruptive PG), as in the present case, is considered an extremely rare event.2,5 This variant is not well-documented in the literature,3 but it is known that it usually occurs on the shoulders or upper trunk of children and, sometimes, in association with visceral disease.2,5 Radiological screening in our patient did not find any visceral disease. As classic PGs, disseminated eruptive PG presents as lobulated, pedunculated, or sessile benign vascular tumors but with the sudden appearance of multiple widespread lesions.1,5 Histopathologic features are capillary lobules separated by fibromyxoid stroma containing scattered spindle- and stellate-shaped connective tissue cells.2,4 A superficial lymphocyte-predominant inflammatory reaction can occur in ulcerated lesions,2,3 but inflammation does not seem to be an intrinsic feature.4 Collarette of acanthotic epidermis constricting the base of the lesion is a typical finding.2,5 Immunohistochemical studies demonstrate positive labeling of endothelial cells for factor VIII-related antigen, CD31, CD34, and of perithelial cells for muscle-specific actin and type IV collagen.3–5 The present case shows characteristic histopathology, with positive
International Journal of Dermatology 2014, 53, 1423–1424
Prates et al.
staining for CD31 and CD34. GLUT-1 negativity distinguished it from infantile hemangioma.6 Particularly for patients with multiple lesions, Kaposi’s sarcoma and angiosarcoma should be considered as differential diagnoses.3,5 In our patient, clinicopathological aspects allowed us to differentiate it. Elevated polypoid lesions are rare in Kaposi’s sarcoma, and a much greater degree of atypia is found in well-differentiated angiosarcoma.3 Bacillary angiomatosis is more frequent in HIV-infected patients with a history of contact with cats,1,5 which is not consistent with the present report. Treatment of PG can be done with surgical excision, curettage, sclerotherapy, cryotherapy, cautery, or laser.2–4 Lesions do not rarely recur and, if not removed, can persist indefinitely.2 The initial approach in our patient resulted in recurrence and the appearance of satellite lesions; however, excision with subsequent cauterization was successful. Finally, we emphasize that, although it is extremely rare, the eruptive PG should be considered as a diagnostic possibility in cases similar to which we report herein. Despite the benign nature, patient and family should be counseled about the possibility of relapse. References 1 Palmero ML, Pope E. Eruptive pyogenic granulomas developing after drug hypersensitivity reaction. J Am Acad Dermatol 2009; 60: 855–857. 2 Baselga E, Wassef M, Lopez S, et al. Agminated, eruptive pyogenic granuloma-like lesions developing over congenital vascular stains. Pediatr Dermatol 2012; 29: 186–190. 3 Ceyhan AM, Basak PY, Akkaya VB, et al. A case of multiple, eruptive pyogenic granuloma developed on a region of the burned skin: can erythromycin be a treatment option? J Burn Care Res 2007; 28: 754–757. 4 Wollina U. Multiple eruptive periungual pyogenic granulomas during anti-CD20 monoclonal antibody therapy for rheumatoid arthritis. J Dermatol Case Rep 2010; 19: 44–46. 5 Wilson BB, Greer KE, Cooper PH. Eruptive disseminated lobular capillary hemangioma (pyogenic granuloma). J Am Acad Dermatol 1989; 21: 391–394. 6 Villapalos JL, Wolfe K, Kangesu L. GLUT-1: an extra diagnostic tool to differentiate between haemangiomas and vascular malformations. Br Assoc Plast Surg 2005; 58: 348–352.
ª 2014 The International Society of Dermatology
