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Correspondence
3 Murakami T, Fukai K, Oiso N, et al. New KIT mutations in patients with piebaldism. J Dermatol Sci 2004; 35: 29–33. 4 Matsunaga H, Tanioka M, Utani A, et al. Familial case of piebaldism with regression of white forelock. Clin Exp Dermatol 2008; 33: 511–512. 5 Arase N, Wataya-Kaneda M, Oiso N, et al. Repigmentation of leukoderma in a piebald patient associated with a novel c-KIT gene mutation, G592E, of the tyrosine kinase domain. J Dermatol Sci 2011; 64: 147–149. 6 Makino T, Yanagihara M, Oiso N, et al. Repigmentation of the epidermis around the acrosyringium in piebald skin: an ultrastructural examination. Br J Dermatol 2013; 168: 910–912.
A case of nasal glial heterotopia with complete excision
Nasal glial heterotopia (also known as nasal glioma) is a rare developmental abnormality typically present at birth or in early childhood, but there have been reported cases in a wider age group.1 Nasal glial heterotopia is a rare condition that is thought to originate from either entrapped neuroectodermal tissue during the closure of the covering of the brain or nasal encephalocele, which is covered by dura, pia, and arachnoid and later disconnected from the intracranial cavity. We report a rare case of nasal glial heterotopia. A 1-year-old male infant visited our clinic for evaluation of a congenital tumor on the nose. On examination, the tumor was a 1 9 1 cm, skin-colored, firm, mobile, (a)
and nonpulsatile subcutaneous mass located on the upper part of the right side of the nose (Fig. 1). Doppler ultrasonography revealed a solid mass without vascular proliferation. Facial computed tomography (CT) demonstrated a 1 cm sized polypoid high-attenuation mass in the subcutaneous tissue of the right side of the nose without intracranial extension. The tumor was excised externally. Histologic examination revealed a prominent dermal and subcutaneous neural proliferation composed of astrocytes, neurons, and neuroglial fibers intermixed with a fibrous connective tissue stroma (Fig. 2a,b). Immunohistochemical staining for glial fibrillary acidic protein confirmed the presence of the extensive glial tissue (Fig. 2c). After the excision, there was no recurrence during the 1-year follow-up period. Based on the clinical and histopathological findings, a final diagnosis of nasal glial heterotopia was made. Glial heterotopia is a rare condition composed of mature brain tissue isolated from the cranial cavity or spinal canal. The nose and nasopharynx are the most common locations for glial heterotopia. Nasal glioma may manifest as an extranasal (60%), intranasal (30%), or combined (10%) lesion.2 Vilarinho et al.3 reported an extranasal glial heterotopia located on the nasal bridge. Riffaud et al.4 reported a case of nasal glial heterotopia located on the right paranasal region. Magnetic resonance imaging (MRI) of the head was conducted in both cases, which showed no internal connection, and they were surgically excised and diagnosed with nasal glial heterotopia without recurrence and complication.3,4
(b) Figure 1 (a) Firm, well demarcated, and movable nodule on the right side of the nose. (b) Doppler ultrasonography: lobulated and heterogeneously hyperechoic lesion (1.1 9 1.4 cm) with some hypoechoic portion in the subcutaneous layer of right nose and no definite increased internal vascularity
(a)
(b)
(c) Figure 2 (a) Subcutaneous nodule (hematoxylin and eosin stain 9 40). (b) Astrocytes, neurons, and neuroglial fibers intermixed with a fibrous connective tissue stroma (hematoxylin and eosin stain 9 400). (c) Immunohistochemistry using antibody against glial fibrillary acidic protein highlights strongly stained neuroglial tissue
International Journal of Dermatology 2015, 54, e233–e249
ª 2015 The International Society of Dermatology
Correspondence
The differential diagnosis includes hemangiomas, dermoid cysts, encephaloceles, and malignant tumors.2 Imaging studies must be performed before surgery to exclude other diagnoses and determine appropriate surgical margin.3,5 Biopsy should be avoided because procedures could result in meningitis. The MRI and CT are useful to exclude encephalocele and other congenital vascular tumors. MRI is known to be the most useful imaging approach to identify the intracranial connection of a nasal tumor, but the cost is very high.6,7 In this case, Doppler ultrasonography and facial CT helped to exclude hemangioma and the possibility of connection to intracranial extension. Histologically, nasal glial heterotopia and encephaloceles are characterized by varying proportions of astrocytes and neuroglial fibers associated with fibrous connective tissue.1,8 Complete surgical excision is the treatment of choice in nasal glial heterotopia. A delay in treatment can result in deformation of the septum and nasal bone, or airway obstruction. Recurrence after complete excision is rare.
Hye Rang On, MD Jimyung Seo, MD Kee Yang Chung, MD, PhD Department of Dermatology and Cutaneous Biology Research Institute Yonsei University College of Medicine Seoul Korea E-mail: [email protected]
References 1 Penner CR, Thompson L. Nasal glial heterotopia: a clinicopathologic and immunophenotypic analysis of 10 cases with a review of the literature. Ann Diagn Pathol 2003; 7: 354–359. 2 Jaffe BF. Classification and management of anomalies of the nose. Otolaryngol Clin North Am 1981; 14: 989–1004. 3 Vilarinho C, Ventura F, Vieira AP, et al. Nasal glial heterotopia in a newborn infant. Int J Dermatol 2009; 48: 1225–1227. 4 Riffaud L, Ndikumana R, Azzis O, Cadre B. Glial heterotopia of the face. J Pediatr Surg 2008; 43: e1–e3. 5 Pereyra-Rodriguez JJ, Bernabeu-Wittel J, Fajardo M, et al. Nasal glial heterotopia (nasal glioma). J Pediatr 2010; 156: 688–688.e681. 6 Hoeger PH, Schaefer H, Ussmueller J, Helmke K. Nasal glioma presenting as capillary haemangioma. Eur J Pediatr 2001; 160: 84–87. 7 Jartti PH, Jartti AE, Karttunen AI, et al. MR of a nasal glioma in a young infant. Acta Radiol 2002; 43: 141–143. ª 2015 The International Society of Dermatology
8 Rahbar R, Resto VA, Robson CD, et al. Nasal glioma and encephalocele: diagnosis and management. Laryngoscope 2003; 113: 2069–2077.
Acute generalized exanthematous pustulosis by azithromycin
Acute generalized exanthematous pustulosis (AGEP) is a rare drug-induced hypersensitivity skin reaction, characterized by numerous small, primarily nonfollicular, sterile pustules, arising within large areas of edematous erythema and preferentially found in the intertriginous areas. This pustular eruption is commonly accompanied by high fever and leukocytosis with neutrophils. It can also affect the kidney, liver, lungs, bone marrow, and mucous membranes.1 AGEP was first described by Macmillan in 1973 as a generalized pustular drug rash.2 In 1980, Beylot et al.3 introduced the term AGEP pustulose exanthematique aigu€e generalisee or acute generalized exanthematous pustulosis.3 It has been reported that 80–90% of cases of druginduced AGEP are caused mainly by antibiotics such as aminopenicillins (beta-lactams) or macrolides.1,4 In 1994, a single case caused by azithromycin was published as toxic pustuloderma in Italy.5 We report a new and rare case induced by azithromycin, which supports this presentation. An 18-year-old female patient with inflammatory acne was treated with azithromycin 500 mg/daily. On the second day after using the medication, the patient developed hundreds of nonfollicular pustules and erythematous edema, especially on the face, neck, and upper portion of the trunk, accompanied by low fever (Fig. 1). The patient denied infectious symptoms, history of psoriasis, or use of other medication. The bacteriological examination of the lesions revealed no infection. The laboratory examination showed neutrophilia, and the serological screening was negative for HIV-1/2 and viral hepatitis. Skin cultures were negative. After immediate discontinuation of the antibiotic, the patient was treated with oral prednisone 40 mg for seven days, with rapid improvement. Histopathology showed pustules in the superficial epidermis and neutrophilic spongiosis, associated edematous papillary dermis, and mixed inflammatory infiltrate in the upper dermis (Fig. 2). The mechanism of the disease is not completely understood. An in vitro study showed that the T cells produce high amounts of the neutrophil-attracting chemokine, interleukin-8. This explains the presence of neutrophils in the blood and the accumulation of neutrophils in the skin lesions. The short time between exposure and onset of rash suggests a possible previous awareness and an International Journal of Dermatology 2015, 54, e233–e249
e247
Correspondence
3 Murakami T, Fukai K, Oiso N, et al. New KIT mutations in patients with piebaldism. J Dermatol Sci 2004; 35: 29–33. 4 Matsunaga H, Tanioka M, Utani A, et al. Familial case of piebaldism with regression of white forelock. Clin Exp Dermatol 2008; 33: 511–512. 5 Arase N, Wataya-Kaneda M, Oiso N, et al. Repigmentation of leukoderma in a piebald patient associated with a novel c-KIT gene mutation, G592E, of the tyrosine kinase domain. J Dermatol Sci 2011; 64: 147–149. 6 Makino T, Yanagihara M, Oiso N, et al. Repigmentation of the epidermis around the acrosyringium in piebald skin: an ultrastructural examination. Br J Dermatol 2013; 168: 910–912.
A case of nasal glial heterotopia with complete excision
Nasal glial heterotopia (also known as nasal glioma) is a rare developmental abnormality typically present at birth or in early childhood, but there have been reported cases in a wider age group.1 Nasal glial heterotopia is a rare condition that is thought to originate from either entrapped neuroectodermal tissue during the closure of the covering of the brain or nasal encephalocele, which is covered by dura, pia, and arachnoid and later disconnected from the intracranial cavity. We report a rare case of nasal glial heterotopia. A 1-year-old male infant visited our clinic for evaluation of a congenital tumor on the nose. On examination, the tumor was a 1 9 1 cm, skin-colored, firm, mobile, (a)
and nonpulsatile subcutaneous mass located on the upper part of the right side of the nose (Fig. 1). Doppler ultrasonography revealed a solid mass without vascular proliferation. Facial computed tomography (CT) demonstrated a 1 cm sized polypoid high-attenuation mass in the subcutaneous tissue of the right side of the nose without intracranial extension. The tumor was excised externally. Histologic examination revealed a prominent dermal and subcutaneous neural proliferation composed of astrocytes, neurons, and neuroglial fibers intermixed with a fibrous connective tissue stroma (Fig. 2a,b). Immunohistochemical staining for glial fibrillary acidic protein confirmed the presence of the extensive glial tissue (Fig. 2c). After the excision, there was no recurrence during the 1-year follow-up period. Based on the clinical and histopathological findings, a final diagnosis of nasal glial heterotopia was made. Glial heterotopia is a rare condition composed of mature brain tissue isolated from the cranial cavity or spinal canal. The nose and nasopharynx are the most common locations for glial heterotopia. Nasal glioma may manifest as an extranasal (60%), intranasal (30%), or combined (10%) lesion.2 Vilarinho et al.3 reported an extranasal glial heterotopia located on the nasal bridge. Riffaud et al.4 reported a case of nasal glial heterotopia located on the right paranasal region. Magnetic resonance imaging (MRI) of the head was conducted in both cases, which showed no internal connection, and they were surgically excised and diagnosed with nasal glial heterotopia without recurrence and complication.3,4
(b) Figure 1 (a) Firm, well demarcated, and movable nodule on the right side of the nose. (b) Doppler ultrasonography: lobulated and heterogeneously hyperechoic lesion (1.1 9 1.4 cm) with some hypoechoic portion in the subcutaneous layer of right nose and no definite increased internal vascularity
(a)
(b)
(c) Figure 2 (a) Subcutaneous nodule (hematoxylin and eosin stain 9 40). (b) Astrocytes, neurons, and neuroglial fibers intermixed with a fibrous connective tissue stroma (hematoxylin and eosin stain 9 400). (c) Immunohistochemistry using antibody against glial fibrillary acidic protein highlights strongly stained neuroglial tissue
International Journal of Dermatology 2015, 54, e233–e249
ª 2015 The International Society of Dermatology
Correspondence
The differential diagnosis includes hemangiomas, dermoid cysts, encephaloceles, and malignant tumors.2 Imaging studies must be performed before surgery to exclude other diagnoses and determine appropriate surgical margin.3,5 Biopsy should be avoided because procedures could result in meningitis. The MRI and CT are useful to exclude encephalocele and other congenital vascular tumors. MRI is known to be the most useful imaging approach to identify the intracranial connection of a nasal tumor, but the cost is very high.6,7 In this case, Doppler ultrasonography and facial CT helped to exclude hemangioma and the possibility of connection to intracranial extension. Histologically, nasal glial heterotopia and encephaloceles are characterized by varying proportions of astrocytes and neuroglial fibers associated with fibrous connective tissue.1,8 Complete surgical excision is the treatment of choice in nasal glial heterotopia. A delay in treatment can result in deformation of the septum and nasal bone, or airway obstruction. Recurrence after complete excision is rare.
Hye Rang On, MD Jimyung Seo, MD Kee Yang Chung, MD, PhD Department of Dermatology and Cutaneous Biology Research Institute Yonsei University College of Medicine Seoul Korea E-mail: [email protected]
References 1 Penner CR, Thompson L. Nasal glial heterotopia: a clinicopathologic and immunophenotypic analysis of 10 cases with a review of the literature. Ann Diagn Pathol 2003; 7: 354–359. 2 Jaffe BF. Classification and management of anomalies of the nose. Otolaryngol Clin North Am 1981; 14: 989–1004. 3 Vilarinho C, Ventura F, Vieira AP, et al. Nasal glial heterotopia in a newborn infant. Int J Dermatol 2009; 48: 1225–1227. 4 Riffaud L, Ndikumana R, Azzis O, Cadre B. Glial heterotopia of the face. J Pediatr Surg 2008; 43: e1–e3. 5 Pereyra-Rodriguez JJ, Bernabeu-Wittel J, Fajardo M, et al. Nasal glial heterotopia (nasal glioma). J Pediatr 2010; 156: 688–688.e681. 6 Hoeger PH, Schaefer H, Ussmueller J, Helmke K. Nasal glioma presenting as capillary haemangioma. Eur J Pediatr 2001; 160: 84–87. 7 Jartti PH, Jartti AE, Karttunen AI, et al. MR of a nasal glioma in a young infant. Acta Radiol 2002; 43: 141–143. ª 2015 The International Society of Dermatology
8 Rahbar R, Resto VA, Robson CD, et al. Nasal glioma and encephalocele: diagnosis and management. Laryngoscope 2003; 113: 2069–2077.
Acute generalized exanthematous pustulosis by azithromycin
Acute generalized exanthematous pustulosis (AGEP) is a rare drug-induced hypersensitivity skin reaction, characterized by numerous small, primarily nonfollicular, sterile pustules, arising within large areas of edematous erythema and preferentially found in the intertriginous areas. This pustular eruption is commonly accompanied by high fever and leukocytosis with neutrophils. It can also affect the kidney, liver, lungs, bone marrow, and mucous membranes.1 AGEP was first described by Macmillan in 1973 as a generalized pustular drug rash.2 In 1980, Beylot et al.3 introduced the term AGEP pustulose exanthematique aigu€e generalisee or acute generalized exanthematous pustulosis.3 It has been reported that 80–90% of cases of druginduced AGEP are caused mainly by antibiotics such as aminopenicillins (beta-lactams) or macrolides.1,4 In 1994, a single case caused by azithromycin was published as toxic pustuloderma in Italy.5 We report a new and rare case induced by azithromycin, which supports this presentation. An 18-year-old female patient with inflammatory acne was treated with azithromycin 500 mg/daily. On the second day after using the medication, the patient developed hundreds of nonfollicular pustules and erythematous edema, especially on the face, neck, and upper portion of the trunk, accompanied by low fever (Fig. 1). The patient denied infectious symptoms, history of psoriasis, or use of other medication. The bacteriological examination of the lesions revealed no infection. The laboratory examination showed neutrophilia, and the serological screening was negative for HIV-1/2 and viral hepatitis. Skin cultures were negative. After immediate discontinuation of the antibiotic, the patient was treated with oral prednisone 40 mg for seven days, with rapid improvement. Histopathology showed pustules in the superficial epidermis and neutrophilic spongiosis, associated edematous papillary dermis, and mixed inflammatory infiltrate in the upper dermis (Fig. 2). The mechanism of the disease is not completely understood. An in vitro study showed that the T cells produce high amounts of the neutrophil-attracting chemokine, interleukin-8. This explains the presence of neutrophils in the blood and the accumulation of neutrophils in the skin lesions. The short time between exposure and onset of rash suggests a possible previous awareness and an International Journal of Dermatology 2015, 54, e233–e249
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