PEDIATRIC DERMATOLOGY PHOTOQUIZ Editor: Antonio Torrelo, M.D.

NODULES

ON THE

CHEEK OF

A

6-YEAR-OLD GIRL

Young H. Lee, M.D.,* Andrea L. Zaenglein, M.D.,*,† Klaus F. Helm, M.D.,*,‡ and Catherine G. Chung, M.D.*,‡ Departments of *Dermatology, †Pediatrics, and ‡Pathology, Milton S. Hershey Medical Center, Pennsylvania State University, Hershey, Pennsylvania

A 6-year-old girl presented with a 6-month history of two nodules on her right cheek. Her medical history was significant for a choledochal cyst that had been treated with surgical excision when she was 2 years old with no significant long-term sequelae. Physical examination revealed two nontender, firm nodules on her right cheek with a violaceous hue but otherwise no overlying surface change (Fig. 1). She also had several pink papules on her cheeks and chin. A punch biopsy was performed on one of the nodules (Fig. 2).

A

Figure 1. Clinical photograph at presentation.

B

What is the diagnosis?

Figure 2. Biopsy specimen (hematoxylin and eosin; (A) 409, (B) 2009.

A TUMOR

ON THE

LEG OF A 2-YEAR-OLD GIRL

Trinidad Hasbun, M.D.,* Wendy Schumacher, D.O.,* Michael Sidiropoulos, M.D.,† and Anthony J. Mancini, M.D.*,† Departments of *Pediatrics and †Dermatology, Feinberg School of Medicine, Northwestern University and Ann & Robert H. Lurie Children’s Hospital of Chicago, Chicago, Illinois

A 2-year-old girl presented for evaluation of a plaque on her right lower leg that had been present for 9 months. It initially appeared as a firm, nontender plaque, with the subsequent development of central ulceration. There was no history of preceding trauma and she had unremarkable past medical and family histories. Physical examination revealed a 1.5-cm 9 1.0-cm pink nodular plaque with central superficial ulceration and crusting on the medial aspect of the right lower leg (Fig. 1). There was no tenderness to palpation. There was no palpable popliteal or inguinal lymphadenopathy. An excisional biopsy was performed. Histopathologic features are illustrated in Figs. 2 and 3.

Figure 1. Figure 3.

What is the diagnosis?

Figure 2.

AN INFANT PRESENTING

WITH

MULTIPLE SCALP ULCERS

Karan Lal, B.S.,* Ncoza Dlova, M.B.Ch.B., F.C.Derm.,† Anisa Mosam, M.B.Ch.B., F.C.Derm., M.Med., Ph.D.,† and Nokubonga Khoza, M.B.Ch.B., F.C.Derm.† *New York Institute of Technology, College of Osteopathic Medicine, Old Westbury, New York, †Nelson R. Mandela School of Medicine, University of KwaZulu-Natal, Durban, South Africa

A 9-month-old HIV-positive African boy presented to the dermatology clinic with a 1-month history of multiple, punched-out, partially necrotic ulcers on the back of his scalp. According to the mother, the lesions were asymptomatic, and he had no recent infections or illnesses. The past medical history was noncontributory for mother and child and there was no history of medications or any known allergies. The mother was also HIV positive, CD4 count unknown, and did not have a history of any mucocutaneous lesions or similar-appearing lesions. The child was born through a normal spontaneous vaginal delivery with no known complications at birth. His current CD4 count was also unknown. Physical examination revealed five ulcerative lesions on the scalp, one measuring 0.5 cm 9 2 cm in the right superior frontoparietal region, and four on the posterior scalp, measuring 0.5 cm 9 1 cm, 1 cm 9 0.7 cm, 0.7 cm 9 1.2 cm, and 1 cm 9 1.2 cm (Figs. 1 and 2). The largest lesion on the right posterior occipital region had central necrosis. All ulcers were well demarcated with surrounding indurated borders and alopecia. No exudates were found within the ulcers and tenderness was not elicited upon palpation. The rest of the skin examination was normal. A 4-mm punch biopsy was performed on the largest ulcer.

Figure 3. Figure 1.

Figure 2.

What is the diagnosis?

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NODULES

ON THE

CHEEK

Diagnosis: Idiopathic Facial Aseptic Granuloma.

Microscopic Findings and Clinical Course The biopsy specimen demonstrated granulomatous inflammation characterized by epithelioid histiocytes in the dermis surrounded by lymphocytes, plasma cells, and multinucleate giant cells. Stains for infectious organisms were negative, including acidfast bacilli, Gomori–Grocott methamine silver, Gram stain, and periodic acid–Schiff. The patient was seen for follow-up 4 months after initial evaluation. The nodules were still present, although they had decreased in size and had a less prominent violaceous hue. Given the clinical presentation with the histologic analysis, a diagnosis of idiopathic facial aseptic granuloma (IFAG) was made.

Discussion IFAG is a chronic, benign inflammatory condition characterized by painless red or violaceous nodules on the face of children, most often located on the cheek (1). There are no identifiable associated factors, such as a positive family history or distinctive accompanying clinical findings (1). Most cases report a solitary lesion (2,3), but several children have presented with multiple nodules (4), with some resolving as others appear (1,5–7). Histologic analysis often shows chronic dermal granulomatous inflammation composed of lymphocytes, histiocytes, neutrophils, at times plasma cells or eosinophils, and numerous foreign body–type giant cells (1,2,4,7). The infiltrate may be centered around a hair follicle or contain a concomitant perifolliculitis (3). Tissue cultures and stains for

A TUMOR

ON THE

6-YEAR-OLD GIRL

OF A

microorganisms are negative (1,4,5,7). Ultrasound examination typically shows a well-demarcated hypoechoic mass without calcification (1,5). The etiology of IFAG is unclear. In some cases, patients report a history of trauma or insect bites (1,5,7). Some authors have hypothesized that the nodules represent a granulomatous response to a developmental abnormality (1,7). Others have proposed that IFAG is on the spectrum of childhood rosacea, given its association with conjunctivitis and chalazions, facial telangiectasias and flushing, papulopustules, and occasional appearance on the eyelid (3,5,6). In one study, children with IFAG were found to have at least two criteria for childhood rosacea over a mean follow-up of 4 years (6). In our case, the patient also presented with pink papules on her cheeks and chin, findings consistent with periorificial dermatitis or rosacea-like papules, which are findings often associated with IFAG (6,8). The differential diagnosis of IFAG includes cysts, such as an epidermoid cyst or dermoid cyst; growths, such as a pilomatricoma, xanthogranuloma, pyogenic granuloma, or Spitz nevus; infections, such as a bacterial abscess, mycobacterial infection, or fungal infection; and inflammatory conditions, such as infantile nodulocystic acne (7). Histologically the findings are similar to those of granulomatous rosacea, including perifolliculitis with granulomatous inflammation. The histopathologic differential diagnosis includes other causes of granulomatous inflammation, such as a foreign body–type reaction, and infection, such as deep fungal or mycobacterial infection. The nodules resolve spontaneously, usually without scarring, with an average course of approximately 11 months, although prolonged cases of several years can occur (1). Treatments are usually unsuccessful, although topical and oral antimicro-

LEG OF

A

Granular cell tumor (GCT) is a rare neural neoplasm, most likely derived from Schwann cells (1). These usually benign tumors can affect patients of any age, race, or sex, although they are uncommon in children. Of 34 patients with GCT that Torrijos-Aguilar et al analyzed, 6 (17.6%) were younger than 18 years of age (2). The diagnosis of GCT is based on histopathologic findings, and the clinical differential diagnosis may include dermatofibroma, neurofibroma, angiolipoma, keloid, and squamous cell carcinoma (2,3). Granular cell tumor usually presents as an asymptomatic, slow-growing, flesh-colored to red solitary nodule, most commonly involving the head, neck, tongue, or vulva (2). In children, the most common locations are the extremities and the oral mucosa (2–6). Multiple lesions may occur in up to 25% of patients (3). In this context, association with other findings has been reported, including axillary freckling, cafe-au-lait spots, and diffuse lentiginosis, although a minority of these patients fit the criteria for neurofibromatosis (3). Patients with Noonan

AN INFANT PRESENTING

WITH

Histologic examination revealed a large ulcerated tumor composed of cells arranged in sheets and nests with round to oval nuclei and abundant granular eosinophilic cytoplasm. Adjacent to the ulcer and overlying the tumor, the epidermis showed prominent pseudoepitheliomatous hyperplasia. The tumor extended to the deep dermis, forming an expansile nodule abutting the subcutaneous tissue. The cells of the deep nodule had a spindled morphology with a high nuclear:cytoplasmic ratio and high mitotic activity (1 mitosis/10 hpf). High-grade nuclear atypia and foci of necrosis were absent. The cells were strongly immunoreactive for S-100 protein and CD68.

Discussion

Discussion The biopsy specimen (hematoxylin and eosin, original magnification 1009) revealed a dense lymphocytic infiltrate in the dermis with purple-staining intranuclear inclusion bodies with surrounding halos suggestive of cytomegalovirus (CMV) infection (Fig. 3). Cytomegalovirus is a double-stranded DNA virus in the Herpesviridae family. It is prevalent in up to 85% of adults in the United States, with a greater prevalence in Asian and African countries (1–3). CMV causes infection through mucosal penetration with viral secretion into bodily fluids. Congenital infection is common in children and coinfection in HIV-infected children is even more common and is associated with greater mortality and neurologic abnormalities (4,5). Cutaneous CMV is rare, but CMV ulcers are the most common cutaneous presentation of CMV and have been reported mostly in the perineal and genital regions of individuals with AIDS (6). No other cases have been reported on the scalp. We postulate that pressure from prolonged periods of infants lying supine may be a predisposing factor for the development of pressure ulcers that become infected during the neovascularization phase of wound healing. The role of CMV in ulcer formation is unknown, but it has been found with coinfection in Kaposi’s sarcoma, bacillary angiomatosis, herpes simplex virus (HSV) types 1 and 2, varicella zoster virus, acid-fast bacilli, and Staphylococcus aureus (6– 8). Because of CMV’s preference for the dermis and the known associations between coinfected lesions of HSV and CMV, it is postulated that HSV is the cause of ulcerogenesis, with CMV being a bystander

References 1. Boralevi F, Leaute-Labreze C, Lepreux S et al. Idiopathic facial aseptic granuloma: a multicentre prospective study of 30 cases. Br J Dermatol 2007;156:705–708. 2. Hiraldo-Gamero A, Vera-Casano A, Sanz-Trelles A. Idiopathic facial aseptic granuloma. Actas Dermosifiliogr 2013;104:635–636. 3. Neri I, Raone B, Dondi A et al. Should idiopathic facial aseptic granuloma be considered granulomatous rosacea? Report of three pediatric cases. Pediatr Dermatol 2013;30:109–111. 4. Martinez-Diaz GJ, Kim J, Bruckner AL. A toddler with facial nodules: a case of idiopathic facial aseptic granuloma. Dermatol Online J 2010;16:9. 5. Baroni A, Russo T, Faccenda F et al. Idiopathic facial aseptic granuloma in a child: a possible expression of childhood rosacea. Pediatr Dermatol 2013;30:394–395. 6. Prey S, Ezzedine K, Mazereeuw-Hautier J et al. IFAG and childhood rosacea: a possible link? Pediatr Dermatol 2013;30:429–432. 7. Roul S, Leaute-Labreze C, Boralevi F et al. Idiopathic aseptic facial granuloma (pyodermite froide du visage): a pediatric entity? Arch Dermatol 2001;137:1253–1255. 8. Chamaillard M, Mortemousque B, Boralevi F et al. Cutaneous and ocular signs of childhood rosacea. Arch Dermatol 2008;144:167–171. 9. Rallis E, Korfitis C. Isotretinoin for the treatment of granulomatous rosacea: case report and review of the literature. J Cutan Med Surg 2012;16:438–441. Address correspondence to Young H. Lee, M.D., Department of Dermatology, Milton S. Hershey Medical Center, Pennsylvania State University, Hershey, PA 17033, USA, or e-mail: [email protected].

2-YEAR-OLD GIRL

syndrome and neurofibromatosis type 1 with concomitant multiple GCT have been described (7). Histopathologically, GCTs are characterized by broad cellular fascicles arranged in nests or sheets infiltrating the dermis. The tumor cells are large and polygonal, with a granular cytoplasm and small, round to oval nuclei. GCTs express markers associated with neural (S-100 protein, peripheral nerve myelin proteins, neuron-specific enolase) and histiocytic (CD68) differentiation. The presence of cytologic atypia, spindle cell morphology, high mitotic activity, abnormal mitotic forms, and areas of necrosis are suggestive of the potential for more aggressive behavior (8,9). Malignant GCTs make up 1% to 2% of all cases and are defined on the basis of clinical and pathologic data. Fanburg-Smith et al (10) reviewed 73 cases of benign, atypical, and malignant GCTs. Malignant tumors were diagnosed if they met three or more of six histologic criteria, and atypical tumors were diagnosed if they met up to two of the criteria (necrosis, spindling, vesicular nuclei with large nucleoli, high mitotic rate, high nuclear:cytoplasmic ratio, and pleomorphism) (10). These criteria have since been updated, and some authors believe the only unequivocal marker of malignancy is metastasis. To our knowledge, only two cases of atypical GCTs have been published in the pediatric literature (2,5), with no malignant GCTs reported in this population. The treatment of choice for GCTs is complete surgical excision. All of the 34 patients that TorrijosAguilar et al (2) reported underwent complete excision, and no recurrences were reported (with a follow-up range of 4–27 yrs). Mohs micrographic surgery has also been reported (4,8). In conclusion, GCTs are neural tumors that occur infrequently in children. The prognosis is good in the absence of aggressive growth patterns and atypical histologic features. The treatment of choice

Diagnosis: Atypical Granular Cell Tumor

bials, including topical metronidazole and oral clarithromycin and erythromycin, and topical steroids have been tried with varying results (1,3). Some persistent cases have been treated with surgical excision (1,5,7). Isotretinoin is used for the treatment of granulomatous rosacea in adults (9) and therefore may be a therapeutic consideration for IFAG, but the safety and efficacy of treatment of IFAG with isotretinoin has not been established.

is complete surgical excision with appropriate clinical follow-up. If multiple lesions are present, syndromic association (type 1 neurofibromatosis or Noonan syndrome) should be considered.

References 1. Seo IS, Azarelli B, Warner TF et al. Multiple visceral and cutaneous granular cell tumours: ultrastructural and immunocytochemical evidence of Schwann cell origin. Cancer 1984;53:2104–2110. 2. Torrijos-Aguilar A et al. [Cutaneous granular cell tumor: a clinical and pathologic analysis of 34 cases]. Actas Dermosifiliogr 2009;100:126–132. 3. Tomson N, Abdullah A, Tan CY. Multiple granular cell tumors in a child with growth retardation. Report of a case and review of the literature. Int J Dermatol 2006;45:1358–1361. 4. Abraham T, Jackson B, Davis L et al. Mohs surgical treatment of a granular cell tumor on the toe of a child. Pediatr Dermatol 2007;24:235–237. 5. Moos D, Droitcourt C, Rancherevince D et al. Atypical granular cell tumor occurring in an individual with Noonan syndrome treated with growth hormone. Pediatr Dermatol 2012;29:665–666. pez V, Santoja N, Jorda E. Granular cell tumor on the 6. Lo sole of a child: a case report. Pediatr Dermatol 2011;28:473–474. 7. Ramaswamy PV, Storm CA, Filiano JJ et al. Multiple granular cell tumors in a child with Noonan syndrome. Pediatr Dermatol 2012;27:209–211. 8. Smith SB, Farley MF, Albertini HG et al. Mohs micrographic surgery for granular cell tumor using S-100 immunostain. Dermatol Surg 2002;28:1076–1078. 9. Miracco C, Andreassi A, Laurini L et al. Granular cell tumour with histological signs of malignancy: report of a case and comparison with 10 benign and 4 atypical cases. Br J Dermatol 1999;141:573–575. 10. Fanburg-Smith JC et al. Malignant granular cell tumor of soft tissue: diagnostic criteria and clinicopathologic correlation. Am J Surg Pathol 1998;22:779–794. Address correspondence to Anthony J. Mancini, M.D., Division of Dermatology, Ann & Robert H. Lurie Children’s Hospital of Chicago, Mailbox #107, 225 East Chicago Avenue, Chicago, IL 60611, or e-mail: [email protected].

MULTIPLE SCALP ULCERS

maintaining the dermal component of the ulcer (6). This may be the case only in individuals with AIDS, because a study by Choi et al (9) examined nine patients with immunosuppressed states other than AIDS and only one had lesions coinfected with CMV and HSV. Cutaneous CMV infection has a poor prognostic outcome because it often represents a manifestation of systemic disease and may indicate an underlying immunosuppressive condition, such as HIV, so diagnosis is imperative and a high index of suspicion is required (6). It is also beneficial to look for CMV antigens in blood to establish whether active viral dissemination is present. Viral cultures may reveal false positives because of the ability of copresent viruses, such as HSV and varicella zoster virus, to destroy the superficial layer of fibroblasts on the culture, preventing growth of CMV (6). Hematoxylin and eosin staining of specimens along with microscopic examination of lesions is the most sensitive way to diagnose cutaneous CMV (6). In the patient’s biopsy specimen, hematoxylin and eosin staining revealed cytomegalic inclusion bodies in cells within the lesion, with no evidence of other viral, bacterial, or fungal organisms. Cutaneous CMV can be treated using ganciclovir or valganciclovir and foscarnet for resistant strains (10). Our patient was started on ganciclovir, but was lost to follow-up.

References 1. Cytomegalovirus (CMV) and congenital CMV infection. Atlanta, GA: Centers for Disease Control and Prevention [online]. http://www.cdc.gov/cmv/overview.html. Accessed on June 23, 2011.

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2. Burchett SK, Dalgic N. Viral infections. In: Cloherty JP, Eichenwald EC, Stark AR, eds. Manual of neonatal care, 6th ed. Philadelphia: Lippincott Williams & Wilkins, 2008:244–274. 3. Staras SA, Dollard SC, Radford KW et al. Seroprevalence of cytomegalovirus infection in the United States, 1988– 1994. Clin Infect Dis 2006;43:1143–1151. 4. Kovacs A, Schluchter M, Easley K et al. Cytomegalovirus infection and HIV-1 disease progression in infants born to HIV-1-infected women. Pediatric Pulmonary and Cardiovascular Complications of Vertically Transmitted HIV Infection Study Group. N Engl J Med 1999;341:77–84. 5. Doyle M, Atkins JT, Rivera-Matos IR. Congenital cytomegalovirus infection in infants infected with human immunodeficiency virus type 1. Pediatr Infect Dis J 1996;15:1102– 1106. ndez-Buezo G, Fraga J et al. Mucocuta6. Dauden E, Ferna neous presence of cytomegalovirus associated with human immunodeficiency virus infection: discussion regarding its pathogenetic role. Arch Dermatol 2001;137:443–448. 7. Kempf W, Adams V, Pfaltz M et al. Human herpesvirus type 6 and cytomegalovirus in AIDS-associated Kaposi’s sarcoma: no evidence for an etiological association. Hum Pathol 1995;26:914–919. 8. Boudreau S, Hines HC, Hood AF. Dermal abscesses with Staphylococcus aureus, cytomegalovirus and acid-fast bacilli in a patient with acquired immunodeficiency syndrome (AIDS). J Cutan Pathol 1988;15:53–57. 9. Choi Y, Kim J, Jang K et al. Characteristics of cutaneous cytomegalovirus infection in non-acquired immune deficiency syndrome, immunocompromised patients. Br J Dermatol 2006;155:977–982. 10. Belazarian T, Lorenzo ME, Pearson A et al. Chapter 192. Exanthematous viral diseases. In Goldsmith LA, Katz SI, Gilchrest BA et al, eds. Fitzpatrick’s dermatology in general medicine, 8th ed. New York: McGraw-Hill, 2012 [online]. http://accessmedicine.mhmedical.com/content.aspx?bookid=392§ionid=41138921. Accessed on November 7, 2014. Address correspondence to Karan Lal, B.S., 81-07 249 Street, Bellerose, NY 11426, or e-mail: [email protected].

A tumor on the leg of a 2-year-old girl.

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