148 Pediatric Dermatology Vol. 32 No. 1 January/February 2015
Figure 1. Coalescing pustules over erythematous skin involving the right lower limb and trunk forming lakes of pus (arrow).
Ulcerated Spitz Nevus Masquerading as a Juvenile Xanthogranuloma Abstract: We report a case of ulcerated atypical Spitz nevi that demonstrated a yellow to light orange background under dermoscopy, which can be seen in juvenile xanthogranuloma (JXG) and is referred to as the “setting sun” appearance. This yellow to orange appearance was due to serous crusting and not histiocytic infiltration, which is seen in JXG. This case highlights overlapping dermatoscopic features between the two skin lesions and polymorphous vascular structures, which are unique to atypical Spitz nevi.
Figure 2. Microscopy of eyebrow hair showing bamboo hair (trichorrhexis invaginata) (109).
REFERENCE 1. Chavanas S, Bodemer C, Rochat A et al. Mutations in SPINK5, encoding a serine protease inhibitor, cause Netherton syndrome. Nat Genet 2000;25:141–142. Vibhu Mendiratta, M.D.* Pravesh Yadav, M.D.* Ram Chander, M.D.* Shilpi Aggarwal, M.D.† *Department of Dermatology and Sexually Transmitted Diseases and †Department of Pathology, Lady Hardinge Medical College and Suchita Kriplani Hospital, Shaheed Bhagat Singh Marg, Delhi, India Address correspondence to Pravesh Yadav, RZ-97, Phase III, Prem Nagar, Najafgarh, New Delhi 110043, India, or e-mail: rao. [email protected].
A healthy 17-month-old girl presented to our pediatric dermatology clinic for an enlarging lesion on the forehead. The parents reported a small pimple-like lesion 2 months prior that grew rapidly and did not resolve with mupirocin. They denied preceding trauma. The grandfather had a history of melanoma. Clinical examination revealed a well-circumscribed 0.5-cm 9 0.5-cm dome-shaped red to yellow papule with central superficial crusting on the forehead (Fig. 1). Dermoscopy was remarkable for a homogeneously pigmented yellow to light orange lesion with scattered dotted, comma-like, and serpentine vascular structures (Fig. 2). Because of the yellow to light orange dermoscopic pattern, a diagnosis of juvenile xanthogranuloma (JXG) was favored over that of a Spitz nevus.
Brief Reports 149
child has since undergone wide local excision without complications. DISCUSSION
Figure 1. Well-circumscribed 0.5-cm 9 0.5-cm domeshaped red to yellow papule with crusting on the forehead.
Histologic sections demonstrated a nested proliferation of S100/Melan-A-positive spindled to epithelioid-appearing melanocytes arising from the epidermis and extending into the dermis. Dermal maturation was present in addition to a broad central ulceration with fibrinoid crust. Focal Kamino bodies were present and mitoses were conspicuously present in the junctional and dermal components. The final diagnosis was atypical Spitz nevus. The
A
Dermoscopy enhances the clinical evaluation of skin lesions. Features typically associated with Spitz nevi include brown to black peripheral globules and streaking, referred to as the starburst pattern. Nonpigmented Spitz nevi typically contain monomorphous vascular dotted structures and a pink background. This case suggests that ulcerated Spitz nevi may be yellow to orange, similar to a JXG, which has been referred to as the “setting sun” appearance (Fig. 2) because of xanthomatized histiocytes (1). It is likely that serous crusting, which has been described for other cutaneous ulcerated lesions including squamous cell carcinoma and cutaneous leishmaniasis, is the cause of this finding (within melanocytic lesions) (2). Other findings of JXG include linear or branched vascular structures, whitish streaks, and yellow globules (1). These overlapping features are a clinical and diagnostic pitfall when evaluating these lesions. Features that may aid clinicians in distinguishing these lesions are the polymorphous vascular structures (dotted, comma like, and serpentine), because these are commonly found in atypical melanocytic lesions (3). In conclusion, ulcerated Spitz nevi can have dermoscopic patterns containing yellow and orange that can mimic the setting sun pattern of JXG, but a polymorphous vascular pattern is not found in JXG. Any ulceration of a lesion on clinical examination must be taken into account when interpreting dermoscopic patterns.
B
Figure 2. Dermatoscopic examination of (A) an ulcerated atypical Spitz (homogeneously pigmented yellow to light-colored papule with scattered dotted, serpentine, and comma-like vascular structures) and (B) a juvenile xanthogranuloma (typical setting sun pattern with subtle vascular structures).
150 Pediatric Dermatology Vol. 32 No. 1 January/February 2015
REFERENCES 1. Song M, Kim SH, Jung DS, et al. Structural correlations between dermoscopic and histopathological features of juvenile xanthogranuloma. J Eur Acad Dermatol Venereol 2011;25:259–263. 2. Llambrich A, Zaballos P, Terrasa F, et al. Dermoscopy of cutaneous leishmaniasis. Br J Dermatol 2009;160:756– 761. 3. Argenziano G, Zalaudek I, Corona R, et al. Vascular structures in skin tumors: a dermoscopy study. Arch Dermatol 2004;140:1485–1489.
Figure 1. Friable papule on the cheek of a 5-month-old.
Grant K. Ghahramani, M.D. Brian L. Swick, M.D. Heather Ciliberto, M.D. Department of Dermatology, University of Iowa Hospitals and Clinics, Iowa City, Iowa Address correspondence to Grant K. Ghahramani, M.D., Department of Dermatology, University of Iowa, 200 Hawkins Drive/40024 PFP, Iowa City, IA 52242, or e-mail: [email protected].
Pyogenic Granuloma in a 5-Month-Old Treated with Topical Timolol Abstract: A 5-month-old healthy female presented with a pyogenic granuloma on the cheek. The lesion was treated with topical 0.5% gel-forming solution, resulting in regression of the lesion after 1 month of treatment and no recurrence at 8 months. This case suggests that treatment of pyogenic granulomas with topical timolol may be considered, especially when other treatment modalities are challenging or could result in significant scarring.
CASE REPORT A 5-month-old healthy girl presented for evaluation of a vascular growth on the left cheek. The growth had appeared at 1 month of age, had grown and bled, with the superficial papule detaching after trauma then recurring. On examination there was a 7-mm pink friable papule with hemorrhagic crust (Fig. 1). History and morphology were most consistent with a pyogenic granuloma. The location of the lesion on the face and the broad base made shave removal or surgical excision a less favorable option because of the likelihood of significant scarring. Topical timolol gel was suggested as an alternative treatment to try to encourage involution of the lesion. The lesion involuted over 1 month of treatment with timolol 0.5% gel-forming solution, leaving a 7-mm hyperpigmented
Figure 2. Residual erosion and surrounding hyperpigmentation after 1 month of topical timolol.
patch with a 3- to 4-mm erythematous slightly eroded center (Fig. 2). The patient’s family was encouraged to continue topical treatment with timolol until full re-epithelization of the area occurred. Follow-up by telephone at 8 months of age revealed that the lesion had not recurred. DISCUSSION Pyogenic granulomas are common vascular lesions in children and appear most often on the face and upper extremities (1). They are typically small, friable, red papules or nodules. Although the differential diagnosis of a vascular lesion at this age includes infantile hemangioma or Spitz nevus, we favored the diagnosis of a pyogenic granuloma based on the history of being easily friable and morphology. The exact pathogenesis remains unknown but may be due to an unregulated angiogenic stimulus, and some develop at sites of trauma. If untreated, these lesions most often persist and may enlarge and continue to bleed intermittently (2). They may infrequently resolve on their own, but most mature, increase their fibrous component, and persist indefinitely (3). Current treatment options for pyogenic granulomas include shave removal with electrodesiccation to the base, surgical excision, pulsed dye laser, cryotherapy, and topical silver nitrate; one case series has reported the use of topical imiquimod (4). Surgical excision often leads to localized scarring and the potential for infection, limiting
Figure 1. Coalescing pustules over erythematous skin involving the right lower limb and trunk forming lakes of pus (arrow).
Ulcerated Spitz Nevus Masquerading as a Juvenile Xanthogranuloma Abstract: We report a case of ulcerated atypical Spitz nevi that demonstrated a yellow to light orange background under dermoscopy, which can be seen in juvenile xanthogranuloma (JXG) and is referred to as the “setting sun” appearance. This yellow to orange appearance was due to serous crusting and not histiocytic infiltration, which is seen in JXG. This case highlights overlapping dermatoscopic features between the two skin lesions and polymorphous vascular structures, which are unique to atypical Spitz nevi.
Figure 2. Microscopy of eyebrow hair showing bamboo hair (trichorrhexis invaginata) (109).
REFERENCE 1. Chavanas S, Bodemer C, Rochat A et al. Mutations in SPINK5, encoding a serine protease inhibitor, cause Netherton syndrome. Nat Genet 2000;25:141–142. Vibhu Mendiratta, M.D.* Pravesh Yadav, M.D.* Ram Chander, M.D.* Shilpi Aggarwal, M.D.† *Department of Dermatology and Sexually Transmitted Diseases and †Department of Pathology, Lady Hardinge Medical College and Suchita Kriplani Hospital, Shaheed Bhagat Singh Marg, Delhi, India Address correspondence to Pravesh Yadav, RZ-97, Phase III, Prem Nagar, Najafgarh, New Delhi 110043, India, or e-mail: rao. [email protected].
A healthy 17-month-old girl presented to our pediatric dermatology clinic for an enlarging lesion on the forehead. The parents reported a small pimple-like lesion 2 months prior that grew rapidly and did not resolve with mupirocin. They denied preceding trauma. The grandfather had a history of melanoma. Clinical examination revealed a well-circumscribed 0.5-cm 9 0.5-cm dome-shaped red to yellow papule with central superficial crusting on the forehead (Fig. 1). Dermoscopy was remarkable for a homogeneously pigmented yellow to light orange lesion with scattered dotted, comma-like, and serpentine vascular structures (Fig. 2). Because of the yellow to light orange dermoscopic pattern, a diagnosis of juvenile xanthogranuloma (JXG) was favored over that of a Spitz nevus.
Brief Reports 149
child has since undergone wide local excision without complications. DISCUSSION
Figure 1. Well-circumscribed 0.5-cm 9 0.5-cm domeshaped red to yellow papule with crusting on the forehead.
Histologic sections demonstrated a nested proliferation of S100/Melan-A-positive spindled to epithelioid-appearing melanocytes arising from the epidermis and extending into the dermis. Dermal maturation was present in addition to a broad central ulceration with fibrinoid crust. Focal Kamino bodies were present and mitoses were conspicuously present in the junctional and dermal components. The final diagnosis was atypical Spitz nevus. The
A
Dermoscopy enhances the clinical evaluation of skin lesions. Features typically associated with Spitz nevi include brown to black peripheral globules and streaking, referred to as the starburst pattern. Nonpigmented Spitz nevi typically contain monomorphous vascular dotted structures and a pink background. This case suggests that ulcerated Spitz nevi may be yellow to orange, similar to a JXG, which has been referred to as the “setting sun” appearance (Fig. 2) because of xanthomatized histiocytes (1). It is likely that serous crusting, which has been described for other cutaneous ulcerated lesions including squamous cell carcinoma and cutaneous leishmaniasis, is the cause of this finding (within melanocytic lesions) (2). Other findings of JXG include linear or branched vascular structures, whitish streaks, and yellow globules (1). These overlapping features are a clinical and diagnostic pitfall when evaluating these lesions. Features that may aid clinicians in distinguishing these lesions are the polymorphous vascular structures (dotted, comma like, and serpentine), because these are commonly found in atypical melanocytic lesions (3). In conclusion, ulcerated Spitz nevi can have dermoscopic patterns containing yellow and orange that can mimic the setting sun pattern of JXG, but a polymorphous vascular pattern is not found in JXG. Any ulceration of a lesion on clinical examination must be taken into account when interpreting dermoscopic patterns.
B
Figure 2. Dermatoscopic examination of (A) an ulcerated atypical Spitz (homogeneously pigmented yellow to light-colored papule with scattered dotted, serpentine, and comma-like vascular structures) and (B) a juvenile xanthogranuloma (typical setting sun pattern with subtle vascular structures).
150 Pediatric Dermatology Vol. 32 No. 1 January/February 2015
REFERENCES 1. Song M, Kim SH, Jung DS, et al. Structural correlations between dermoscopic and histopathological features of juvenile xanthogranuloma. J Eur Acad Dermatol Venereol 2011;25:259–263. 2. Llambrich A, Zaballos P, Terrasa F, et al. Dermoscopy of cutaneous leishmaniasis. Br J Dermatol 2009;160:756– 761. 3. Argenziano G, Zalaudek I, Corona R, et al. Vascular structures in skin tumors: a dermoscopy study. Arch Dermatol 2004;140:1485–1489.
Figure 1. Friable papule on the cheek of a 5-month-old.
Grant K. Ghahramani, M.D. Brian L. Swick, M.D. Heather Ciliberto, M.D. Department of Dermatology, University of Iowa Hospitals and Clinics, Iowa City, Iowa Address correspondence to Grant K. Ghahramani, M.D., Department of Dermatology, University of Iowa, 200 Hawkins Drive/40024 PFP, Iowa City, IA 52242, or e-mail: [email protected].
Pyogenic Granuloma in a 5-Month-Old Treated with Topical Timolol Abstract: A 5-month-old healthy female presented with a pyogenic granuloma on the cheek. The lesion was treated with topical 0.5% gel-forming solution, resulting in regression of the lesion after 1 month of treatment and no recurrence at 8 months. This case suggests that treatment of pyogenic granulomas with topical timolol may be considered, especially when other treatment modalities are challenging or could result in significant scarring.
CASE REPORT A 5-month-old healthy girl presented for evaluation of a vascular growth on the left cheek. The growth had appeared at 1 month of age, had grown and bled, with the superficial papule detaching after trauma then recurring. On examination there was a 7-mm pink friable papule with hemorrhagic crust (Fig. 1). History and morphology were most consistent with a pyogenic granuloma. The location of the lesion on the face and the broad base made shave removal or surgical excision a less favorable option because of the likelihood of significant scarring. Topical timolol gel was suggested as an alternative treatment to try to encourage involution of the lesion. The lesion involuted over 1 month of treatment with timolol 0.5% gel-forming solution, leaving a 7-mm hyperpigmented
Figure 2. Residual erosion and surrounding hyperpigmentation after 1 month of topical timolol.
patch with a 3- to 4-mm erythematous slightly eroded center (Fig. 2). The patient’s family was encouraged to continue topical treatment with timolol until full re-epithelization of the area occurred. Follow-up by telephone at 8 months of age revealed that the lesion had not recurred. DISCUSSION Pyogenic granulomas are common vascular lesions in children and appear most often on the face and upper extremities (1). They are typically small, friable, red papules or nodules. Although the differential diagnosis of a vascular lesion at this age includes infantile hemangioma or Spitz nevus, we favored the diagnosis of a pyogenic granuloma based on the history of being easily friable and morphology. The exact pathogenesis remains unknown but may be due to an unregulated angiogenic stimulus, and some develop at sites of trauma. If untreated, these lesions most often persist and may enlarge and continue to bleed intermittently (2). They may infrequently resolve on their own, but most mature, increase their fibrous component, and persist indefinitely (3). Current treatment options for pyogenic granulomas include shave removal with electrodesiccation to the base, surgical excision, pulsed dye laser, cryotherapy, and topical silver nitrate; one case series has reported the use of topical imiquimod (4). Surgical excision often leads to localized scarring and the potential for infection, limiting
