398 Pediatric Dermatology Vol. 31 No. 3 May/June 2014
sign. Sinus pericranii should be considered when evaluating midline scalp nodules with a hair collar sign. REFERENCES
Figure 2. Magnetic resonance imaging with contrast (sagittal view) shows a venous anomaly on the scalp with communication to the underlying superior sagittal sinus via the transosseous emissary vein (arrow).
but has also been observed in parietal, occipital, and temporal locations (2). Its appearance can mimic an infantile hemangioma, arteriovenous fistula, dermoid cyst, cephalocele, aplasia cutis congenita, leptomeningeal cyst, eosinophilic granuloma, meningioma, or heterotopic brain tissue (1). Although the lesion is typically asymptomatic, there have been reports of headache, vertigo, and localized pain (1,2). Rare, but serious, reported complications include bradycardia, bradypnea, hearing loss, hemorrhage, infection, traumatic air embolism, and ataxia. The skin manifestations of sinus pericranii are variable, so a clinical diagnosis is difficult. MRI with contrast is the study of choice to diagnose sinus pericranii and detect coexisting vascular anomalies (3). This condition is treated with surgical or endovascular procedures for symptomatic relief and cosmetic reasons and to prevent serious complications. In our case, a hair collar sign marked the location of the sinus pericranii. The hair collar sign is a ring of terminal hairs surrounding a congenital scalp nodule, and its presence is suggestive of cranial dysraphism. The hair collar is typically seen surrounding midline developmental defects such as encephaloceles, meningoceles, and heterotopic brain tissue, although it has also been reported in five cases surrounding cerebrovascular abnormalities, including arteriovenous fistulas, arterial ectasias, and a prominent venous structure (4,5). A PubMed literature search performed using the search term “sinus pericranii” yielded 121 cases in the English-language literature that described or had a photograph of the lesion. We found no other reported cases of sinus pericranii with a hair collar
1. Sheu M, Fauteux G, Chang H et al. Sinus pericranii: dermatologic considerations and literature review. J Am Acad Dermatol 2002;46:934–941. 2. Akram H, Prezerakos G, Haliasos N et al. Sinus pericranii: an overview and literature review of a rare cranial venous anomaly (a review of the existing literature with case examples). Neurosurg Rev 2012;35:15–26; discussion 26. 3. Bigot JL, Iacona C, Lepreux A et al. Sinus pericranii: advantages of MR imaging. Pediatr Radiol 2000;30:710– 712. 4. Stevens CA, Galen W. The hair collar sign. Am J Med Genet 2008;146A:484–487. 5. Herron MD, Coffin CM, Vanderhooft SL. Vascular stains and hair collar sign associated with congenital anomalies of the scalp. Pediatr Dermatol 2005;22:200– 205. Nikoo Cheraghi, M.D.* Sophia Delano, M.D.† Courtney Csikesz, M.D.†,‡ Sathish Dundamadappa, M.D.§ Karen Wiss, M.D.†,‡ *Department of Medicine, †Division of Dermatology, ‡Department of Pediatrics, and §Department of Radiology, University of Massachusetts Medical School, Worcester, Massachusetts Address correspondence to Karen Wiss, M.D., Pediatric Dermatology, Division of Dermatology, Departments of Medicine and Pediatrics, University of Massachusetts Medical School, 281 Lincoln Street, Worcester, MA 01605, or e-mail: [email protected].
Rapidly Involuting Congenital Hemangioma with Pustules: Two Cases Abstract: Rapidly involuting congenital hemangiomas (RICHs) are rare tumors that usually present as well-defined bluish or violaceous plaques or tumors with scattered telangiectasias and central or peripheral pallor. We report two previously unreported cases of RICH with associated pustules.
Rapidly involuting congenital hemangioma (RICH) is a subtype of congenital hemangioma (CH) that have a tendency to involute by 6 to 14 months of age (1). They usually present as well-defined bluish or violaceous plaques or tumors with scattered telangiectasias and central or peripheral pallor. We report
Brief Reports 399
two infants with RICH who presented with surface pustules, a previously unreported association.
CASE REPORT Patient 1 A 1-day-old baby was transferred to the dermatology service with a large, congenital vascular tumor of the face. The tumor involved the right cheek, right side of the nose, and lower eyelid and medial canthus. It was a large, violaceous tumor, with a woody texture and a subtle peripheral rim of pallor. Scattered pustules, at varying stages of resolution, were noted on the surface of the lesion (Fig. 1). No pustules were identified elsewhere on the patient. A skin biopsy specimen that stained negative for glut1 from a nonpustular area confirmed a diagnosis of CH. Routine blood tests were normal and the baby was otherwise well. By 8 weeks of age the lesion had dramatically reduced in size and pustular lesions were no longer evident. On last review, at 7 months of age, the lesion was 50% its original size. Patient 2 A 4-week-old baby was referred to the dermatology service with a congenital vascular lesion of the right lower leg. The lesion was a violaceous mass with a rim of pallor and pustules predominately around its periphery (Fig. 2). No pustules were evident elsewhere. A cutaneous swab from a pustule grew normal flora and fungal culture was negative. Within 4 weeks the lesion had regressed by 75% and the pustules had resolved. Review at 18 months of age revealed resolution of the hemangioma with residual subtle atrophic scarring. The parents reported occasional pustules at the site during involution.
Figure 1. Rapidly involuting congenital hemangioma involving the right cheek, the right side of the nose, the lower eyelid, and the medial canthus with pustules scattered across the surface.
DISCUSSION CHs are rare vascular tumors that proliferate in utero and present fully formed or with signs of involution at birth, without further enlargement postnatally (2). These tumors are rare, accounting for fewer than 3% of all hemangiomas (3). RICH is a subtype of CH that has a tendency to involute by 6 to 14 months of age. RICHs usually present as well-defined bluish or violaceous plaques or tumors with scattered telangiectasias and central or peripheral pallor. They may have a central ulcer, scar, or depression that quickly regresses, often leaving striking dermal and subcutaneous atrophy (2). We report two cases of RICH, presenting with pustules that resolved completely within a few weeks in one case, whereas in the second there were occasional recurrences at the site of the resolved hemangioma for several months. Neither infant had pustules elsewhere, and a cutaneous swab taken from a pustule in patient 2 grew normal skin flora. A smear was not performed. In retrospect, this would have been helpful to confirm the pustular nature of the lesion. We considered milia in the differential diagnosis, because these are common in the neonatal period, but the yellow color and focal brown crusting as the lesions resolved indicated a pustular lesion. Pustules in CH would appear to be rare. We see approximately eight cases of RICH per year in our department and have seen this sign on only two occasions in 20 years. In infantile hemangiomas, myeloid cells are more associated with the proliferative than the involution phase (4). Necrotic cell death
Figure 2. Rapidly involuting congenital hemangioma of the lower right leg with pustules predominantly around the periphery of the lesion.
400 Pediatric Dermatology Vol. 31 No. 3 May/June 2014
can generate sterile inflammation, characterized by the accumulation of innate immune effector cells, namely neutrophils (5). Neutrophil collections may be associated with tissue necrosis, occurring with rapid involution. Other causes of pustular eruptions in infancy include erythema toxicum neonatorum, a self-limiting eruption of papules, pustules, or macules rarely seen beyond the second week of life; eosinophilic pustular folliculitis, an idiopathic dermatosis consisting of follicular pustules; acropustulosis of infancy, characterized by recurrent pruritic pustules on the palms and soles; and transient neonatal pustular melanosis, a self-limiting disorder comprising superficial vesiculopustules that rupture and evolve into hyperpigmented macules. Pustules have not been reported in lesions that mimic RICH, such as kaposiform hemangioendothelioma, fibrosarcoma, hemangiopericytoma, nasal glioma, rhabdomyosarcoma, and myofibromatosis. This distinctive appearance, when present, may thus be a useful clinical sign in differentiating RICH from other vascular tumors of infancy.
Acquired Epidermodysplasia Verruciformis in a Child with Atopic Dermatitis Abstract: A 4-year-old girl with an established diagnosis of atopic dermatitis, previously severe and treated with cyclosporine, developed widespread papules that demonstrated changes consistent with epidermodysplasia verruciformis on biopsy. Human papilloma virus (HPV) typing was performed and was consistent with epidermodysplasia verruciformistype HPV (type 5). These lesions rapidly resolved with a 2-week course of imiquimod. Rapid resolution and no family history of epidermodysplasia verruciformis make this most consistent with acquired epidermodysplasia verruciformis. This case is the first reported case of acquired epidermodysplasia verruciformis in a child without the human immunodeficiency virus and may be linked to cyclosporine use, which also has never been previously reported.
CASE REPORT REFERENCES 1. Browning JC, Metry DW, Berenguer B et al. Rapidly involuting congenital hemangioma: case report and review of the literature. Dermatol Online J 2008;14:11. 2. Berenguer B, Mulliken JB, Enjolras O et al. Rapidly involuting congenital hemangioma: clinical and histopathologic features. Pediatr Dev Pathol 2003;6: 495–510. 3. Bruckner AL, Frieden IJ. Infantile haemangiomas and other vascular tumors. In: Irvine AD, Hoeger PH, Yan AC, eds. Harper’s textbook of pediatric dermatology, 3rd ed. Hoboken, NJ: Wiley-Blackwell, 2011:113–122. 4. Ritter M, Reinisch J, Friedlander Fallon S et al. Myeloid cells in infantile hemangioma. Am J Pathol 2006;168: 621–628. 5. Nathan C, Aihao D. Nonresolving inflammation. Cell 2010;140:871–882.
A 4-year-old Kenyan girl with severe atopic dermatitis, previously treated with cyclosporine, developed flat-topped papules on her neck 2 months after discontinuing therapy. The patient’s mother applied pimecrolimus 1% cream twice daily for 3 to 4 weeks. Three months later these papules had spread to her neck, chest, and upper extremities (Fig. 1). Biopsy demonstrated mild acanthosis and hypergranulosis. Keratinocytes with vacuolated blue cytoplasm were present throughout the epidermis,
Rosalind Hughes, M.B., B.C.H., B.A.O.* Maeve McAleer, M.B., B.C.H., B.A.O.* Rosemarie Watson, M.D., F.R.C.P.I.* Sinead Collins, M.B., B.C.H., B.A.O.† Alan Irvine, M.D., F.R.C.P.I.* Martin White, M.B., B.C.H., B.A.O.‡ *Department of Dermatology, Our Lady’s Hospital for Sick Children, Crumlin, Dublin, Ireland, †Department of Dermatology, Our Lady of Lourdes Hospital, Drogheda, Ireland, ‡Department of Neonatology, Our Lady’s Hospital for Sick Children, Crumlin, Dublin, Ireland Address correspondence to Rosalind Hughes, M.B., B.C.H., B.A.O., Department of Dermatology, Our Lady’s Hospital for Sick Children, Crumlin, Dublin 12, Ireland, or e-mail: [email protected].
Figure 1. Epidermodysplasia verruciformis. Hundreds of hypopigmented flat-topped papules distributed across the neck, upper trunk, and proximal arms.
sign. Sinus pericranii should be considered when evaluating midline scalp nodules with a hair collar sign. REFERENCES
Figure 2. Magnetic resonance imaging with contrast (sagittal view) shows a venous anomaly on the scalp with communication to the underlying superior sagittal sinus via the transosseous emissary vein (arrow).
but has also been observed in parietal, occipital, and temporal locations (2). Its appearance can mimic an infantile hemangioma, arteriovenous fistula, dermoid cyst, cephalocele, aplasia cutis congenita, leptomeningeal cyst, eosinophilic granuloma, meningioma, or heterotopic brain tissue (1). Although the lesion is typically asymptomatic, there have been reports of headache, vertigo, and localized pain (1,2). Rare, but serious, reported complications include bradycardia, bradypnea, hearing loss, hemorrhage, infection, traumatic air embolism, and ataxia. The skin manifestations of sinus pericranii are variable, so a clinical diagnosis is difficult. MRI with contrast is the study of choice to diagnose sinus pericranii and detect coexisting vascular anomalies (3). This condition is treated with surgical or endovascular procedures for symptomatic relief and cosmetic reasons and to prevent serious complications. In our case, a hair collar sign marked the location of the sinus pericranii. The hair collar sign is a ring of terminal hairs surrounding a congenital scalp nodule, and its presence is suggestive of cranial dysraphism. The hair collar is typically seen surrounding midline developmental defects such as encephaloceles, meningoceles, and heterotopic brain tissue, although it has also been reported in five cases surrounding cerebrovascular abnormalities, including arteriovenous fistulas, arterial ectasias, and a prominent venous structure (4,5). A PubMed literature search performed using the search term “sinus pericranii” yielded 121 cases in the English-language literature that described or had a photograph of the lesion. We found no other reported cases of sinus pericranii with a hair collar
1. Sheu M, Fauteux G, Chang H et al. Sinus pericranii: dermatologic considerations and literature review. J Am Acad Dermatol 2002;46:934–941. 2. Akram H, Prezerakos G, Haliasos N et al. Sinus pericranii: an overview and literature review of a rare cranial venous anomaly (a review of the existing literature with case examples). Neurosurg Rev 2012;35:15–26; discussion 26. 3. Bigot JL, Iacona C, Lepreux A et al. Sinus pericranii: advantages of MR imaging. Pediatr Radiol 2000;30:710– 712. 4. Stevens CA, Galen W. The hair collar sign. Am J Med Genet 2008;146A:484–487. 5. Herron MD, Coffin CM, Vanderhooft SL. Vascular stains and hair collar sign associated with congenital anomalies of the scalp. Pediatr Dermatol 2005;22:200– 205. Nikoo Cheraghi, M.D.* Sophia Delano, M.D.† Courtney Csikesz, M.D.†,‡ Sathish Dundamadappa, M.D.§ Karen Wiss, M.D.†,‡ *Department of Medicine, †Division of Dermatology, ‡Department of Pediatrics, and §Department of Radiology, University of Massachusetts Medical School, Worcester, Massachusetts Address correspondence to Karen Wiss, M.D., Pediatric Dermatology, Division of Dermatology, Departments of Medicine and Pediatrics, University of Massachusetts Medical School, 281 Lincoln Street, Worcester, MA 01605, or e-mail: [email protected].
Rapidly Involuting Congenital Hemangioma with Pustules: Two Cases Abstract: Rapidly involuting congenital hemangiomas (RICHs) are rare tumors that usually present as well-defined bluish or violaceous plaques or tumors with scattered telangiectasias and central or peripheral pallor. We report two previously unreported cases of RICH with associated pustules.
Rapidly involuting congenital hemangioma (RICH) is a subtype of congenital hemangioma (CH) that have a tendency to involute by 6 to 14 months of age (1). They usually present as well-defined bluish or violaceous plaques or tumors with scattered telangiectasias and central or peripheral pallor. We report
Brief Reports 399
two infants with RICH who presented with surface pustules, a previously unreported association.
CASE REPORT Patient 1 A 1-day-old baby was transferred to the dermatology service with a large, congenital vascular tumor of the face. The tumor involved the right cheek, right side of the nose, and lower eyelid and medial canthus. It was a large, violaceous tumor, with a woody texture and a subtle peripheral rim of pallor. Scattered pustules, at varying stages of resolution, were noted on the surface of the lesion (Fig. 1). No pustules were identified elsewhere on the patient. A skin biopsy specimen that stained negative for glut1 from a nonpustular area confirmed a diagnosis of CH. Routine blood tests were normal and the baby was otherwise well. By 8 weeks of age the lesion had dramatically reduced in size and pustular lesions were no longer evident. On last review, at 7 months of age, the lesion was 50% its original size. Patient 2 A 4-week-old baby was referred to the dermatology service with a congenital vascular lesion of the right lower leg. The lesion was a violaceous mass with a rim of pallor and pustules predominately around its periphery (Fig. 2). No pustules were evident elsewhere. A cutaneous swab from a pustule grew normal flora and fungal culture was negative. Within 4 weeks the lesion had regressed by 75% and the pustules had resolved. Review at 18 months of age revealed resolution of the hemangioma with residual subtle atrophic scarring. The parents reported occasional pustules at the site during involution.
Figure 1. Rapidly involuting congenital hemangioma involving the right cheek, the right side of the nose, the lower eyelid, and the medial canthus with pustules scattered across the surface.
DISCUSSION CHs are rare vascular tumors that proliferate in utero and present fully formed or with signs of involution at birth, without further enlargement postnatally (2). These tumors are rare, accounting for fewer than 3% of all hemangiomas (3). RICH is a subtype of CH that has a tendency to involute by 6 to 14 months of age. RICHs usually present as well-defined bluish or violaceous plaques or tumors with scattered telangiectasias and central or peripheral pallor. They may have a central ulcer, scar, or depression that quickly regresses, often leaving striking dermal and subcutaneous atrophy (2). We report two cases of RICH, presenting with pustules that resolved completely within a few weeks in one case, whereas in the second there were occasional recurrences at the site of the resolved hemangioma for several months. Neither infant had pustules elsewhere, and a cutaneous swab taken from a pustule in patient 2 grew normal skin flora. A smear was not performed. In retrospect, this would have been helpful to confirm the pustular nature of the lesion. We considered milia in the differential diagnosis, because these are common in the neonatal period, but the yellow color and focal brown crusting as the lesions resolved indicated a pustular lesion. Pustules in CH would appear to be rare. We see approximately eight cases of RICH per year in our department and have seen this sign on only two occasions in 20 years. In infantile hemangiomas, myeloid cells are more associated with the proliferative than the involution phase (4). Necrotic cell death
Figure 2. Rapidly involuting congenital hemangioma of the lower right leg with pustules predominantly around the periphery of the lesion.
400 Pediatric Dermatology Vol. 31 No. 3 May/June 2014
can generate sterile inflammation, characterized by the accumulation of innate immune effector cells, namely neutrophils (5). Neutrophil collections may be associated with tissue necrosis, occurring with rapid involution. Other causes of pustular eruptions in infancy include erythema toxicum neonatorum, a self-limiting eruption of papules, pustules, or macules rarely seen beyond the second week of life; eosinophilic pustular folliculitis, an idiopathic dermatosis consisting of follicular pustules; acropustulosis of infancy, characterized by recurrent pruritic pustules on the palms and soles; and transient neonatal pustular melanosis, a self-limiting disorder comprising superficial vesiculopustules that rupture and evolve into hyperpigmented macules. Pustules have not been reported in lesions that mimic RICH, such as kaposiform hemangioendothelioma, fibrosarcoma, hemangiopericytoma, nasal glioma, rhabdomyosarcoma, and myofibromatosis. This distinctive appearance, when present, may thus be a useful clinical sign in differentiating RICH from other vascular tumors of infancy.
Acquired Epidermodysplasia Verruciformis in a Child with Atopic Dermatitis Abstract: A 4-year-old girl with an established diagnosis of atopic dermatitis, previously severe and treated with cyclosporine, developed widespread papules that demonstrated changes consistent with epidermodysplasia verruciformis on biopsy. Human papilloma virus (HPV) typing was performed and was consistent with epidermodysplasia verruciformistype HPV (type 5). These lesions rapidly resolved with a 2-week course of imiquimod. Rapid resolution and no family history of epidermodysplasia verruciformis make this most consistent with acquired epidermodysplasia verruciformis. This case is the first reported case of acquired epidermodysplasia verruciformis in a child without the human immunodeficiency virus and may be linked to cyclosporine use, which also has never been previously reported.
CASE REPORT REFERENCES 1. Browning JC, Metry DW, Berenguer B et al. Rapidly involuting congenital hemangioma: case report and review of the literature. Dermatol Online J 2008;14:11. 2. Berenguer B, Mulliken JB, Enjolras O et al. Rapidly involuting congenital hemangioma: clinical and histopathologic features. Pediatr Dev Pathol 2003;6: 495–510. 3. Bruckner AL, Frieden IJ. Infantile haemangiomas and other vascular tumors. In: Irvine AD, Hoeger PH, Yan AC, eds. Harper’s textbook of pediatric dermatology, 3rd ed. Hoboken, NJ: Wiley-Blackwell, 2011:113–122. 4. Ritter M, Reinisch J, Friedlander Fallon S et al. Myeloid cells in infantile hemangioma. Am J Pathol 2006;168: 621–628. 5. Nathan C, Aihao D. Nonresolving inflammation. Cell 2010;140:871–882.
A 4-year-old Kenyan girl with severe atopic dermatitis, previously treated with cyclosporine, developed flat-topped papules on her neck 2 months after discontinuing therapy. The patient’s mother applied pimecrolimus 1% cream twice daily for 3 to 4 weeks. Three months later these papules had spread to her neck, chest, and upper extremities (Fig. 1). Biopsy demonstrated mild acanthosis and hypergranulosis. Keratinocytes with vacuolated blue cytoplasm were present throughout the epidermis,
Rosalind Hughes, M.B., B.C.H., B.A.O.* Maeve McAleer, M.B., B.C.H., B.A.O.* Rosemarie Watson, M.D., F.R.C.P.I.* Sinead Collins, M.B., B.C.H., B.A.O.† Alan Irvine, M.D., F.R.C.P.I.* Martin White, M.B., B.C.H., B.A.O.‡ *Department of Dermatology, Our Lady’s Hospital for Sick Children, Crumlin, Dublin, Ireland, †Department of Dermatology, Our Lady of Lourdes Hospital, Drogheda, Ireland, ‡Department of Neonatology, Our Lady’s Hospital for Sick Children, Crumlin, Dublin, Ireland Address correspondence to Rosalind Hughes, M.B., B.C.H., B.A.O., Department of Dermatology, Our Lady’s Hospital for Sick Children, Crumlin, Dublin 12, Ireland, or e-mail: [email protected].
Figure 1. Epidermodysplasia verruciformis. Hundreds of hypopigmented flat-topped papules distributed across the neck, upper trunk, and proximal arms.
