Brief Reports 397
difference in our formula is the way in which the active agents are added to the water phase of the emulsion to obtain better homogeneity in the final product. An effective and easy-to-use cream is obtained with this formula, making topical Nacetylcysteine an excellent therapy in the management of ichthyosis. CONCLUSION We report a case of inherited lamellar ichthyosis that showed a good response to treatment with a new formula of topical N-acetylcysteine cream. With this new formula the odor of sulfur was eliminated and we obtained excellent adherence in the use of N-acetylcysteine cream. REFERENCES 1. Redondo P, Bauza A. Topical N-acetylcysteine for lamellar ichthyosis. Lancet 1999;354:1880. 2. Sarici SU, Sahin M, Yurdakok M. Topical N-acetylcysteine treatment in neonatal ichthyosis. Turk J Pediatr 2003;45:245–247. 3. Bassotti A, Moreno S, Criado E. Successful treatment with topical N-acetylcysteine in urea in five children with congenital lamellar ichthyosis. Pediatr Dermatol 2011; 28:451–455. 4. Deffenbacher B. Successful experimental treatment of congenital ichthyosis in an infant. BMJ Case Rep 2013 Mar 6;2013. pii: bcr2013008688. doi: 10.1136/bcr-2013008688. 5. Abajo del Alamo C, Aparicio Lucas L, Canoura Fernanfez L. 10% N-acetilcistein in cold cream pomade: development and clinical application in lamellar ichthyosis. Poster communication. 54 SEFH Congress 2009. Paula Davila-Seijo, M.D.* Angeles Fl orez, Ph.D.* Carmen Davila-Pousa, M.D.† Nuria No, M.D.* Carlos Ferreira, M.D.† Carlos De la Torre, Ph.D.* *Department of Dermatology and †Department of Pharmacy, Complexo Hospitaladio de Pontevedra, Pontevedra, Spain
typically appears as a soft mass with a blue hue on the scalp. We describe a patient with sinus pericranii and associated hair collar sign.
Sinus pericranii is a rare anomalous communication in which the extracranial veins are connected to the intracranial venous system (typically a transosseous emissary vein connecting a scalp varix with the dural venous sinus). It has a variable clinical appearance. We describe an unusual finding of midline sinus pericranii with an associated hair collar sign. A 53-day-old boy was seen for a congenital scalp nodule with surrounding hair growth. The nodule had been increasing in size since birth. It became red and swollen when he cried and was tender to touch. The infant was born via normal vaginal delivery without instrumentation. Examination of the midline vertex of the scalp showed a soft, nonpulsatile nodule with surrounding dark terminal hairs forming an irregular hair collar (Fig. 1). The nodule was in the center of a light red capillary stain that became more obvious when the patient cried. There were no other midline defects. Magnetic resonance imaging (MRI) with and without contrast showed findings consistent with sinus pericranii (Fig. 2). An appointment at a multidisciplinary vascular anomalies clinic for further examination and treatment is pending. Sinus pericranii typically appears as a soft, fluctuant, pulsatile or nonpulsatile, blue or red mass fixed to the scalp. The malformation can be described as a “hair of snakes” secondary to the twisting and dilatation of the underlying veins. Sometimes there is overlying alopecia (1). The mass tends to increase in size with crying or the Valsalva maneuver and decreases in size with standing. Congenital sinus pericranii is usually located in the midline frontal region of the scalp (40%)
Address correspondence to Paula Davila-Seijo, Department of Dermatology, Complexo Hospitalario de Pontevedra, Loureiro Crespo Nº 2, Pontevedra, Spain, or e-mail: pauladavilaseijo@gmail. com.
Sinus Pericranii with a Hair Collar Sign Abstract: Sinus pericranii is a rare vascular malformation in which the extracranial veins are connected to the intracranial venous circulation. It
Figure 1. A soft nodule with an irregular hair collar of dark terminal hairs.
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
difference in our formula is the way in which the active agents are added to the water phase of the emulsion to obtain better homogeneity in the final product. An effective and easy-to-use cream is obtained with this formula, making topical Nacetylcysteine an excellent therapy in the management of ichthyosis. CONCLUSION We report a case of inherited lamellar ichthyosis that showed a good response to treatment with a new formula of topical N-acetylcysteine cream. With this new formula the odor of sulfur was eliminated and we obtained excellent adherence in the use of N-acetylcysteine cream. REFERENCES 1. Redondo P, Bauza A. Topical N-acetylcysteine for lamellar ichthyosis. Lancet 1999;354:1880. 2. Sarici SU, Sahin M, Yurdakok M. Topical N-acetylcysteine treatment in neonatal ichthyosis. Turk J Pediatr 2003;45:245–247. 3. Bassotti A, Moreno S, Criado E. Successful treatment with topical N-acetylcysteine in urea in five children with congenital lamellar ichthyosis. Pediatr Dermatol 2011; 28:451–455. 4. Deffenbacher B. Successful experimental treatment of congenital ichthyosis in an infant. BMJ Case Rep 2013 Mar 6;2013. pii: bcr2013008688. doi: 10.1136/bcr-2013008688. 5. Abajo del Alamo C, Aparicio Lucas L, Canoura Fernanfez L. 10% N-acetilcistein in cold cream pomade: development and clinical application in lamellar ichthyosis. Poster communication. 54 SEFH Congress 2009. Paula Davila-Seijo, M.D.* Angeles Fl orez, Ph.D.* Carmen Davila-Pousa, M.D.† Nuria No, M.D.* Carlos Ferreira, M.D.† Carlos De la Torre, Ph.D.* *Department of Dermatology and †Department of Pharmacy, Complexo Hospitaladio de Pontevedra, Pontevedra, Spain
typically appears as a soft mass with a blue hue on the scalp. We describe a patient with sinus pericranii and associated hair collar sign.
Sinus pericranii is a rare anomalous communication in which the extracranial veins are connected to the intracranial venous system (typically a transosseous emissary vein connecting a scalp varix with the dural venous sinus). It has a variable clinical appearance. We describe an unusual finding of midline sinus pericranii with an associated hair collar sign. A 53-day-old boy was seen for a congenital scalp nodule with surrounding hair growth. The nodule had been increasing in size since birth. It became red and swollen when he cried and was tender to touch. The infant was born via normal vaginal delivery without instrumentation. Examination of the midline vertex of the scalp showed a soft, nonpulsatile nodule with surrounding dark terminal hairs forming an irregular hair collar (Fig. 1). The nodule was in the center of a light red capillary stain that became more obvious when the patient cried. There were no other midline defects. Magnetic resonance imaging (MRI) with and without contrast showed findings consistent with sinus pericranii (Fig. 2). An appointment at a multidisciplinary vascular anomalies clinic for further examination and treatment is pending. Sinus pericranii typically appears as a soft, fluctuant, pulsatile or nonpulsatile, blue or red mass fixed to the scalp. The malformation can be described as a “hair of snakes” secondary to the twisting and dilatation of the underlying veins. Sometimes there is overlying alopecia (1). The mass tends to increase in size with crying or the Valsalva maneuver and decreases in size with standing. Congenital sinus pericranii is usually located in the midline frontal region of the scalp (40%)
Address correspondence to Paula Davila-Seijo, Department of Dermatology, Complexo Hospitalario de Pontevedra, Loureiro Crespo Nº 2, Pontevedra, Spain, or e-mail: pauladavilaseijo@gmail. com.
Sinus Pericranii with a Hair Collar Sign Abstract: Sinus pericranii is a rare vascular malformation in which the extracranial veins are connected to the intracranial venous circulation. It
Figure 1. A soft nodule with an irregular hair collar of dark terminal hairs.
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
