Letters to the Editor
Periocular granuloma annulare in a child Sir, Granuloma annulare is a benign, inflammatory disorder of the dermis and/or subcutis characterized by degeneration of connective tissue surrounded by lymphocytes and histiocytes. It can occur at any age but is more commonly seen in children and young adults. Characteristically, there are asymptomatic, skin colored firm, papules or plaques in an annular pattern on the trunk and extremities.[1] It is rare in the periocular region where nodules with a predilection for upper eyelids have been reported. We found 14 previously published cases of pediatric periocular granuloma, but none from India. An 8-year-old boy was referred by the ophthalmologist for evaluation of asymptomatic, recurrent papules and nodules on both upper eyelids and eyebrows for 4 years. The lesions first appeared on the lateral brow at the age of 4 years, spontaneously disappeared in a few months and recurred at multiple sites. Cutaneous examination revealed multiple, non-tender, mobile, skin colored nodules measuring 3-5 mm located on both upper eyelids, medial and lateral canthi and the left eyebrow [Figure 1]. Ophthalmological examination was normal. Skin biopsy from the lateral canthus showed a normal epidermis with mild interstitial and perivascular lymphocytic infiltrates. A palisade of histiocytes and lymphocytes was seen surrounding a large area of mucin in the middle and deep dermis [Figure 2a and b]. The lesions resolved without treatment within 2 weeks [Figure 3]. Localized, generalized, subcutaneous and perforating granuloma annulare are the most common variants of the condition while periocular granuloma annulare is quite rare.[2] Firm, skin-colored nodules similar to rheumatoid nodules are seen in the periocular region and some case reports refer to these lesions as “pseudorheumatoid nodules,” although they are not related to rheumatoid arthritis.[3] Though the etiology of granuloma annulare is not known, implicated trigger factors include local trauma, sun exposure, cutaneous infections, insect bites, diabetes, tuberculosis, sarcoidosis and hereditary predisposition. The age of onset in previous reports of pediatric periocular granuloma annulare ranged from 1 year to 17 years and there was no sex predilection. Most patients were African American. No co-morbidities were reported in any children.[4] Differential diagnoses include eyelid lesions
Figure 1: Multiple nodules on both upper eyelids, medial and lateral canthus
a
b
Figure 2: (a) Normal epidermis with an area showing mucin in the deep dermis (H and E, ×40), (b) Higher magnification showing mucin deposition surrounded by a palisade of histiocytes and a few lymphocytes (H and E, ×400)
Figure 3: Nodules have resolved on left upper eyelid and lateral canthus
such as chalazion, lupus miliaris disseminatus faciei, tuberculosis, sarcoidosis, childhood granulomatous periorificial dermatitis and granulomatous infectious processes.[5] Periocular granuloma annulare regresses spontaneously without treatment. Therapeutic interventions need to be considered only if there is functional visual impairment. Recurrences vary from 15% to 40% and regular follow-up is necessary. Parents should be counseled about the uneventful natural course of the disease to allay anxiety.
Indian Journal of Dermatology, Venereology, and Leprology | March-April 2014 | Vol 80 | Issue 2
171
Letters to the Editor
Sahana M. Srinivas, Moupia Goswami1, Madhavi Naik2 Department of Pediatric Dermatology, Indira Gandhi Institute of Child Health, 1Department of Ophthalmology, Prabha Eye Clinic, 2 Department of Pathology, St. Theresa’s Hospital, Bengaluru, Karnataka, India Address for correspondence: Dr. Sahana M. Srinivas, Department of Pediatric Dermatology, Indira Gandhi Institute of Child Health, Bengaluru, Karnataka, India. E-mail: [email protected]
REFERENCES 1.
2. 3.
4. 5.
Cronquist SD, Stashower ME, Benson PM. Deep dermal granuloma annulare presenting as an eyelid tumor in a child, with review of pediatric eyelid lesions. Pediatr Dermatol 1999;16:377-80. De Becker I, Summers CG, Kaye V, Traboulsi EI, Schoenfield L, Markowitz G, et al. Periocular granuloma annulare in four children. J AAPOS 2010;14:280-2. Burnstine MA, Headington JT, Reifler DM, Oestreicher JH, Elner VM. Periocular granuloma annulare, nodular type. Occurrence in late middle age. Arch Ophthalmol 1994;112:1590-3. Chiang K, Bhalla R, Mesinkovska NA, Piliang MP, Tamburro JE. Periocular granuloma annulare: A case report and review of literature. Pediatr Dermatol 2013; [In Press]. Goldstein SM, Douglas RS, Binenbaum G, Katowitz JA. Paediatric periocular granuloma annulare. Acta Ophthalmol Scand 2003;81:90-1. Access this article online Quick Response Code:
Website: www.ijdvl.com DOI: 10.4103/0378-6323.129410 PMID: 129410
Distinctive manifestations of cutaneous intraneural perineurioma of fingers, compared with typical intraneural perineurioma Sir, Perineurioma is an uncommon tumor composed entirely of neoplastic perineural cells. The disease is classified into two variants with distinct clinicopathological manifestations, an intraneural subtype and an extraneural soft tissue sub-type. Both variants are rare, and each represents approximately 1% of all 172
cases of nerve sheath and soft tissue neoplasms.[1] The intraneural type is rarer than the extraneural type. Only one case report of the cutaneous form of intraneural perineurioma has been published.[2] Our report describes a 33-year-old woman who presented to the Dermatology Department with asymptomatic skin lesions on her right second and third fingers which had been present for approximately 20 years. There were multiple small skin-colored papules with an elastic consistency involving the palmar and lateral sides of the distal phalanx. The diameters of both fingers were asymmetrically enlarged, predominantly on the medial side [Figure 1]. The third finger showed earlier and more severe invasion compared with the second finger, along with lateral deviation. X-rays showed that the bone structure was intact. We observed no neurofibroma or cafe au lait spots on her skin. She denied any history of trauma. No one else in the family had a similar illness. An incisional biopsy performed on the third fingertip showed numerous concentric proliferative nests of spindle-shaped cells in the dermis arranged in a structure shaped like an onion-bulb. Immunohistochemical analysis showed that the proliferative tumor cells were positive for the epithelial membrane antigen and negative for S-100 protein. The S-100 protein highlighted only the central myelinated axons [Figure 2]. A diagnosis of cutaneous intraneural perineurioma was confirmed. Because our patient experienced no functional impairment and we found no evidence of a malignant transformation of the intraneural perineurioma,[3] a clinical follow-up was deemed the best option. Intraneural perineurioma is a rare and benign neoplasm
Figure 1: Multiple small skin-colored and elastic papules involving the glabrous skin of distal and focal middle phalanges of the right second and third fingers
Indian Journal of Dermatology, Venereology, and Leprology | March-April 2014 | Vol 80 | Issue 2
Copyright of Indian Journal of Dermatology, Venereology & Leprology is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
Periocular granuloma annulare in a child Sir, Granuloma annulare is a benign, inflammatory disorder of the dermis and/or subcutis characterized by degeneration of connective tissue surrounded by lymphocytes and histiocytes. It can occur at any age but is more commonly seen in children and young adults. Characteristically, there are asymptomatic, skin colored firm, papules or plaques in an annular pattern on the trunk and extremities.[1] It is rare in the periocular region where nodules with a predilection for upper eyelids have been reported. We found 14 previously published cases of pediatric periocular granuloma, but none from India. An 8-year-old boy was referred by the ophthalmologist for evaluation of asymptomatic, recurrent papules and nodules on both upper eyelids and eyebrows for 4 years. The lesions first appeared on the lateral brow at the age of 4 years, spontaneously disappeared in a few months and recurred at multiple sites. Cutaneous examination revealed multiple, non-tender, mobile, skin colored nodules measuring 3-5 mm located on both upper eyelids, medial and lateral canthi and the left eyebrow [Figure 1]. Ophthalmological examination was normal. Skin biopsy from the lateral canthus showed a normal epidermis with mild interstitial and perivascular lymphocytic infiltrates. A palisade of histiocytes and lymphocytes was seen surrounding a large area of mucin in the middle and deep dermis [Figure 2a and b]. The lesions resolved without treatment within 2 weeks [Figure 3]. Localized, generalized, subcutaneous and perforating granuloma annulare are the most common variants of the condition while periocular granuloma annulare is quite rare.[2] Firm, skin-colored nodules similar to rheumatoid nodules are seen in the periocular region and some case reports refer to these lesions as “pseudorheumatoid nodules,” although they are not related to rheumatoid arthritis.[3] Though the etiology of granuloma annulare is not known, implicated trigger factors include local trauma, sun exposure, cutaneous infections, insect bites, diabetes, tuberculosis, sarcoidosis and hereditary predisposition. The age of onset in previous reports of pediatric periocular granuloma annulare ranged from 1 year to 17 years and there was no sex predilection. Most patients were African American. No co-morbidities were reported in any children.[4] Differential diagnoses include eyelid lesions
Figure 1: Multiple nodules on both upper eyelids, medial and lateral canthus
a
b
Figure 2: (a) Normal epidermis with an area showing mucin in the deep dermis (H and E, ×40), (b) Higher magnification showing mucin deposition surrounded by a palisade of histiocytes and a few lymphocytes (H and E, ×400)
Figure 3: Nodules have resolved on left upper eyelid and lateral canthus
such as chalazion, lupus miliaris disseminatus faciei, tuberculosis, sarcoidosis, childhood granulomatous periorificial dermatitis and granulomatous infectious processes.[5] Periocular granuloma annulare regresses spontaneously without treatment. Therapeutic interventions need to be considered only if there is functional visual impairment. Recurrences vary from 15% to 40% and regular follow-up is necessary. Parents should be counseled about the uneventful natural course of the disease to allay anxiety.
Indian Journal of Dermatology, Venereology, and Leprology | March-April 2014 | Vol 80 | Issue 2
171
Letters to the Editor
Sahana M. Srinivas, Moupia Goswami1, Madhavi Naik2 Department of Pediatric Dermatology, Indira Gandhi Institute of Child Health, 1Department of Ophthalmology, Prabha Eye Clinic, 2 Department of Pathology, St. Theresa’s Hospital, Bengaluru, Karnataka, India Address for correspondence: Dr. Sahana M. Srinivas, Department of Pediatric Dermatology, Indira Gandhi Institute of Child Health, Bengaluru, Karnataka, India. E-mail: [email protected]
REFERENCES 1.
2. 3.
4. 5.
Cronquist SD, Stashower ME, Benson PM. Deep dermal granuloma annulare presenting as an eyelid tumor in a child, with review of pediatric eyelid lesions. Pediatr Dermatol 1999;16:377-80. De Becker I, Summers CG, Kaye V, Traboulsi EI, Schoenfield L, Markowitz G, et al. Periocular granuloma annulare in four children. J AAPOS 2010;14:280-2. Burnstine MA, Headington JT, Reifler DM, Oestreicher JH, Elner VM. Periocular granuloma annulare, nodular type. Occurrence in late middle age. Arch Ophthalmol 1994;112:1590-3. Chiang K, Bhalla R, Mesinkovska NA, Piliang MP, Tamburro JE. Periocular granuloma annulare: A case report and review of literature. Pediatr Dermatol 2013; [In Press]. Goldstein SM, Douglas RS, Binenbaum G, Katowitz JA. Paediatric periocular granuloma annulare. Acta Ophthalmol Scand 2003;81:90-1. Access this article online Quick Response Code:
Website: www.ijdvl.com DOI: 10.4103/0378-6323.129410 PMID: 129410
Distinctive manifestations of cutaneous intraneural perineurioma of fingers, compared with typical intraneural perineurioma Sir, Perineurioma is an uncommon tumor composed entirely of neoplastic perineural cells. The disease is classified into two variants with distinct clinicopathological manifestations, an intraneural subtype and an extraneural soft tissue sub-type. Both variants are rare, and each represents approximately 1% of all 172
cases of nerve sheath and soft tissue neoplasms.[1] The intraneural type is rarer than the extraneural type. Only one case report of the cutaneous form of intraneural perineurioma has been published.[2] Our report describes a 33-year-old woman who presented to the Dermatology Department with asymptomatic skin lesions on her right second and third fingers which had been present for approximately 20 years. There were multiple small skin-colored papules with an elastic consistency involving the palmar and lateral sides of the distal phalanx. The diameters of both fingers were asymmetrically enlarged, predominantly on the medial side [Figure 1]. The third finger showed earlier and more severe invasion compared with the second finger, along with lateral deviation. X-rays showed that the bone structure was intact. We observed no neurofibroma or cafe au lait spots on her skin. She denied any history of trauma. No one else in the family had a similar illness. An incisional biopsy performed on the third fingertip showed numerous concentric proliferative nests of spindle-shaped cells in the dermis arranged in a structure shaped like an onion-bulb. Immunohistochemical analysis showed that the proliferative tumor cells were positive for the epithelial membrane antigen and negative for S-100 protein. The S-100 protein highlighted only the central myelinated axons [Figure 2]. A diagnosis of cutaneous intraneural perineurioma was confirmed. Because our patient experienced no functional impairment and we found no evidence of a malignant transformation of the intraneural perineurioma,[3] a clinical follow-up was deemed the best option. Intraneural perineurioma is a rare and benign neoplasm
Figure 1: Multiple small skin-colored and elastic papules involving the glabrous skin of distal and focal middle phalanges of the right second and third fingers
Indian Journal of Dermatology, Venereology, and Leprology | March-April 2014 | Vol 80 | Issue 2
Copyright of Indian Journal of Dermatology, Venereology & Leprology is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
