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

Letters to the Editor

Although cutaneous intraneural perineurioma is benign, a slowly progressive course resulting in various degrees of deformity may occur.

Szu-Ying Chin1, Yu-Chien Kao2, Chia-Fen Yang3, Woan-Ruoh Lee1,4,5 a

c

b

Departments of 1Dermatology, and 2Pathology, Taipei Medical University, Shuang Ho Hospital, 3Department of Pathology, En Chu Kong Hospital, 5Department of Dermatology, School of Medicine, Taipei Medical University, Taipei, Taiwan, 4 Cancer Biology, Harvard University, U.S.A. Address for correspondence: Dr. Woan-Ruoh Lee, No. 291, Jhongzheng Road, Jhonghe, Taipei 23561, Taiwan. E-mail: [email protected]

d

Figure 2: (a) Concentric proliferation of spindle-shaped tumor cells displaying an onion-like appearance in superficial and middle dermis (arrowed), low power view (H and E, ×40). (b) High power view (H and E, ×200). (c) Epithelial membrane antigen (EMA) positivity in the tumor cells (Immunohistochemistry, EMA, ×200). (d) Only central myelinated axons were highlighted by S-100 protein (Immunohistochemistry, S-100 protein, ×200)

that typically presents in adolescents or young adults. The symptom of progressive muscle weakness, with or without obvious atrophy occurs more frequently than sensory disturbance.[1] Commonly affected sites include the large peripheral nerves of the extremities such as the ulnar, median, or sciatic nerves.[3] The location of the primary tumor in a cutaneous site is extremely rare and we found only one other similar published report.[2] This first case of cutaneous intraneural perineurioma was described by Santos-Briz A et al., whose patient was a 42-yearold man.[2] Both the previous case and our patient presented multiple asymptomatic dermal papules on the glabrous skin of the fingertips and the lateral aspect of distal phalanges. In the previous case, the patient’s right second finger was affected and showed an enlarged diameter, whereas our patient’s right second and third fingers were affected with medial deviation and deformity visible on the third finger. The glabrous skin of the hands is one of the most sensitive areas in the human body. Studies have shown that the density of nerves and mechanoreceptors increases markedly in a proximo-distal direction in the hands.[4,5] This might explain the predilection for the affected sites in both reported cases of cutaneous intraneural perineurioma. Neither patient complained of motor dysfunction or sensory disturbance. These symptoms are usually noted in patients with intraneural perineurioma involving the peripheral nerve of the extremities. The reason for this difference between the cutaneous form and traditional lesions has yet to be determined.

REFERENCES 1.

2. 3.

4. 5.

Scheithauer BW, Woodruff JM, Antonescu CR. Perineurioma. In: Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, editors. World Health Organization Classification of Tumours – Pathology and Genetics of Tumours of the Nervous System. France, Lyon: International Agency for Research on Cancer; 2007. p. 169-71. Santos-Briz A, Godoy E, Cañueto J, García JL, Mentzel T. Cutaneous intraneural perineurioma: A case report. Am J Dermatopathol 2013;35:e45-8. Boyanton BL Jr, Jones JK, Shenaq SM, Hicks MJ, Bhattacharjee MB. Intraneural perineurioma: A systematic review with illustrative cases. Arch Pathol Lab Med 2007;131:1382-92. Johansson RS, Vallbo AB. Tactile sensibility in the human hand: Relative and absolute densities of four types of mechanoreceptive units in glabrous skin. J Physiol 1979;286:283-300. Kelly EJ, Terenghi G, Hazari A, Wiberg M. Nerve fibre and sensory end organ density in the epidermis and papillary dermis of the human hand. Br J Plast Surg 2005;58:774-9. Access this article online Quick Response Code:

Website: www.ijdvl.com DOI: 10.4103/0378-6323.129412 PMID: 129412

Mondor’s disease Sir, Henry Mondor first described superficial vein thrombosis involving the subcutaneous veins of the anterolateral thoracoabdominal wall in 1939.[1] It is a relatively rare entity, especially in males.[2] Most cases are idiopathic, although varied etiologies such as trauma, surgery, exercise, jellyfish sting, procoagulant states, and breast cancer have been proposed.[3] Herein, we report a case of Mondor’s disease in a male patient

Indian Journal of Dermatology, Venereology, and Leprology | March-April 2014 | Vol 80 | Issue 2

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Distinctive manifestations of cutaneous intraneural perineurioma of fingers, compared with typical intraneural perineurioma.

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