Letters to the Editor

trichoepitheliomas. Lack of CD34 staining in both lesions was in contrast to the study conducted by Illueca et  al.[8] that reported CD34 expression in multiple familial trichoepitheliomas but not in basal cell carcinoma. Taken together, as in other reports,[9,10] the immunomarkers used in our patient were not very helpful in differentiating these two conditions. Immunostaining with relatively novel markers such as androgen receptor, transforming growth factor‑β, p75 neurotrophin receptor and pleckstrin homology‑like domain, family A, member 1, (not currently available in our laboratory) may be more useful in this regard. Trichoepitheliomas can undergo transformation to malignant neoplasms such as basal cell carcinoma.[1] According to Pincus et al. (2008), six English language reports have appeared from 1959 to 2008 describing eleven patients who developed this malignant neoplasm from multiple familial trichoepitheliomas. Cases of sporadic trichoepitheliomas associated with basal cell carcinoma have also been reported. In four of the above reports, patients presented when the lesions started to bleed and/or ulcerate.[9] Interestingly, one of our patients (the grandmother) sought medical attention because of ulceration and pruritus in a lesion. The history, clincal features, histopathological and immunohistochemistry findings were suggestive of the evolution of basal cell carcinoma directly from trichoepithelioma in our case. Clinicians should examine these patients carefully and be vigilant about any rapid growth or ulceration in the pre‑existing lesions that may indicate malignant transformation.[9]

Mohammad Ali Mapar, Nastaran Ranjbari1, Nasim Afshar, Iman Karimzadeh2, Amin Karimzadeh Departments of Dermatology, and 1Pathology, Imam Khomeini Hospital, Jondishapur University of Medical Sciences, Ahvaz, Iran, 2 Department of Clinical Pharmacy, Faculty of Pharmacy, Tehran University of Medical Sciences, Tehran, Iran Address for correspondence: Dr. Amin Karimzadeh, Department of Dermatology, Imam Khomeini Hospital, Jondishapur University of Medical Sciences, Ahvaz, Iran. E‑mail: [email protected]

REFERENCES 1. Calonje E. Tumors of the skin appendages. In: Burns T, Breathnach S, Cox N, Griffiths C, editors. Rook’s Textbook of Dermatology. Chichester: Wiley‑Blackwell; 2010. 2. Poniecka AW, Alexis JB. An immunohistochemical study of basal cell carcinoma and trichoepithelioma. Am J Dermatopathol 1999;21:332‑6. 3. Ackerman AB, Reddy VB, Soyer HP. Neoplasms with Follicular 352

Differentiation. New York: Ardor Scribendi; 2001. 4. Young AL, Kellermayer R, Szigeti R, Tészás A, Azmi S, Celebi JT. CYLD mutations underlie Brooke‑Spiegler, familial cylindromatosis, and multiple familial trichoepithelioma syndromes. Clin Genet 2006;70:246‑9. 5. Verhaegh ME, Arends JW, Majoie IM, Hoekzema R, Neumann HA. Transforming growth factor‑beta and bcl‑2 distribution patterns distinguish trichoepithelioma from basal cell carcinoma. Dermatol Surg 1997;23:695‑700. 6. Abdelsayed RA, Guijarro‑Rojas M, Ibrahim NA, Sangueza OP. Immunohistochemical evaluation of basal cell carcinoma and trichepithelioma using Bcl‑2, Ki67, PCNA and P53. J Cutan Pathol 2000;27:169‑75. 7. Heidarpour M, Rajabi P, Sajadi F. CD10 expression helps to differentiate basal cell carcinoma from trichoepithelioma. J Res Med Sci 2011;16:938‑44. 8. Illueca C, Monteagudo C, Revert A, Llombart‑Bosch A. Diagnostic value of CD34 immunostaining in desmoplastic trichilemmoma. J Cutan Pathol 1998;25:435‑9. 9. Pincus LB, McCalmont TH, Neuhaus IM, Kasper R, Oh DH. Basal cell carcinomas arising within multiple trichoepitheliomas. J Cutan Pathol 2008;35 Suppl 1:59‑64. 10. Arits AH, Parren LJ, van Marion AM, Sommer A, Frank J, Kelleners‑Smeets NW. Basal cell carcinoma and trichoepithelioma: A possible matter of confusion. Int J Dermatol 2008;47 Suppl 1:13‑7. Access this article online Quick Response Code:

Website: www.ijdvl.com DOI: 10.4103/0378-6323.136924 PMID: *****

Panniculitis‑polyarthritis‑ pancreatitis syndrome Sir, Pancreatic panniculitis is a rare variant of panniculitis characterized by subcutaneous fat necrosis that affects 0.3‑3% of patients across a range of different pancreatic disorders. Skin lesions are the presenting feature in about 40% of pancreatic panniculitis and precede the abdominal symptoms by 1‑7 months.[1] Clinically, they present with painful, tender, ill‑defined, erythematous to violaceous nodules that may undergo spontaneous ulceration and discharge of an oily brown, viscous material resulting from liquefactive necrosis of adipocytes. These lesions usually involve the lower extremities although they may also appear on the buttocks, trunk, arms and scalp. In addition to the skin, fat necrosis may involve peri‑articular, abdominal and intramedullary adipose tissue. The

Indian Journal of Dermatology, Venereology, and Leprology | July-August 2014 | Vol 80 | Issue 4

Letters to the Editor

triad of pancreatitis, panniculitis and polyarthritis is known as pancreatitis, panniculitis and polyarthritis (PPP)‑syndrome.[2] It is a rare syndrome with high morbidity and mortality so a high index of suspicion is required to diagnose it and initiate timely and appropriate treatment.

a

c

b

d

Figure 1: Multiple tender subcutaneous nodules over the lower legs and abdomen (a and b) and swelling of the joints of hand and knee (c and d)

c

a

b Figure 2: (a) Lobular coagulative panniculitis involving several fat lobules (H and E, ×10) and (b) fat lobules are surrounded by inflammatory infiltrate of lipophages, lymphocytes and eosinophils with thickening of fat septa (H and E, ×40). (c) Atypical cells on cytology (×10)

a

b

Figure 3: Computed tomography scan showing (a) mass in the head of pancreas and (b) common bile duct

A 57‑year‑old man with a 20‑year history of heavy alcohol abuse presented with painful subcutaneous nodules on shins, buttocks, abdomen and shoulder accompanied by pain and swelling of both knees, ankles and elbows for the last two weeks. One month back, he had an episode of abdominal pain, nausea and vomiting. Records revealed that he was then diagnosed with acute pancreatitis and improved with symptomatic treatment. Physical examination revealed multiple tender subcutaneous nodules over the ankles, shins, thighs, abdomen and shoulders [Figure 1a and b]. There was no ulceration and oozing from the lesions. He had pallor and swelling and restricted mobility of the involved joints [Figure 1c and d]. The rest of the systemic examination was normal. Laboratory investigations revealed that the erythrocyte sedimentation rate was raised, C‑reactive protein was positive by latex agglutination method, gamma‑glutamyl transpeptidase was raised three times the normal level, serum lipase was twice the normal and serum amylase was grossly raised to about five times normal. Screening for hepatitis B and C was negative and the tumor marker, carcinoembryonic antigen was within the normal range. Biopsy of the skin nodule showed lobular panniculitis and intense necrosis of adipocytes surrounded by an inflammatory infiltrate of lymphocytes and eosinophils, features characteristic of pancreatic panniculitis [Figure 2a and b]. Ultrasonography guided fine-needle aspiration cytology revealed atypical cells [Figure 2c]. Joint X‑rays were normal. The abdominal computed tomography scan showed an ill‑defined hypodense mass, approximately 2.2 × 2.7 cm with a craniocaudal extent of 2.6 cm in the head of pancreas with the pancreatic duct dilated to 4 mm throughout its course [Figure 3a and b]. There were also multiple soft tissue nodules in the subcutaneous plane. These findings pointed towards the possibilities of chronic pancreatitis or adenocarcinoma of pancreas. With confirmation of panniculitis on histopathology and investigations confirming pancreatic disease and associated joint involvement, a diagnosis of PPP‑syndrome was made and the patient was referred to the department of surgery for further management. A triple bypass surgery for chronic pancreatitis was performed. There was good improvement in skin lesions and joint symptoms. In 80‑100% of cases of pancreatic panniculitis, the underlying condition is pancreatitis or pancreatic carcinoma, most commonly pancreatic

Indian Journal of Dermatology, Venereology, and Leprology | July-August 2014 | Vol 80 | Issue 4

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islet cell tumors, ductal adenocarcinoma and acinar‑cell carcinoma.[1] Other causes include pancreatic pseudocyst, post‑traumatic pancreatitis, pancreatic divisum or hemophogocytic syndrome. Pancreatic lipase and amylase are known to have a pathogenic role causing subcutaneous fat necrosis leading to panniculitis.[3] Ghost‑like anucleated cells with shadowy walls are pathognomonic findings of pancreatic panniculitis on skin biopsy.[4] PPP‑syndrome is a rare condition with about 25 well‑documented cases reported in the literature so far.[2] Early recognition of this triad is critical due to the high mortality from pancreatic disease when the diagnosis is delayed. Appearance of panniculitis in such cases and its detection can be life‑saving. [5]

Subhash Kashyap, Vinay Shanker, Sandhya Kumari, Lokesh Rana1 Departments of Dermatology, and 1Radiodiagnosis, Indira Gandhi Medical College, Shimla, Himachal Pradesh, India Address for correspondence: Dr. Subhash Kashyap, Department of Dermatology, Indira Gandhi Medical College, Shimla ‑ 171 001, Himachal Pradesh, India. E‑mail: [email protected]

REFERENCES 1.

Rongioletti F, Caputo V. Pancreatic panniculitis. G Ital Dermatol Venereol 2013;148:419‑25. 2. Narváez J, Bianchi MM, Santo P, de la Fuente D, Ríos‑Rodriguez V, Bolao F, et al. Pancreatitis, panniculitis, and polyarthritis. Semin Arthritis Rheum 2010;39:417‑23. 3. Borowicz J, Morrison M, Hogan D, Miller R. Subcutaneous fat necrosis/panniculitis and polyarthritis associated with acinar cell carcinoma of the pancreas: A rare presentation of pancreatitis, panniculitis and polyarthritis syndrome. J Drugs Dermatol 2010;9:1145‑50. 4. Gorovoy IR, McSorley J, Gorovoy JB. Pancreatic panniculitis secondary to acinar cell carcinoma of the pancreas. Cutis 2013;91:186‑90. 5. Kocabaş H, Melikoglu MA, Sezer I, Gurbuz U, Bulent B. Acute pancreatitis presenting with polyarthritis and intraosseous fat necrosis: A case report. Turk J Rheumatol 2010;25:221-4.

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354

EMPACT syndrome Sir, The term EMPACT syndrome (Erythema multiforme, Phenytoin and Cranial irradiation Therapy) was coined by Ahmed et al. to describe the development of erythema multiforme lesions in patients receiving cranial irradiation and phenytoin[1] and suggested that concomitant radiotherapy increases the adverse reaction potential of the anticonvulsant. We report a patient who manifested Stevens‑Johnson syndrome (SJS) while on phenytoin and cranial irradiation. A 46‑year‑old male presented with fever for 5 days and redness, scaling, edema and bullous lesions over the scalp for 4 days [Figure 1]. The skin lesions had started over the posterior aspect of scalp. On day 3 of the illness, he manifested a pruritic maculopapular rash on trunk and limbs [Figure 2], conjunctival congestion and erosions of lips [Figure 3]. He had undergone surgery for thalamic glioma 6 weeks perviously and had received whole‑brain radiation therapy (WBRT) and was on phenytoin 100 mg three times daily for seizure prophylaxis. The eruption developed after the 15th sitting of WBRT after a total radiation dose of 30 Gray and on the 37th day after starting phenytoin. He was not on any other medicines. Laboratory workup including complete hemogram, absolute eosinophil count, liver and renal function tests, peripheral smear analysis, chest radiography, and ultrasonography of abdomen were normal. A biopsy taken from a vesicle on the nape of neck showed vacuolar changes in the basal layer and a mild perivascular inflammatory infiltrate of eosinophils and lymphocytes in the dermis consistent with the diagnosis of erythema multiforme. We clinically labelled the patient as Stevens‑Johnson syndrome since he had constitutional symptoms and involvement of two mucosal surfaces in addition to the skin lesions. Since our patient was on radiotherapy and phenytoin, a final diagnosis of EMPACT syndrome was made. Phenytoin was replaced by levetiracetam. The patient had complete resolution of lesions with oral

Indian Journal of Dermatology, Venereology, and Leprology | July-August 2014 | Vol 80 | Issue 4

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Panniculitis-polyarthritis-pancreatitis syndrome.

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