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CR 2 Paraneoplastic Pemphigus Padhiyar JK.Indd Case Report http://dx.doi.org/10.3126/njdvl.v16i1.19416 Paraneoplastic Pemphigus Presenting as Toxic Epidermal Necrolysis Padhiyar JK1, Patel NH1, Ninama K2, Bilimoria FE2, Mahajan R2, Gajjar T1, Buch M1 1Gujarat Cancer Society Medical College, Hospital & Research Centre, Ahmedabad, Gujarat, India; 2Smt. B K Shah Medical Institute and Research Centre, Vadoddara, Gujarat, India. Abstract Polymorphous skin lesions have classically been described in paraneoplastic pemphigus (PNP), but it can present as toxic epidermal necrolysis (TEN) though this type of presentation is extremely rare. We report a case of PNP presenting as TEN in a young male patient. Patient had history of fever and diarrhoea six weeks before starting of lesions in oral cavity, for which he was treated with injectable medicines. Then patient developed generalized necrosis and peeling of skin with involvement of conjunctiva, oropharynx and genital mucosa. For this, the patient was given intravenous dexamethasone considering it as TEN, but after transient improvement initially skin lesions recurred when dose of dexamethasone was reduced. On seventh day, patient developed few circular deep ulcers over arms and back. Nikolsky sign was positive with tzanck smear showing acantholytic cells. Hence, we added PNP as one of the differential diagnosis. On further investigations patient was found to have B cell lymphoma in mediastinum and skin biopsy and direct immunofluorescence were confirmative of PNP. Unfortunately, patient then succumbed to death due to multi- organ failure and electrolyte imbalance. The onset of PNP can be as acute as TEN and clinical picture being initially undistinguishable, high index of suspicion is required in diagnosis. Key words: Acantholysis; fluorescent antibody technique, direct, pemphigus; stevens-johnson syndrome Introduction DIF in case of TEN shows diff use intraepidermal deposi on of immunoreactants.6 araneoplas c pemphigus (PNP) or paraneoplas c Pautoimmune mul organ syndrome (PAMS) is Camisa C et al has revised diagnos c criteria for PNP.7 characterized clinically by polymorphic skin lesions Major criteria include polymorphic skin erup ons, with neoplasia. As described by Anhalt GJ et al PNP neoplasia and immunoprecipita on of specifi c has mucocutaneous and systemic presenta on.1 PNP an bodies. Minor criteria include histological evidence presen ng as Toxic epidermal necrolysis (TEN) is very of acantholysis, DIF showing intercellular and basement rare.2,3 Most common malignancy reported with PNP is membrane staining and indirect immunofl uorescence B cell lymphoma.4,5 (IIF) staining with rat bladder epithelium. Three major or two major and two minor criteria are needed for Hisopathology from lesion showed acantholysis, diagnosis. suprabasal cle , dyskerato c kera nocytes, basal cell Submitted: 19th November 2017 vacuoliza on and exocytosis of infl ammatory cells. The Accepted: 15th January 2018 dis nc ve feature of PNP is presence of dyskerato c Published: 21st March 2018 kera nocytes in all layers of epidermis and around zone of acantholysis. Though immunoprecipita on How to cite this article is highly sensi ve, direct immunofl uorescence (DIF) Padhiyar JK, Patel NH, Ninama K, Bilimoria FE, Mahajan, showing combina on of intercellular and subepidermal R, Gajjar T, et al. Paraneoplastic pemphigus presenting deposi on of immunoreactants is a clue to diagnosis. as toxic epidermal necrolysis. Nepal Journal of Dermatology Venereology and Leprology. 2018;16(1):59-62. doi: http:// Address of Correspondence: dx.doi.org/10.3126/njdvl.v16i1.19416 Dr. Jignaben K Padhiyar, Assistant Professor Department of Dermatology, Venereology and Leprology Gujarat Cancer Society Medical College, Hospital & Research Centre, Opp. DRM Office, Nr. Chamunda Bridge, Naroda Road, Licensed under CC BY 4.0 International License which permits Ahmedabad-380025, Gujarat, India. use, distribution and reproduction in any medium, provided E-mail: [email protected], [email protected] the original work is properly cited. 59 Case report with loss of intercellular junc ons between suprabaslal kera nocytes and mixed infl ammatory infi ltrate in A 26-year-old male pa ent was referred to department superfi cial epidermis. DIF showed IgG posi vity in of dermatology from another ter ary care hospital. intercellular space in epidermis and at basement Pa ent had history of oral ulcera on for last six membrane zone (Figure 3). Computed Tomography weeks which had started a er treatment for episode (CT) scan (plain and contrast enhanced) scan showed of fever and diarrhoea with unknown injectable mass of 8.8 x 6.7 x 12 cm in posterior medias num medicine at primary care centre. Evalua on of case with mul ple subcentrimetric lymph nodes in record of referring ter ary care hospital revealed bilateral axillary region. CT guided biopsy of same was that pa ent was treated with intravenous 12 mg sugges ve of lymphoma. Immunohistochemistry of dexamethasone along with other suppor ve therapy. same showed posi vity for LCA and CD-20 sugges ng But pa ent then developed generalized skin necrosis B cell lymphoma (Figure 4). Pa ent was started on and peeling of skin (>90% BSA- body surface area) intravenous 12 mg of dexamethasone, cefi pime- along with involvement of conjunc va, oropharynx tazobactum 1.125 mg thrice daily, linezolide 600 twice and genital mucosa as the dose of dexamethasone daily along with other suppor ve therapy and local was reduced to 8 mg a er 7 days. So, dose was care. Despite of fl uid replacement with normal saline escalated to 12 mg of dexamethasone without pa ent had persistent hyponatremia. Serum An - remarkable improvement and pa ent was referred diure c hormone es ma on was not done because of to our centre. We con nued pa ent’s management non-availability at our centre. Pa ent’s haemoglobin with injectable 12 mg of dexamethasone, intravenous also decreased gradually to 4 gm/dl because of blood fl uid replacement, broad spectrum an bio cs keeping loss from erosions for which two packed cell volumes TEN as our primary diff eren al diagnosis. On 7th day (PCV) was given. Pa ent’s general condi on con nued of presenta on pa ent developed few circular deep to deteriorate hence chemotherapy could not be ulcers of < 1 cm size over back and upper limb over started. Eventually pa ent succumbed to death due to background of necrosed skin (Figure 1). There were metabolic acidosis (pCO2: 27.9, HCO3: 18.2, Lac c acid: few bullous lesions along with mul ple erosions in 3.02) and mul -organ failure on 13th day of admission. palms and diff use peeling in planter surface (Figure 2). Nikolsky’s sign was posi ve. Hence, we added Discussion PNP as one of the diff eren al diagnosis. Pa ent had low haemoglobin (8.8 gm/dl), electrolyte imbalance Most of the cases of PNP are reported beyond the age (Sodium-126.5, potassium 3.3, chloride-93.1 mmol/L), of 45 but our pa ent was quite young, only 26 years ESR-42 mm/hour and pus culture and sensi vity of age. 1,2,3,8 showed methicillin resistant Staphylococcus aureus and mul drug resistant Klebsiella. Tzank smear Ini ally our pa ent had generalized skin necrosis and showed mul ple acantholy c cells. Skin biopsy peeling of skin with mul ple mucosal sites involvement showed dyskerato c kera nocytes, suprabasal cle s and presence of misguiding history of taking some Figure 1: (a,b) Generalized skin necrosis with mucosal involvement (c) Development of circular deep ulcers over back on 7th day of admission. NJDVL. Vol 16, No.1, 2018 60 parenteral medicine followed by development of According to revised criteria for diagnosis of PNP oral and skin lesions prompted us to suspect TEN. proposed by Camisa C et al, our pa ent ini ally Development of mul ple ulcers, blisters and poor fulfi lled only one major criteria (neoplasia) with two response to therapy made us to revise our diff eren al minor criteria of histological acantholysis and posi ve diagnosis. In our pa ent clinical presenta on was DIF.7 On 7th day of admission when pa ent developed strongly sugges ve of TEN. Malignancy can be bullous lesions in hand and ulcers over back and hand, ae ology for both TEN and PNP, so it was diffi cult to another major criterion in the form of polymorphic diff eren ate both only on clinical presenta on. Despite lesions was fulfi lled. of extensive search we could only fi nd two cases of PNP mimicking TEN reported ll now.2,3 Mar n García et al Mortality and morbidity are high in pa ents of PNP has described confl uent area of denuda on involving owing to its mul organ involvement. Persistent 60% BSA in PNP mimicking TEN though lesions were hyponatremia despite of proper fl uid management polymorphic to start with.8 as in our pa ent may be because of paraneoplas c syndrome adding further complica on in managing a pa ent with skin failure. Respiratory complica ons and failure are high in pa ents of PNP.9 Our pa ent developed metabolic acidosis and eventually succumbed to death because of mul organ failure. Serum ADH level, immunoprecipita on and IIF was not done in our pa ent because of its non-availability at our ins tute as well as in our state. But, cases of PNP without detec on of auto-an bodies has also been reported10,11 with a sugges on that pathophysiological mechanisms may not be limited to humoral immunity when bullous lesions predominate, it may resemble gra versus host disease or lichen planus when cell mediated mechanisms are present.12 Figure 2: (a) Bullous lesions with erosions over palm (b) peeling of skin over soles. Figure 3: (a) Suprabasal cle s with dyskerato c kera nocytes.
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