Geeti Khullar

Tumor necrosis factor-α antagonists: Side effects and their management

As elevated levels of tumor necrosis factor-alpha (TNF-α) are associated with disease severity in psoriasis and psoriatic arthritis, TNF-α antagonists are being used to treat moderate to severe disease in patients who have contraindications, fail to respond or develop side effects to conventional systemic therapies. It is of utmost importance to be well versed with the possible adverse effects and contraindications of TNF-α antagonists so that they can be used effectively and safely. Many of their adverse effects have been well studied in patients of rheumatoid arthritis (RA) and inflammatory bowel disease (IBD) and may not be completely applicable in psoriasis. This is because patients with RA and IBD are on multiple immunosuppressants while those with psoriasis are mostly receiving single systemic therapy and often have comorbidities that distinguish them from those with RA or IBD. Also, some of the side effects are still controversial and debated. Long-term prospective randomized controlled studies are needed to better understand the associated risk in patients of psoriasis. Baseline screening and periodic monitoring during treatment can reduce and help in early identification and appropriate management of the adverse outcomes. This article reviews the side effects known to be associated with TNF-α antagonists, their pathomechanisms and management guidelines. Some of the common side effects include infusion and injection site reactions, infections particularly reactivation of tuberculosis, autoantibody formation and drug induced lupus erythematosus, liver function abnormalities, hematological, and solid organ malignancies.

Nonmelanoma skin cancers: An Indian perspective

Nonmelanoma skin cancers (NMSCs), which mainly include basal cell carcinoma (BCC) and squamous cell carcinoma (SCC), are infrequent in the Indian subcontinent, compared with white skinned individuals. Although BCC in most cases arises de novo on sun-exposed sites, it may rarely develop in photoprotected areas and in the setting of certain risk factors. In contrast to BCC, SCC in dark skin has a tendency to develop in nonhealing ulcers, chronic scars, and inflammatory and infectious dermatoses. Histopathology is the gold standard in confirming the diagnosis and determining the prognosis. As the existing literature on NMSCs in India is limited mostly to case reports and few reviews only, this article is an attempt to create an awareness regarding the premalignant potential of an expanding list of cutaneous lesions, which would help in timely diagnosis and prompt treatment of NMSCs.

Clinical, demographic and immunopathological spectrum of subepidermal autoimmune bullous diseases at a tertiary center: A 1-year audit.

Background: The subepidermal autoimmune bullous diseases are a subset of immunobullous diseases encountered less frequently in the Indian population. There is a paucity of data on the prevalence, demographic and clinicopathological spectrum of various subepidermal autoimmune bullous diseases from India. Aim: To determine the demographic and clinicopathological profile of subepidermal autoimmune bullous diseases in Indian patients, presenting to the Immunobullous Disease Clinic of Postgraduate Institute of Medical Education and Research, Chandigarh. Methods: Patients seen from November 2013 to November 2014 who fulfilled the preset diagnostic criteria of subepidermal autoimmune bullous diseases were identified from case records. Data regarding demographic characteristics, clinical profile, immunopathological findings and treatment were collected from the predesigned proforma. Results: Of 268 cases of autoimmune bullous diseases registered, 50 (18.7%) were subepidermal autoimmune bullous diseases. Bullous pemphigoid was most frequently seen in 20 (40%) cases, followed by dermatitis herpetiformis in 14 (28%), mucous membrane pemphigoid in 6 (12%), chronic bullous dermatosis of childhood / linear immunoglobulin A bullous dermatosis in 5 (10%), lichen planus pemphigoides in 3 (6%), pemphigoid gestationis and epidermolysis bullosa acquisita in 1 (2%) case each. None of the patients had bullous systemic lupus erythematosus. Limitations: We could not perform direct and indirect immunofluorescence using salt-split skin as a substrate and immunoblotting due to non-availability of these facilities. Therefore, misclassification of subepidermal autoimmune bullous diseases in some cases cannot be confidently excluded. Conclusion: Subepidermal autoimmune bullous diseases are not uncommon in Indian patients. Bullous pemphigoid contributes maximally to the burden of subepidermal autoimmune bullous diseases in India, similar to that in the West, although the proportion is lower and disease onset is earlier. Dermatitis herpetiformis was observed to have a higher prevalence in our population, compared to that in the West and the Far East countries. The prevalence of other subepidermal autoimmune bullous diseases is relatively low. Detailed immunofluorescence and immunoblotting studies on larger patient numbers would help better characterize the pattern of subepidermal autoimmune bullous diseases and their features in Indian patients.

Lipomatous neurofibromas with giant pigmented lesion

Neurofibroma is the most common benign neural tumour. Among the large number of histopathological variants reported, lipomatous neurofibroma is exceedingly infrequent, and shows the presence of intratumoral adipocytes admixed with spindle cells in the dermis. We present a case of a 37‐year‐old man with lipomatous neurofibromas associated with a giant garment‐like pigmented lesion involving his lower trunk and right thigh.

Granulomatous Invasive Aspergillosis of Paranasal Sinuses Masquerading as Actinomycosis and Review of Published Literature

Cutaneous aspergillosis is a common systemic mycosis affecting immunosuppressed patients. Here, we describe a novel morphological type of cutaneous aspergillosis in a young immunocompetent woman who presented with a chronic history of multiple nodules and discharging sinuses over left side of the face, mimicking cervicofacial actinomycosis. Skin biopsy showed granulomatous inflammation, and of septate fungal hyphae with acute-angled branching, morphologically resembling Aspergillus. This was confirmed on fungal culture as Aspergillus flavus.

Congenital Onychoheterotopia Involving Multiple Toe Nails

Onychoheterotopia is an uncommon condition in which nail tissue is found beyond the common nail unit of the digits of the hands and feet, most often on the fifth digit of the hand. It represents an extra and independent nail that can be present either congenitally, or more commonly, acquired following trauma. The exact pathogenesis of the congenital type is undetermined. We report a 25-year-old male with multiple congenital ectopic nails of the toes since birth, which has not been reported before.

Correlation between salivary and serum anti‐desmoglein 1 and 3 antibody titres using ELISA and between anti‐desmoglein levels and disease severity in pemphigus vulgaris

ELISA for anti‐desmoglein antibodies (Dsg) is commonly used for diagnosis and assessment of treatment response in pemphigus vulgaris (PV). The present study was conducted to assess the relationship between salivary and serum Dsg1 and Dsg3 levels, and whether salivary Dsg1 and Dsg3 levels correlate with clinical disease severity of oral mucosal lesions in PV. In total 43, patients with PV with predominantly mucosal involvement were recruited. Both serum and salivary samples were collected from the cases, and salivary samples were also collected from five controls. There was a statistically significant correlation between serum and salivary Dsg1 levels and between serum and salivary Dsg3 levels. There was no correlation between serum or salivary Dsg1 and Dsg3 levels with the objective component of the oral mucosal Autoimmune Bullous Skin Disorder Intensity Score (ABSIS). Serum Dsg1 levels significantly correlated with cutaneous ABSIS, but there was no correlation between cutaneous ABSIS and either salivary Dsg1, salivary Dsg3 or serum Dsg3. As salivary Dsg titres correlate with serum levels, saliva can serve as a simple and noninvasive alternative to serum for Dsg ELISA.

Bullous Lichen Planus of the Nails

Additional Contributions: We thank Shu-Tuan Chiang, MSc, Chuang Song-Zong Pharmaceutical Co, Ltd, for the preparation of the study medication and Hsiao-Jung Tseng, MS, Biostatistical Center for Clinical Research (supported by grant CLRPG340599 from Chang Gung Memorial Hospital at Linkou) and Louis Tuan, MBA, Contract Research Organization Service Division, Formosa Biomedical Technology Corp, for statistical analysis of the clinical data. Chuang Song-Zong Pharmaceutical Co, Ltd, was compensated for producing the drugs used in the study. Ms Tseng and Mr Tuan were not compensated for their contributions.

Isolated corymbose collagenoma responding to intralesional triamcinolone acetonide and hyaluronidase injections.

Collagenomas are connective tissue nevi with circumscribed hamartomatous proliferation of collagen. Due to their benign nature and lack of any simple medical treatment, they are most often left untreated. We present a case of isolated corymbose collagenoma, a distinct morphological variant not described hitherto that was successfully treated with intralesional injections of combination of triamcinolone acetonide and hyaluronidase.

An unusual case of idiopathic localized giant cell lichenoid dermatitis

To the Editor, Giant cell lichenoid dermatitis (GCLD) is a rare lichenoid reaction with distinct histological features. It presents clinically as a generalized erythematous to lichenoid papulosquamous eruption usually after drug intake. We present a unique case of GCLD that was localized in distribution and occurred in the absence of any underlying etiology. A 52-year-old woman presented with 1 year history of mildly itchy erythematous papules on her right lower limb. There was no history of preceding drug intake, cough, shortness of breath, ocular or any other systemic complaints. There was no history of any prior treatment and the lesions did not increase in number or improve spontaneously. Physical examination revealed monomorphic, erythematous to lichenoid, flat-topped papules (15-20 in number) measuring 0.5 cm in size with overlying adherent white scaling on the lateral aspect of her right leg and thigh in a dermatomal distribution (Figure 1). Mucosae and nails were not involved. Mantoux test measured 5 × 5 mm after 72 hours. Angiotensin converting enzyme levels, chest radiograph and ultrasound of the abdomen were normal. Skin biopsy revealed parakeratosis, basal cell vacuolization, occasional apoptotic keratinocytes, dense band-like lympho-histiocytic infiltrate with interspersed multinucleated giant cells in the upper dermis and melanin incontinence (Figure 2A and B). No acid fast bacilli or fungal hyphae were identified on special stains. Based on the clinical and histological findings, a diagnosis of giant cell lichenoid dermatitis was made. Giant cell lichenoid dermatitis (GCLD), initially described in 1986, is an uncommon lichenoid reaction with unique histopathologic features. Clinically it is characterized by itchy erythematous and violaceous macules, papules, plaques or nodules with or without scaling, usually distributed on the trunk and extremities, although face, scalp, palms and mucosa may also be involved. The exact pathogenesis of this entity is unknown. It was first reported as a lichenoid drug reaction presenting as generalized pruritic papulosquamous eruption secondary to methyldopa and chlorothiazide in a patient with systemic lupus erythematosus. Goldberg et al reported 3 cases of GCLD, all of whom had drugs as the inciting factor. GCLD has also been reported in a patient with baboon syndrome 3 days after administering amoxicillin-clavulanic acid. The strong association of GCLD with drug intake is supported by subsidence of the lesions after drug withdrawal and presence of eosinophils in histopathology. Cordoba et al described a patient with acute lymphoblastic leukemia who developed GCLD limited to the site of scars of healed herpes zoster. The authors suggested that the hypersensitivity reaction caused by the virus could have played a role in triggering this condition. Other conditions that can present as post-herpetic isotopic response include granuloma annulare, sarcoidosis, granulomatous folliculitis, granulomatous vasculitis, tuberculoid granuloma and nongranulomatous reactions like lichen planus, leukemia cutis, pseudolymphoma and acneiform eruption. However, despite the lesions being dermatomal in distribution in our patient, there was no preceding history suggestive of herpes zoster. The characteristic histologic features of GCLD include hyperkeratosis, acanthosis, exocytosis of lymphocytes, focal basal cell vacuolization, cytoid bodies, band-like mixed inflammatory infiltrate comprising of lymphocytes, histiocytes and few plasma cells in the dermis. The peculiar finding in GCLD is the presence of multinucleated giant cells in the epidermis, at the dermoepidermal junction, in the papillary, reticular dermis or adnexae described in different reports. The histopathologic differential diagnoses include lichen nitidus, deep fungal, mycobacterial infections and secondary syphilis. The lichenoid infiltrate in our case was band-like and not confined to 1 or 2 adjacent dermal papillae, thereby ruling out lichen nitidus, while the remaining possibilities were excluded clinically and on special staining on the biopsy sample. Among the clinical possibilities, lichen planus was ruled out by the presence of multinucleated giant cells in the dermis and absence of hypergranulosis. Lichenoid FIGURE 1 Scaly, erythematous to lichenoid, flat-topped papules measuring 0.5 cm localized on right lower limb Received: 25 October 2016 Revised: 23 April 2017 Accepted: 8 May 2017