Ophthalmic manifestations in patients with collagen vascular disorders: a hospital-based retrospective observational study.

AIM: To study frequency and characteristics of ocular manifestations in Indian patients with collagen vascular disorders. METHODS: The medical records of 73 patients (Males: Females 16:57) aged between 22 and 78 years (mean ± SD = 43.5 ± 12.9 years) with collagen vascular diseases were analyzed retrospectively for demography, subtypes of collagen vascular disease, and findings of complete ophthalmic examination. RESULTS: Lupus erythematosus (LE) in 39(53.4%, (SLE 18, DLE 21), systemic sclerosis in 27(37%), dermatomyositis in 5(6.8%), and primary Sjögren's syndrome in 2(2.7%) patients, respectively, were observed. Only 35(47.9%) patients had ocular manifestations. In LE keratoconjunctivitis sicca (n = 6), keratitis (n = 5), severe blepharitis (n = 3), retinopathy (n = 2), and optic neuritis in one patient, respectively, were major ocular manifestations. Major abnormalities occurring in systemic sclerosis included restricted eyelid mobility of variable severity (n = 8), eyelid telangiectasia (n = 5), keratoconjunctivitis sicca (n = 6), cataract (n = 5), shallow fornices (n = 4), conjunctival surface disease (n = 4), and uveitis, keratitis, episcleritis in one patient each, respectively. One patient with dermatomyositis had heliotrope rash. Two patients with primary Sjögren's syndrome had keratoconjunctivitis sicca. CONCLUSIONS: The study shows that LE frequently presented with keratoconjunctivitis sicca, retinopathy, and optic neuritis. Systemic sclerosis commonly develops eyelid immobility, blepharitis and telangiectasia, ocular surface disease and keratoconjunctivitis sicca, corneal abnormalities, and uveitis. A comprehensive ocular evaluation is imperative for early detection and management particularly of ocular surface disease, uveitis, and retinopathy to prevent potential sight-threatening complications. Limitations include retrospective study design and small number of patients for stratification.

Erythema Dyschromicum Perstans: Response to Topical Tacrolimus.

BACKGROUND: Erythema dyschromicum perstans, a rare dermatosis of obscure etiopathogenesis and significant cosmetic morbidity, have no satisfactory treatment. OBSERVATIONS: Two patients with having characteristic asymptomatic and slowly progressive, slate-grey macular lesions with distinct red borders involving the face, neck, upper trunk and limbs were diagnosed clinicopathologically as erythema dyschromicum perstans. Both were treated successfully with topical tacrolimus 0.1% ointment. CONCLUSIONS: Overall, response to several therapeutic modalities including clofazimine and dapsone therapy is said to vary from complete failure to variable or inconsistent. Topical tarolimus provides an effective and safe alternative therapeutic option in erythema dyschromicum perstans.

Poikiloderma vasculare atrophicans: a distinct clinical entity?

This paper describes a typical case of poikiloderma vasculare atrophicans (PVA) in a 48-year-old female. Histologically, the features were suggestive of PVA with the absence of Pautrier's microabscess or atypical lymphoid cells. The biopsy specimen was positive for cluster of differentiation (CD) 8 on immunohistochemical staining. Its exact pathogenesis remains obscure, and it remains unclear whether PVA actually is mycosis fungoides (MF), a forme fruste of MF, or a distinct and benign dermatosis with CD8+ phenotype that can perhaps be labeled as PVA. However, it has a long benign clinical course without progression to tumor stage of MF in most cases, and its status within the spectrum of cutaneous T-cell lymphoma remains poorly understood. Yet it is imperative to distinguish PVA from poikilodermic MF.

Early treatment with addition of low dose prednisolone to methotrexate improves therapeutic outcome in severe psoriatic arthritis.

Psoriatic arthritis (PsA) is increasingly being recognized to cause progressive joint damage and disability. PsA unresponsive to non-steroidal anti-inflammatory drugs (NSAIDs), the conventional first-line choice of treatment, is usually managed with disease-modifying antirheumatic drugs (DMARDs) especially methotrexate. An 18-year-old HIV-negative male had progressively severe PsA of 4-month duration that was nearly confining him to a wheel chair. He did not respond to multiple NSAIDs, alone or in combination with methotrexate (15 mg/week), given for 4 weeks. Addition of prednisolone (10 mg on alternate days) controlled his symptoms within a week. The NSAIDs could be withdrawn after 4 weeks as the treatment progressed. The doses were tapered for methotrexate (5 mg/week) and prednisolone (2.5 mg on alternate days) every 8 weekly subsequently during 15 months of follow-up without recurrence/deformities or drug toxicity. For years, the use of corticosteroids in psoriasis has been criticized for their propensity to exacerbate the skin disease on withdrawal. However, monitored use of corticosteroids, even in low doses, combined with DMARDs may be a good therapeutic option in early stage of the PsA rather than 'steroid rescue' later. This will help in early control of joint inflammation, prevent joint damage and maintain long-term good functional capacity and quality of life. This may be useful when the cost or availability of biologics precludes their use. However, we discourage the use of corticosteroids as monotherapy.

Metastatic Basal cell carcinoma: a biological continuum of Basal cell carcinoma?

Basal cell carcinoma (BCC) accounts for 80% of all nonmelanoma skin cancers. Its metastasis is extremely rare, ranging between 0.0028 and 0.55 of all BCC cases. The usual metastasis to lymph nodes, lungs, bones, or skin is from the primary tumor situated in the head and neck region in nearly 85% cases. A 69-year-old male developed progressively increasing multiple, fleshy, indurated, and at places pigmented noduloulcerative plaques over back, chest, and left axillary area 4 years after wide surgical excision of a pathologically diagnosed basal cell carcinoma. The recurrence was diagnosed as infiltrative BCC and found metastasizing to skin, soft tissue and muscles, and pretracheal and axillary lymph nodes. Three cycles of chemotherapy comprising intravenous cisplatin (50 mg) and 5-florouracil (5-FU, 750 mg) on 2 consecutive days and repeated at every 21 days were effective. As it remains unclear whether metastatic BCC is itself a separate subset of basal cell carcinoma, we feel that early BCC localized at any site perhaps constitutes a biological continuum that may ultimately manifest with metastasis in some individuals and should be evaluated as such. Long-standing BCC is itself potentially at risk of recurrence/dissemination; it is imperative to diagnose and appropriately treat all BCC lesions at the earliest.

Paraneoplastic pemphigus: a paraneoplastic autoimmune multiorgan syndrome or autoimmune multiorganopathy?

Paraneoplastic pemphigus (PNP), a clinically and immunopathologically distinct mucocutaneous blistering dermatosis, is a severe form of autoimmune multiorgan syndrome generally associated with poor therapeutic outcome and high mortality. This IgG-mediated disease is initiated by an obvious or occult lymphoproliferative disorder in most cases. Clinically severe mucositis, and polymorphic blistering skin eruptions, and histologically acantholysis, keratinocyte necrosis and interface dermatitis are its hallmark features. A 58-year-old female presented with recurrent, severe, recalcitrant stomatitis and widespread erosions/blistering lesions of one-year duration. Treatment with repeated courses of systemic corticosteroids at a peripheral center would provide temporary relief. She also had fever, productive cough, odynophagia and poor oral intake, herpes zoster ophthalmicus, pain in the abdomen, and watery diarrhea. An array of investigations revealed chronic lymphocytic leukemia (CLL), mediastinal and para-aortic lymphadenopathy, bronchiolitis obliterans, and vertebral osteoporosis/fractures. With the diagnosis of CLL-associated PNP she was managed with dexamethasone-cyclophosphamide pulse (DCP) therapy for 3 cycles initially, followed by COP regimen (cyclophosphamide, vincristine, and prednisolone) for 5 cycles. Remission is being maintained with chlorambucil and prednisolone pulse therapy once in 3 weeks with complete resolution of skin lesions and adequate control of CLL.