We report a rare and interesting case of a combined linear, Blaschkoid and zosteriform pattern of lichen planus pigmentosus. Dermoscopy showed discrete bluish-grey dots, globules, blotches and rods against a brownish background. A skin biopsy confirmed the diagnosis from the presence of civatte bodies, melanin incontinence and band-like inflammation.

BACKGROUND Our aim was to study the clinical and immunological profile of patients with newly detected connective tissue disease presented to a tertiary care centre. MATERIALS AND METHODS The study involved 51 patients with newly-detected Systemic Lupus Erythematosus (SLE) (fulfilling the revise SLICC criteria for SLE) and Systemic Sclerosis (SS), Mixed Connective Tissue Disease (MCTD), etc. attending Sir. T. Hospital, Bhavnagar, between January 2013 and December 2016. All patients were assessed for clinical features and immunological profile. RESULTS Out of the 51 patients, 30 having SLE, 10 having SS, 9 with MCTD, 1 with dermatomyositis and 1 with Rowell's syndrome. Among them, 47 were females and 4 were males. The mean age at presentation was between 15-25 years. The LE-specific skin lesions were noted as malar rash in 25 patients (83%), subacute and acute lupus rashes (80%) and discoid rash (13%). Among LE-nonspecific lesions, non-scarring alopecia was most common followed by oral ulcers, Raynaud's phenomenon, joint pain, scarring alopecia, erythema multiforme, livedo reticularis, vasculitic lesions, urticaria and calcinosis cutis were seen. In MCTD, muscle weakness was common finding. In systemic sclerosis, hide-bound skin and decreases mouth opening were seen in all cases and Raynaud's phenomenon, joint pain, hair loss, calcinosis cutis and respiratory system involvement were other features. Serum ANA was positive in 76% while negative in 3.8% of individuals. The most common pattern observed in ANA profile was speckled (56%) followed by homogenous (32%) and nucleolar (28%). CONCLUSION There is diversity in clinical presentation of autoimmune connective tissue disease with regards to their genetic and environmental backgrounds. Cutaneous features are utmost important having diagnostic and prognostic value as well.

Sturge–Weber syndrome (SWS) is an uncommon, nonhereditary developmental condition characterized by nevus flammeus and ocular and neurological manifestations. Childhood-onset systemic lupus erythematosus (cSLE) is a rare disease with a female preponderance and is associated with multiple complications and a poor prognosis. The coexistence of cSLE and SWS is uncommon and can be associated with an increased risk of thromboembolic and neuropsychiatric complications. Macrophage activation syndrome (MAS) is one of the fatal complications of cSLE and an early diagnosis based on clinical findings and laboratory parameters is vital. We report a rare and unique case of SWS with cSLE in an 11-year-old boy, which was complicated by MAS.