Cutaneous mastocytosis. Getting beneath the skin of the issue: a case report

An eleven month old girl presented with chronic urticaria since three months of age. There was a generalised hyperpigmented maculo-papular rash. Darier sign was positive. The skin biopsy showed plenty of spindle shaped mast cells with eosinophilic cytoplasm infiltrating the dermis and the appendiceal structures. The diagnosis of cutaneous mastocytosis (urticaria pigmentosa) was made. The child received symptomatic relief with chronic oral hydroxyzine and ranitidine therapy. Automated epinephrine self-injectors usually prescribed in this condition for self-management of anaphylactic episodes were not available. Intramuscular administration of (1:1000) diluted adrenaline via a disposable tuberculin syringe was taught to the mother. A medical bracelet containing her diagnosis and instructions in emergency was custom-made for her

Left ventricular noncompaction: A cardiomyopathy often mistaken

Left ventricular noncompaction (LVNC) is a rare genetic cardiomyopathy postulated to be a defect in endomyocardial morphogenesis due to the embryonic arrest of the compaction of myocardial fibers. It could be isolated, without other structural heart defects; or associated with congenital heart defects. It is characterized by prominent ventricular myocardial trabeculations and deep intertrabecular recesses. The clinical manifestations, i.e., heart failure, arrhythmias or thromboembolism, overlap with those of other cardiac disorders. It is often misdiagnosed as restrictive or dilated cardiomyopathy. The high mortality and morbidity associated with it and familial occurrence make diagnosis important. Only 3 pediatric cases have been reported from India. We present 2 cases, that of an 11-year-old girl (familial case) with embolism (documented but rare in children) and atrial flutter (not yet reported), with mother having asymptomatic LVNC; and that of a 4-month-old girl. Both presented with heart failure. The 11-year-old child had sudden death, known to occur in LVNC

Local anaesthetic systemic toxicity following oral ingestion in a child: Revisiting dibucaine

Dibucaine, a potent and toxic local anaesthetic, although currently withdrawn by the United States Food and Drug Administration for use as a spinal anaesthetic, continues to remain available in many over-the-counter topical formulations. Systemic toxicity following oral ingestion of local anaesthetics is rare. We report a case of accidental ingestion of dibucaine (ear drops) in a 7-year-old child who developed diplopia, giddiness, ventricular premature contractions and a right bundle branch block. We also present a brief discussion on the pharmacologic and toxicity profile of dibucaine, the Naranjo algorithm for assessing causality in case of adverse drug reactions and a review of current guidelines on the management of local anaesthetic systemic toxicity

Cutaneous polyarteritis nodosa: Diagnosis lies skin deep

Cutaneous polyarteritis nodosa (CPAN) is an uncommon form of cutaneous vasculitis. Definitive diagnosis is based on skin biopsy. We present a rare case of CPAN in a 2-year-old male child who presented with a history of fever, cutaneous ulcers, and digital gangrene. He was treated with steroids and skin lesions had resolved completely. CPAN needs to be differentiated from systemic polyarteritis nodosa for specific prognosis and management. We report this case to highlight the need of early diagnosis for faster recovery with favorable outcome

A longitudinal study of antibody responses to selected host antigens in rheumatic fever and rheumatic heart disease

Introduction. Group A streptococci can trigger autoimmune responses that lead to acute rheumatic fever (ARF) and rheumatic heart disease (RHD).Gap Statement. Some autoantibodies generated in ARF/RHD target antigens in the S2 subfragment region of cardiac myosin. However, little is known about the kinetics of these antibodies during the disease process.Aim. To determine the antibody responses over time in patients and healthy controls against host tissue proteins - cardiac myosin and peptides from its S2 subfragment, tropomyosin, laminin and keratin.Methodology. We used enzyme-linked immunosorbent assays (ELISA) to determine antibody responses in: (1) healthy controls; (2) patients with streptococcal pharyngitis; (3) patients with ARF with carditis and (4) patients with RHD on penicillin prophylaxis.Results. We observed significantly higher antibody responses against extracellular proteins - laminin and keratin in pharyngitis group, patients with ARF and patients with RHD when compared to healthy controls. The antibody responses against intracellular proteins - cardiac myosin and tropomyosin were elevated only in the group of patients with ARF with active carditis. While the reactivity to S2 peptides S2-1-3, 8-11, 14, 16-18, 21-22 and 32 was higher in patients with ARF, the reactivity in the RHD group was high only against S2-1, 9, 11, 12 when compared to healthy controls. The reactivity against S2 peptides reduced as the disease condition stabilized in the ARF group whereas the reactivity remained unaltered in the RHD group. By contrast antibodies against laminin and keratin persisted in patients with RHD.Conclusion. Our findings of antibody responses against host proteins support the multistep hypothesis in the development of rheumatic carditis. The differential kinetics of serum antibody responses against S2 peptides may have potential use as markers of ongoing cardiac damage that can be used to monitor patients with ARF/RHD