ABSTRACT
We present a case of cutaneous granulomatous disease associated with rubella virus in a 4-year-old girl without an identifiable immunodeficiency. In this case, a combination of anti-inflammatory, anti-viral, and anti-neutrophil therapies successfully treated vision-threatening eyelid, conjunctival, scleral, and orbital inflammation.
Subject(s)
Immunologic Deficiency Syndromes , Skin Diseases , Female , Humans , Child, Preschool , Rubella virus , Granuloma/drug therapy , Skin Diseases/complications , Eyelids , Inflammation/complicationsABSTRACT
Multicentric reticulohistiocytosis (MRH) is a rare systemic inflammatory granulomatous disease marked by severe and often rapidly progressive polyarticular arthritis and cutaneous papulonodules. Initial clinical diagnosis may be difficult. We describe a 2-year-old girl presenting with pink dermal papules on the forehead, thighs, elbows, knees, and palms of the hands. Based on clinical findings and skin biopsy results, she was initially diagnosed with granuloma annulare. At 5 years of age, she developed arthritis, fatigue, and more widespread skin papules leading to the diagnosis of MRH. To our knowledge, this is the youngest individual with MRH yet described. We outline the timeline and unique features of her case and review the literature pertaining to MRH in children. Although rare, MRH can be permanently debilitating, making prompt diagnosis critical. A standardized approach to investigation and management needs to be developed.
Subject(s)
Histiocytosis, Non-Langerhans-Cell/diagnosis , Biopsy , Dermatologic Agents/therapeutic use , Diagnosis, Differential , Female , Histiocytosis, Non-Langerhans-Cell/drug therapy , Humans , Infant , Infliximab/therapeutic useABSTRACT
Primary care physicians should have a working knowledge of rheumatic diseases of childhood that manifest primarily as musculoskeletal pain. Children with juvenile rheumatoid arthritis can present with painless joint inflammation and may have normal results on rheumatologic tests. Significant morbidity may result from associated painless uveitis, and children with juvenile rheumatoid arthritis should be screened by an ophthalmologist. The spondyloarthropathies (including juvenile ankylosing spondylitis and reactive arthritis) often cause enthesitis, and patients typically have positive results on a human leukocyte antigen B27 test and negative results on an antinuclear antibody test. Patients with acute rheumatic fever present with migratory arthritis two to three weeks after having untreated group A beta-hemolytic streptococcal pharyngitis. Henoch-Schbnlein purpura may manifest as arthritis before the classic purpuric rash appears. Systemic lupus erythematosus is rare in childhood but may cause significant morbidity and mortality if not treated early. Nonsteroidal anti-inflammatory drugs and physical therapy may be useful early interventions if a rheumatic illness is suspected. Family physicians should refer children when the diagnosis is in question or subspecialty treatment is required. Part I of this series discusses an approach to diagnosis with judicious use of laboratory and radiologic testing.
Subject(s)
Pain/etiology , Rheumatic Diseases/complications , Child , Chronic Disease , Diagnosis, Differential , Humans , Pain/diagnosis , Pain Measurement , Rheumatic Diseases/diagnosis , Severity of Illness IndexABSTRACT
Musculoskeletal pain can be difficult for children to characterize. Primary care physicians must determine whether the pain may be caused by a systemic disease. Change in activity, constitutional symptoms such as fevers and fatigue, or abnormal examination findings without obvious etiology should raise suspicion for rheumatic disease. A complete physical examination should be performed to look for extra-articular signs of rheumatic disease, focusing on but not limited to the affected areas. A logical and consistent approach to diagnosis is recommended, with judicious use of laboratory and radiologic testing. Complete blood count and erythrocyte sedimentation rate measurement are useful if rheumatic disease is suspected. Other rheumatologic tests (e.g., antinuclear antibody) have a low pretest probability in the primary care setting and must be interpreted cautiously. Plain radiography can exclude fractures or malignancy; computed tomography and magnetic resonance imaging are more sensitive in detecting joint inflammation. Family physicians should refer children to a subspecialist when the diagnosis is in question or subspecialty treatment is required. Part II of this series discusses rheumatic diseases that present primarily with musculoskeletal pain in children, including juvenile arthritis, the spondyloarthropathies, acute rheumatic fever, Henoch-Schönlein purpura, and systemic lupus erythematosus.
