Recurrent postinfectious scleredema of Buschke in a child: Watchful waiting leads to complete recovery

Dear Editor, A 3-year-old child presented with signs of increasing skin thickness and tightening during the past 6 months. About a year earlier, a similar episode had been observed. Having appeared on both forearms, the changes spread to the neck, chest, and back in a couple of weeks before getting more widespread. A recent upper respiratory infection preceded the onset, and the child continued to experience recurrent respiratory symptoms during the disease. General examination, growth, development, and systemic examination were unremarkable. A cutaneous examination revealed shiny, diffuse, symmetrical, and woody induration of the skin on the forearms, arms, neck, chest, back, trunk, and proximal lower limbs [Figure 1a]. The skin appeared mask-like and unpinchable with microstomia (2.4 cm) [Figure 1b and c]. Differentials such as eosinophilic fasciitis, scleroderma, scleromyxedema, and stiff skin syndrome were taken into consideration. Hepatitis C virus, human immunodeficiency virus, and hepatitis B surface antigen testing came out negative. Leukocytosis and relative lymphocytosis were found in the complete blood count with a raised erythrocyte sedimentation rate. The titer for antistreptolysin-O (ASO) was positive (300 IU/mL) during acute respiratory infection. No organisms were found in the throat swab culture. Serum protein electrophoresis and antinuclear antibody were both normal. Echocardiogram and chest radiograph were normal. Although special stains were not performed, skin biopsy revealed marked thickening and homogenization of dermal collagen bundles with attenuation of adnexal structure [Figure 2a and b]. Initially, pediatricians treated the patient with an antibiotic and three intravenous immunoglobulin doses, resulting in lesion resolution after 3 months. The episode was treated with antibiotics, and after counseling, the parents were reassured about the disease course. Over 6 months, the patient gradually improved, the skin became soft and pinchable again, and the mouth opening increased noticeably [Figure 3a and b]. Overview of management and recovery was described in tabular form [Table 1].Figure 1: (a) Shiny, woody induration of the facial skin, (b) unpinchable, thickened skin over the face, (c) reduced mouth opening (2.4 cm) consistent with microstomiaFigure 2: (a) Low-power photomicrograph of the skin showing marked thickening and homogenization of dermal collagen bundles with attenuation of adnexal structure (H and E, ×10). (b) dermis showing thickened, homogenized collagen bundles with interspersed inflammatory cells (H and E, ×40)Figure 3: (a) Clinical photograph showing restoration of facial skin pinchability following treatment. (b) Increased mouth opening to 2.7 cm, demonstrating improvement in microstomiaTable 1: Summary of treatment and recovery, showing good improvement with both initial therapy and later conservative careScleredema of Buschke is a rare connective tissue disorder of unknown etiology, characterized by dermal mucin deposition and classified as a secondary cutaneous mucinosis.[1] Scleredema affects individuals of all ages, not just adults, as initially defined by Buschke. Its exact frequency and prevalence are unknown, and it exhibits no racial or ethnic predisposition.[1] The most common type of scleredema (55%) is type 1, which typically follows a febrile episode caused by a viral or streptococcal throat infection; type 2 is frequently linked to monoclonal gammopathy, and the third subtype, known as scleredema diabeticorum, is linked to poorly controlled diabetes.[1-4] Scleredema has occasionally been linked to solid-organ malignancies.[2] Although primarily restricted to the skin, there have been sporadic reports of extracutaneous involvement of the tongue, throat, lungs, or heart.[3] The prognosis varies; types II and III are more resistant and may last for years, whereas type I frequently resolves on its own.[4] It is still unclear what causes scleredema. Many stimuli, such as infections and inflammatory processes, drugs, genetic factors, and chronic hyperglycemia in the case of type 3 sclerosis, can cause fibroblasts in the reticular dermis to produce excessive amounts of mucin and collagens.[1,3] Scleroderma, eosinophilic fasciitis, systemic amyloidosis, and other collagen vascular disorders are included in the differential diagnosis of scleredema in children.[4] Histopathology with special stains differentiates scleredema from other mimickers, showing dermal sclerosis with homogenized collagen and interstitial mucin. Antibiotics are the main treatment, but physical therapy is also important for patients who may develop contractures.[3,4] It is important to recognize this as a serious complication of streptococcal infection. In addition, scleredema has been reported in association with hepatitis B infection and increased eosinophil counts.[5] The recurrence of scleredema after immunoglobulin therapy, which resolved spontaneously without immunosuppressive treatment, suggests that the condition is usually benign and self-limiting. It also reminds dermatologists and pediatricians that a watchful waiting approach is often reasonable, as children with woody skin thickening, raised ASO titers, and no other abnormalities may take anywhere from six months to 2 years to recover. Regular handwashing helps reduce the spread of streptococcal infections. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.