Scleredema-an uncommon cause of swelling in a child-a case report and review of the literature
© Shrestha et al.; licensee BioMed Central Ltd. 2014
Received: 13 August 2014
Accepted: 22 August 2014
Published: 27 August 2014
Body swelling in a child is a common symptom. Apart from systemic causes like renal, hepatic, and cardiac, rarely such a swelling may be caused by dermatologic conditions.
A child presented with swelling of the body which was subsequently diagnosed as scleredema, a rare and benign dermatologic condition. Scleredema can be confused with similar sounding terms like scleroderma and scleromyxedema.
The case is presented to highlight scleredema as a rare cause of body swelling in paediatrics and to differentiate it from similar sounding rare terms like scleroderma and scleromyxedema.
KeywordsBuschke Scleroderma Scleromyxedema Systemic sclerosis
Swelling of the body is a common problem in paediatric practice. It is generally a manifestation of some underlying systemic disorders which may be renal, hepatic, cardiac, endocrinal, metabolic, anaphylaxis, nutritional and even pulmonary conditions. Rarely, connective tissue disorders and even dermatological causes can lead to body swelling. The investigations for the cause of swelling need to be according to the suspected disorder for prompt diagnosis and successful management. We report a case of body swelling in a child with scleredema. Besides being rare, scleredema can also be confused with diseases having similar terminologies, especially scleroderma and scleromyxedema. This case is reported to familiarize the general paediatrician with the rare entity of scleredema and its differentials, all of which can be among rarer causes of swelling in children.
Scleredema is an uncommon skin disorder which is characterized by non-suppurative, oedematous thickening of the skin, especially the face, neck, shoulders, thorax, proximal extremities but not the fingers . The disease was discovered by Curizo in 1752. Later, Abraham Buschke described the first classical features in an adult patient in 1902 and named it scleredema adultorum to distinguish it from scleredema neonatorum . Subsequently, the disease has been known as scleredema or scleredema of Buschke or scleredema adultorum . The exact incidence, cause and the pathogenesis of the disease are unknown. In 1968, Graff classified the disease into three types-types I, II and III. Type I is the commonest form, and it is especially found in the paediatric population and includes more than 55% of the cases. It typically follows a febrile illness, usually streptococcal throat infection or viral illnesses. This subtype is more common in females and is found in all ages and races. Most cases are below 20 years of age [4, 5]. Type II constitutes around 25% of all cases and is generally progressive and associated with monoclonal gammopathy and multiple myeloma . Type III, also referred to as scleredema diabetocorum or diabetic scleredema, consists around 20% of cases and is a known, rare complication of uncontrolled diabetes mellitus [4, 5]. It is known to occur in 2.5% to 14% of all diabetic cases and found predominantly in men . Scleredema Type I characteristically present with induration of the skin, especially over the face, neck, shoulder girdles and upper extremities but spares the fingers. The mobility of the joints may be reduced. There is absence of Raynaud’s phenomenon which is typically present in scleroderma. In scleredema, there is no organic involvement. The disease is generally self-limiting with active phase being 6–8 weeks and complete resolution within 6 months to 2 years. Occasionally, the disease may be progressive and fatalities have been noted [7, 8]. Rarely, there is involvement of organs like eyes, tongue, oesophagus and the disease may lead to pleural or pericardial or peritoneal effusions . Our patient fitted in type I scleredema, although there was no preceding history of any infection. The treatment modalities for type II and type III is non specific. Various agents that have been tried for the treatment include steroids, methotrexate, cyclosporine, radiotherapy, prostaglandin E1, factor XIII, systemic photochemotherapy with psoralene and ultraviolet A (PUVA), high dose penicillin, d-penicillamine etc [4, 8].
Scleredema is commonly confused with conditions like scleroderma and scleromyxedema. Histologically, scleredema is distinguished by its normal epidermis and thickened dermis, full of swollen collagen fibers and mucin containing clear spaces. Scleroderma is a major cutaneous feature of systemic sclerosis and is typically characterized by pigmented, thickened skin with bilateral, symmetrical involvement and centripetal progression. Histopathologic study shows thin epidermis and thickened dermis with excessive collagen [4, 5, 7, 9]. Scleromyxedema is a papular, waxy, diffuse involvement of the skin of fingers, face, neck and arms. It may be associated with monoclonal gammopathy or multiple myeloma. It is characterized histologically by excessive dermal mucin deposition, fibroblastic proliferation and fibrosis in the absence of thyroid disease [9, 10].
Although rare entities in themselves, miscellaneous skin conditions like scleredema, scleroderma and even scleromyxedema can be the cause of swelling in paediatric population and the primary care paediatrician must be well aware of them so that appropriate investigations can be performed and correct diagnoses made. This is important because in spite of the similarities in terminologies and presentation, the management approach for each of them is quite different.
The case has been highlighted to familiarize the general paediatric practitioner with an uncommon dermatologic cause of body swelling in children. Despite its rarity and relatively benign nature of scleredema and its diagnostic dilemma with similar conditions like scleroderma and scleromyxedema, with careful history, examination and investigations, the diagnosis can be made and treated accordingly.
Written informed consent was taken from the parents for the publication of the case report, along with the accompanying photographs. A copy of the written consent is available for review by the editorial board of the journal.
The authors declare that they have no financial relationship related to this article to disclose.
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