Recurrent Sweet’s Syndrome Leading to Secondary Cutis Laxa in a Child: A Rare Case of Marshall’s Syndrome

Dear Editor, Marshall’s syndrome, also known as postinflammatory elastolysis and cutis laxa (PECL), is a rare acquired disorder of elastic tissue, predominantly seen in children. It begins with an inflammatory phase that leads to the destruction of elastic fibers, causing skin atrophy, wrinkling, and laxity. Cutis laxa refers to a broader group of connective tissue disorders marked by loose, sagging skin due to elastic fiber abnormalities. Its etiology is unclear, and treatment remains nonspecific. Here, we describe a case of a 3-year-old child with classic features of Marshall’s syndrome. A 3-year-old boy presented to the dermatology outpatient department with erythematous, edematous lesions on the face and left upper extremity present for 14 days. The child had a mild fever. There was history of similar episode a year ago on the forearms, which resolved with localized atrophy and laxity. There was no drug history. On examination, multiple well-demarcated erythematous plaques with annular and polycyclic configurations were seen on the face, with scaling along inner edge of the lesions [Figure 1a and 1b]. No systemic signs were noted. The forearms, previously affected, exhibited localized skin laxity and atrophy [Figure 1c].Figure 1: (a and b) Multiple well-circumscribed erythematous and edematous plaques with superficial collarettes of scaling on the right and left sides of the face. (c) Localized wrinkling and laxity of skin at the site of the previous lesionLaboratory tests, including complete blood count, liver function test, triple serology and ANA profile, were within normal limits. Skin biopsies were taken from an active lesion and the previously healed area with lax skin. The acute lesion showed mild epidermal spongiosis and dense neutrophilic infiltration in the upper and mid-dermis, with lymphocytes, histiocytes, and karyorrhectic debris [Figure 2a, 2b, and 2c]. Verhoeff-Van Gieson (VVG) staining of the biopsy from the lax skin revealed reduced elastic fibers in the upper dermis [Figure 2d], confirming Marshall’s syndrome. Alpha-1 antitrypsin levels were not assessed. No evidence of malignancy or underlying systemic disease was found on thorough evaluation for potential causes of Sweet’s syndrome. The likely trigger identified was a preceding upper respiratory tract infection. The child was treated with oral dapsone (2 mg/kg) for 4 weeks, leading to complete resolution [Figure 3a and 3b].Figure 2: (a) Spongiotic epidermis and dermal neutrophilic infiltrate (Hematoxylin and eosion, 100×). (b) interstitial presence of neutrophils, lymphocytes, and histiocytes (Hematoxylin and eosion, 400×). (c) dense infiltrate of neutrophils along with karyorrhexis (Hematoxylin and eosion, 400×). (d) Verhoeff-Van Gieson stain showing reduced elastin fibers in the upper dermisFigure 3: (a and b) Resolving skin lesions post-treatment with dapsoneMarshall’s syndrome was first described in 1966 in South African children with erythematous papular eruptions followed by cutis laxa.[1] Cutis laxa is a connective tissue disorder characterized by inelastic skin due to abnormalities in elastin and other extracellular matrix components. It may be inherited or acquired. Acquired cutis laxa is classified into Type 1 (generalized acquired elastolysis, usually affecting adults with possible systemic involvement) and Type 2 (Marshall’s syndrome), which occurs in children, typically following inflammation and without systemic features.[2] The pathogenesis of acquired cutis laxa Type 2 remains poorly understood. It is hypothesized that elastase released from neutrophils and macrophages degrades elastic fibers. A deficiency in elastase inhibitors such as alpha-1 antitrypsin may also play a role. Copper metabolism abnormalities have also been suggested.[3-5] Marshall’s syndrome typically affects children aged 1–5 years and commonly involves the face and neck, although other areas may also be affected. It has a relapsing, self-limiting course, often with no family history. In our case, the child first developed lesions at the age of two years that resolved spontaneously with residual laxity, and experienced another episode a year later. Differential diagnoses considered were erythema annulare centrifugum, granuloma annulare, and neutrophilic dermatoses. Histopathology supported a diagnosis of Sweet’s syndrome during the acute phase due to neutrophilic infiltration and this ruled out other conditions. For the atrophic area, differential diagnoses included anetoderma and mid-dermal elastolysis. However, anetoderma typically presents with herniated papules, not seen in our case. Mid-dermal elastolysis affects the mid-reticular dermis and usually spares the face, making it less likely. This case not only broadens the clinical spectrum of acquired cutis laxa in children but also emphasizes the importance of recognizing Sweet’s syndrome as a potential precursor to Marshall’s syndrome, even in the absence of systemic involvement or alpha-1 antitrypsin testing. Hence further research is needed for understanding its pathogenesis which will help in managing and anticipating recurrences. A limitation of our case was the inability to assess alpha-1 antitrypsin levels, which may have contributed to the pathogenesis. This was due to resource constraints. Authors’ contributions We confirm that all authors have read and approved the manuscript for submission. All authors meet authorship criteria, and the manuscript represents honest work. Declaration of patient consent The authors certify that they have obtained all appropriate parent consent forms. In the form a parent has given their consent for her images and other clinical information to be reported in the journal. The parent understands that her name and initials will not be published and due efforts will be made to conceal her identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest. Use of artificial intelligence (AI) The preparation of this manuscript was carried out entirely by the authors without the use of artificial intelligence technologies.