|Year : 2021 | Volume
| Issue : 3 | Page : 254-256
Acquired cutis laxa secondary to sweet syndrome (marshall syndrome) in a 2-year-old child
Gowrappala Shanmukhappa Asha, Seetharampura Ramamurthy Radhika, Kanathur Shilpa, Heera Ramesh
Department of Dermatology, Venereology, Leprosy, Bangalore Medical College and Research Institute, Bengaluru, Karnataka, India
|Date of Submission||22-May-2020|
|Date of Decision||04-Jun-2020|
|Date of Acceptance||30-Mar-2021|
|Date of Web Publication||30-Jun-2021|
Seetharampura Ramamurthy Radhika
No. 12, 1st Cross, Sri Krupa Layout, Abbigere, Chikkabanavara Post, Bengaluru - 560 090, Karnataka
Source of Support: None, Conflict of Interest: None
Sweet syndrome (SS) is a neutrophilic dermatosis which is rarely seen in early childhood. Usually, in children, the SS is predominantly of classic/idiopathic type. In 40%–45% of cases, it is secondary to infections. Acquired cutis laxa (ACL) Type II also known as Marshall syndrome is also a very rare cutaneous condition seen in children. Only ten cases have been reported till now. We report this case of a 2-year-old girl with recurrent SS and ACL Type II, who was treated initially with tapering doses of steroids and saturated solution of potassium iodide for maintenance.
Keywords: Acquired cutis laxa, marshall syndrome, saturated solution of potassium iodide, Sweet syndrome
|How to cite this article:|
Asha GS, Radhika SR, Shilpa K, Ramesh H. Acquired cutis laxa secondary to sweet syndrome (marshall syndrome) in a 2-year-old child. Indian J Paediatr Dermatol 2021;22:254-6
|How to cite this URL:|
Asha GS, Radhika SR, Shilpa K, Ramesh H. Acquired cutis laxa secondary to sweet syndrome (marshall syndrome) in a 2-year-old child. Indian J Paediatr Dermatol [serial online] 2021 [cited 2021 Oct 22];22:254-6. Available from: https://www.ijpd.in/text.asp?2021/22/3/254/319954
| Introduction|| |
Sweet's syndrome (SS) (the eponym for acute febrile neutrophilic dermatosis) is characterized by a constellation of clinical symptoms, physical features, and pathologic findings which include fever, neutrophilia, tender erythematous skin lesions (papules, nodules, and plaques), and a diffuse infiltrate consisting predominantly of mature neutrophils that are typically located in the upper dermis.
Cutis laxa (CL) is a rare cutaneous disorder characterized clinically by loose, wrinkled, pendulous, lax skin, and histologically by fragmentation and loss of dermal elastic tissue. It can either be inherited or acquired. Acquired CL (ACL) is further subdivided into Type I and II. Type II, also known as postinflammatory elastolysis and CL or Marshall syndrome More Details, is characterized by only cutaneous changes without any systemic involvement. Here, we report a case of a 2-year-old child with Marshall Syndrome secondary to SS.
| Case Report|| |
A 2-year-old female child was brought with complaints of multiple painful red-colored rashes on face and extremities, associated with fever for 1 month.
On examination, there were multiple, well defined, tender, erythematous, edematous papules and plaques, and central crusting present over few plaques with the largest measuring 5 cm × 5 cm and the smallest measuring 0.5 cm × 0.5 cm in size, present predominately over the face, trunk, and extremities [Figure 1].
|Figure 1: Multiple, well-defined, erythematous, edematous papules and plaques with central crusting|
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General physical examination and systemic examination were within normal limits. There was a history of cough, rhinitis preceding the rash, and fever, but there was no history of diarrhea, pain abdomen, loss of weight and appetite, night sweats, or bleeding tendencies or any drug intake before rash. SS, Erythema multiforme, and urticarial vasculitis were the differential diagnosis that was thought of.
Complete hemogram showed red blood cell count of 4.53 cells/cu mm, total count of 5600 cells/cu mm (neutrophils – 52%), and platelet count of 6.06 cells/cu mm. Erythrocyte sedimentation rate was 42 mm/h and C-reactive protein was positive. Ultrasound abdomen was normal, two-dimensional echocardiography and color Doppler showed normal study. Chest X-ray was normal, and antistreptolysin O titer was negative. A biopsy taken from the erythematous plaque revealed normal epidermis with extensive neutrophilic infiltrate in superficial dermis with evidence of necrobiosis and inflammatory infiltrate [Figure 2].
|Figure 2: Hematoxylin and Eosin stain (×40) – epidermis shows psoriasiform acanthosis, neutrophilic spongiosis, and dermis shows dense interstitisal neutrophilic infiltrate|
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Our patient fulfilled both the major and 3 of the 4 minor diagnostic criteria for SS; thus, a diagnosis of the same was made, and upper respiratory tract infection was thought to be the cause in our case.
The child was started with tapering dose of oral prednisolone (5mg/5ml) and syrup paracetamol according to the body weight. After 15 days of treatment, the lesions showed good response, but the child presented with loosening facial skin at the site of the previous lesions. On examination, there was sagging and wrinkling of the skin [Figure 3]. A biopsy was taken from these lesions which revealed loss of elastic fibers in papillary, upper, and midreticular dermis associated with focal dermal giant cell reaction [Figure 4]. Verhoeff-Van Geison stain revealed reduced elastic fibers in upper and mid dermis. Based on the above findings, a diagnosis of CL was made.
|Figure 4: Hematoxylin and eosin stain (×40) - epidermis normal and dermis shows sparse lymphocytic infiltrate around superficial blood vessels|
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The child started developing recurrence of SS lesions, after being asymptomatic for a year. Due to the recurrent SS, she was evaluated with alpha 1 antitrypsin enzyme levels – 2.04 g/L, no deficiency was found (normal range being – 0.9–2 g/L). The child was initially started on tapering doses of steroids – prednisolone (1 mg/kg/day) and saturated solution of potassium iodide 0.5 g/day was given for maintenance and was followed up on a biweekly basis. The child tolerated the treatment well; the lesion resolved in 1 month and later was treated with tapering doses of Saturated Solution of Potassium Iodide (SSKI) [Figure 5]. Child is being followed for 3 months with no recurrence.
|Figure 5: One month after treatment with SSKI, lesions show good improvement and appreciate the cutis laxa features|
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| Discussion|| |
SS was first described by Dr. Robert Douglas Sweet, originally known as Gomm–Button disease (in reference to the first two patients). SS is an inflammatory skin disorder characterized by the extensive infiltration of neutrophils into the epidermis and dermis. SS can present as one of three clinical types: classical (or idiopathic) SS, malignancy-associated SS, or drug-induced SS. Pediatric cases account for approximately 5% of all cases.
The underlining biological pathways responsible for this cutaneous neutrophilic dermatosis have remained elusive. However, the association of this disease with infection, autoimmune diseases, neoplasms, and drugs suggests an unusual hypersensitivity that may be mediated by cytokines, followed by infiltration of neutrophils that are probably activated by interleukin-1.
Malignancies that are associated with SS are usually hemoproliferative disorders or solid tumors and account for only 20% of the cases. Relapses are noted most commonly in the malignancy associated rather than the idiopathic variety (70% vs. 30%). Other associated conditions include infections, inflammatory bowel disease, rheumatologic diseases, pregnancy, primary immunodeficiency, and drug- or vaccine-induced conditions.
CL is characterized by abnormal elastic fibers, resulting in loose, redundant, hypoelastic skin. ACL is a rare disorder and is insidious in onset. ACL often starts on the face and progresses caudally. Some patients have systemic involvement with emphysema, intestinal diverticula, hernias, and vascular dilatations.
Here, we report a rare case of SS in a 2-year-old child where she later progressed to develop Marshalls Syndrome secondary to the inflammation. Marshall Syndrome is a type of ACL affecting young children and characterized by postinflammatory elastolysis after a neutrophilic dermatosis giving a premature aged look. Neutrophils attached to elastic fibers release elastase, which acts on the amorphous component of elastic fibers, resulting in alteration or destruction of elastic tissue. Dysfunction in elastase inhibitors with low serum levels of copper leads to low lysyl oxidase activity and thus failure of the cross-linkage and elastolysis. Low levels of alpha-1 antitrypsin which prevents proteolysis of pulmonary tissue have been reported with Marshall's syndrome. Histologically, elastic fibers are reduced in ACL, especially in the papillary dermis. Marshall's syndrome is managed symptomatically. Dapsone and prednisolone have been tried. Plastic surgery, face uplift, and botulinum toxin injection can be used.
| Conclusion|| |
Thus, we report this case of a 2-year-old child having ACL secondary to SS (Marshall Syndrome) as it is a very rare case, and only ten cases have been reported in the literature till date. CL secondary to SS in a kid should be thoroughly investigated for malignancy and recurrent infections. Hence, screening for cardiac, respiratory, and gastrointestinal involvement in all ACL cases should be done. Furthermore, the importance of saturated solution of potassium iodide in the maintenance of recalcitrant cases is emphasized here.
Informed consent was taken for clinical images from the patient's mother.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient's mother has given her consent for images and other clinical information to be reported in the journal. The patient's mother understands that name and initial will not be published, and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]