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CASE REPORT
Year : 2016  |  Volume : 17  |  Issue : 2  |  Page : 135-138

Sweet's syndrome leading to acquired cutis laxa in a child (Marshall's syndrome): A rare case report


1 Department of Skin and STD, Mysore Medical College and Research Institute, Mysore, Karnataka, India
2 Department of Pathology, JSS Medical College, Mysore, Karnataka, India

Date of Web Publication30-Mar-2016

Correspondence Address:
H Bangaru
Department of Skin and STD, #14, OPD Block, K. R. Hospital, Mysore Medical College and Research Institute, Mysore - 570 001, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2319-7250.172467

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  Abstract 

Sweet's syndrome is very rare in children, fewer than 80 cases are reported in literature. The lesions usually resolve either spontaneously or after treatment without scarring. Marshall's syndrome is characterised by acute inflammatory skin lesions with subsequent development of localised elastolysis without any internal organ involvement. It is an extremely rare condition in children. There are very few cases reported as Sweet's syndrome leading to acquired cutis laxa - Marshall's syndrome. Sweet's syndrome shows excellent response to systemic steroids, whereas there is no satisfactory treatment for Cutis laxa.

Keywords: Cutis laxa, malignancy, Marshall's syndrome, Sweet's syndrome


How to cite this article:
Bangaru H, Surendran K, Nanjundaswamy B L, Vijaya B. Sweet's syndrome leading to acquired cutis laxa in a child (Marshall's syndrome): A rare case report. Indian J Paediatr Dermatol 2016;17:135-8

How to cite this URL:
Bangaru H, Surendran K, Nanjundaswamy B L, Vijaya B. Sweet's syndrome leading to acquired cutis laxa in a child (Marshall's syndrome): A rare case report. Indian J Paediatr Dermatol [serial online] 2016 [cited 2019 Nov 14];17:135-8. Available from: http://www.ijpd.in/text.asp?2016/17/2/135/172467


  Introduction Top


Sweet's syndrome is characterized by painful erythematous plaques on the skin associated with fever, leukocytosis, and neutrophilia.[1] It is very rare in children, fewer than 80 cases are reported in the literature.[2]

Marshall's syndrome is also rare pediatric skin disease characterized by acquired, localized, neutrophilic dermatosis (Sweet's disease) followed by loss of elastic tissue in the dermis and cutis laxa.[3]

Sweet's syndrome shows a rapid and excellent response to systemic steroids, so the progression of Sweet's syndrome and development of new cutis laxa lesions can be halted.


  Case Report Top


A 4-year-old boy presented with a history of red raised painful skin lesions on the abdomen, chest, and extremities since two months associated with fever.

The lesions started as erythematous papules expanded peripherally, forming large round plaque with prominent raised margin. Initially seen over the abdomen and rapidly progressed to involve arm, back, and gluteal area over a period of 2 weeks.

Past history of similar skin eruptions 1-year back over the forehead, chest, and extremities followed by loose, wrinkled patches in the affected sites.

Cutaneous examination revealed, multiple well-circumscribed, erythematous, edematous, and infiltrated plaques with raised margin. Few lesions showed an illusion of vesiculations. The majority of lesions coalesced to form large polycyclic configuration, distributed over the abdomen, gluteal area, and extremities [Figure 1].
Figure 1: Annular erythematous, infiltrated plaques with pseudo vesicle appearance at periphery with central clearing

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He appeared older than his age with loose, pendulous and wrinkled skin over the forehead, cheeks, around the mouth, chest, and other affected sites [Figure 2]a and [Figure 2]b.
Figure 2: (a) Premature aged look with wrinkles around mouth, forehead (b) wrinkled atrophic plaque over the chest

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Laboratory investigations revealed hemoglobin − 10.6 g/dl, leukocytosis (18,300/dl), neutrophilia (N-71%, L-24%, E-3%, M-2%, and B-0.), erythrocyte sedimentation rate was high (40 mm/h), C-reactive protein was negative, and alkaline phosphatase level normal (125 U/L). Serum α1-antitrypsin level was normal 2.14 g/L (0.9–2.0 g/L).

Blood venereal disease research laboratory and human immunodeficiency viruses were negative. Ultrasound abdomen was normal, two-dimensional echocardiography and color Doppler showed normal study. Computed tomography thorax was normal.

Histopathology from wrinkled patch showed unremarkable epidermis, a mild degree of perivascular lymphocytic infiltration, Verhoeff-Von Gieson stain showed a marked reduction in elastic fibres [Figure 3]a – features were consistent with cutis laxa.
Figure 3: (a) Verhoeff-Van Gieson - Microphotograph showing a marked reduction in elastic fibres. A few fragmented ones noted (Verhoeff-Van Gieson stain, ×40) (b) microphotograph showing diffuse infiltration of the dermis by neutrophilic infiltrate. Karyorrhectic debris noted (H and E, ×40)

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The section from infiltrated plaque showed mild acanthosis and mild edema, dilated capillaries with plump endothelial cells and diffuse infiltration by neutrophils and few lymphocytes and karyorrhectic debris seen [Figure 3]b – suggestive of Sweet's syndrome.

Based on the above clinical findings and laboratory investigations, the diagnosis of acquired cutis laxa type 2 following Sweet's syndrome (Marshall's syndrome) was made.

The patient was started on oral prednisolone 1 mg/kg, the dramatic response was observed in 1-week with rapid clearance of almost all the lesions leaving hyperpigmented atrophic patches over the affected sites. Prednisolone was tapered over a period of 8 weeks without the reappearance of lesions.


  Discussion Top


Sweet's syndrome was first described by Sweet in 1964.[1] Sweet's syndrome is rarely seen in the pediatric population, <80 pediatric cases have been reported in the literature.[2] Fever is the most frequent symptom, skin lesions are typically tender, painful, red or purple-red papules, nodules or plaques seen frequently on upper extremities, face, and neck. The lesions have a transparent vesicle like appearance (pseudo vesicle) because of the pronounced edema in the upper dermis. The central clearing may lead to the annular or arcuate pattern in later stages. The individual lesion may enlarge to form irregular, sharply border plaques. The lesions usually resolve either spontaneously or after treatment without scarring. In two third of patients, it may recur.[3]

One of the major diagnostic criteria for Sweet's syndrome is a diffuse infiltrate of mature neutrophils and edema in the upper dermis. Fragmented neutrophil nuclei (karyorrhexis), swollen endothelial cells and dilated small blood vessels may be present. The primary leukocytoclastic vasculitis is absent.[3]

Sweet's syndrome can present in several settings; classical, malignancy associated, and drug induced. Sweet's syndrome is often idiopathic. A bacterial infection of upper respiratory tract infection, tonsillitis, or intestinal infection may precede skin lesions by 1–3 weeks. The common malignancies associated with Sweet's syndrome are acute myelogenous leukaemia, carcinoma of genitourinary, breast, or gastrointestinal tract. The drug-induced Sweet's syndrome is seen in granulocyte-colony stimulating factor, cotrimoxazole, and minocycline.[3]

Cutis laxa is a rare dermatosis characterized by diffuse laxity of the skin resulting in a prematurely aged appearance.

Cutis laxa can be inherited or acquired condition. Inherited cutis laxa can be autosomal dominant, recessive, or X-linked. Acquired cutis laxa is rare and can be subdivided into type 1 and type 2. Type 1 acquired cutis laxa can be either generalized or localized may occur at any age, though the usual onset is in adulthood, usually follows episodes of inflammatory dermatosis, hypersensitivity reaction to insect bite or drugs such as penicillin, penicillamine, and isoniazide and neoplastic disorders. Systemic involvement is common.[4],[5] Pathogenesis of acquired cutis laxa is unknown, deficiency of α1-antitrypsin may allow protease such as neutrophil elastase to destroy dermal elastic tissues to produce cutis laxa in Marshall's syndrome.[6]

Systemic involvement in type 1 is wide and at times severe in the form of pulmonary emphysema, pneumothorax, aortic ectasia, cor pulmonale, inguinal and umbilical hernias, and gastrointestinal and urogenic diverticulae.[4]

Marshall's syndrome is characterized by acute inflammatory skin lesions with subsequent development of localized elastolysis without any internal organ involvement. It occurs most often in infancy or childhood. Marshall's syndrome was first described by Marshall et al., in a group of five South African children who had acute erythematous infiltrated papular eruption.[7] A senile aged expression is characteristic and in some cases most of the body surface is involved.[8]

There are very few cases reported as Sweet's syndrome leading to acquired cutis laxa – Marshall's syndrome.[9],[10],[11]

The systemic steroid is the first line therapy for Sweet's syndrome, as in our patient, showed excellent response with rapid control of progression. There is no satisfactory treatment for cutis laxa.


  Conclusion Top


Sweet's syndrome is commonly seen in adult females, it is extremely rare in children. Acquired cutis laxa is also a rare condition in children, even though Marshall's syndrome is not having systemic manifestations, when cutis laxa occurs following Sweet's syndrome, patients should be thoroughly investigated for malignancy. Hence, all acquired cutis laxa cases should be screened for cardiac, respiratory, and gastrointestinal involvement as it is common in type 1.

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.

 
  References Top

1.
Sweet RD. An acute febrile neutrophilic dermatosis. Br J Dermatol 1964;76:349-56.  Back to cited text no. 1
    
2.
García-Romero MT, Ho N. Pediatric sweet syndrome. A retrospective study. Int J Dermatol 2015;54:518-22.  Back to cited text no. 2
    
3.
Cohen PR. Sweet's syndrome – A comprehensive review of an acute febrile neutrophilic dermatosis. Orphanet J Rare Dis 2007;2:34.  Back to cited text no. 3
    
4.
Riveros CJ, Gavilán MF, França LF, Sotto MN, Takahashi MD. Acquired localized cutis laxa confined to the face: Case report and review of the literature. Int J Dermatol 2004;43:931-5.  Back to cited text no. 4
    
5.
Reed WB, Horowitz RE, Beighton P. Acquired cutis laxa. Primary generalized elastolysis. Arch Dermatol 1971;103:661-9.  Back to cited text no. 5
    
6.
Hwang ST, Williams ML, McCalmont TH, Frieden IJ. Sweet's syndrome leading to acquired cutis laxa (Marshall's syndrome) in an infant with alpha 1-antitrypsin deficiency. Arch Dermatol 1995;131:1175-7.  Back to cited text no. 6
    
7.
Marshall J, Heyl T, Weber HW. Post inflammatory elastolysis and cutis laxa. S Afr Med J 1966;40:1016-22.  Back to cited text no. 7
[PUBMED]    
8.
Verhagen AR, Woerdeman MJ. Post-inflammatory elastolysis and cutis laxa. Br J Dermatol 1975;92:183-90.  Back to cited text no. 8
[PUBMED]    
9.
Timmer-DE Mik L, Broekhuijsen-VAN Henten DM, Oldhoff JM, DE Geer DB, Sigurdsson V, Pasmans SG. Acquired cutis laxa in childhood sweet's syndrome. Pediatr Dermatol 2009;26:358-60.  Back to cited text no. 9
    
10.
Narayanan M, Phiske M, Jerajani HR, Dhurat R. Sweet's syndrome leading to acquired cutis laxa (Marhall's syndrome) in a child. Indian J Dermatol 2004;49:134-7.  Back to cited text no. 10
  Medknow Journal  
11.
Haider M, Alfadley A, Kadry R, Almutawa A. Acquired cutis laxa type II (Marshall syndrome) in an 18-month-old child: A case report. Pediatr Dermatol 2010;27:89-91.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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