|Year : 2016 | Volume
| Issue : 1 | Page : 68-70
Neha Meena1, Prafulla K Sharma1, Minakshi Bhardwaj2, Sawan Kumar2
1 Department of Dermatology, STD and Leprosy, PGIMER, Dr. Ram Manohar Lohia Hospital, Baba Kharak Singh Marg, New Delhi, India
2 Department of Pathology, PGIMER, Dr. Ram Manohar Lohia Hospital, Baba Kharak Singh Marg, New Delhi, India
|Date of Web Publication||4-Jan-2016|
Department of Dermatology, STD and Leprosy, PGIMER, Dr. Ram Manohar Lohia Hospital, Baba Kharak Singh Marg, New Delhi - 110 001
Source of Support: None, Conflict of Interest: None
Marshall's syndrome is a form of acquired cutis laxa without systemic involvement. It is characterized by acute onset of multiple erythematous papule and plaques that resolve with postinflammatory elastolysis and cutis laxa. We report a case of 4-year-old boy with the typical features of Marshall's syndrome.
Keywords: Anetoderma, cutis laxa, elastophagocytosis, Marshall's syndrome, postinflammatory elastolysis
|How to cite this article:|
Meena N, Sharma PK, Bhardwaj M, Kumar S. Marshall's syndrome. Indian J Paediatr Dermatol 2016;17:68-70
| Introduction|| |
Cutis laxa is characterized by loose, wrinkled skin, and a premature aging look. It is of two types- congenital (autosomal dominant, autosomal recessive, and X-linked) and acquired (type I and type II). Marshall's syndrome is acquired form of cutis laxa without systemic involvement. Marshall's syndrome presents with multiple erythematous papule and plaques in acute phase that resolves and develop postinflammatory elastolysis and cutis laxa in chronic phase., We report a case of 4-year-old boy with typical features of this rare pediatric syndrome.
| Case Report|| |
A 4-year-old boy presented with multiple, tender, and well-defined erythematous, edematous plaques with irregular margins, varying in size from 0.5 cm × 1 cm to 4 cm × 3 cm on face, anterior aspect of neck and upper chest, trunk, dorsum of both hands, extensor aspect of both upper and lower limbs. Plaques on upper chest and dorsum of hands show shiny, wrinkled surface with a few erythematous papules on the margins [Figure 1]. He also had mild fever, bilateral submandibular, nontender, soft, and mobile lymphadenopathy. No history of prior drug intake. Laboratory investigations and chest X-ray were normal. He had multiple similar episodes in last 8 months.
|Figure 1: Dorsum of hand shows shiny wrinkled surface with a few erythematous papules on the margins|
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Biopsy from erythematous papule revealed mild hyperkeratosis and follicular plugging in epidermis. Upper dermis showed perivascular and periadnexal lymphohistiocytic infiltrate [Figure 2]. Verhoeff-Van Geison (VVG) stain revealed reduced elastic fibers in upper and mid dermis [Figure 3]. Biopsy taken 8 months back was reviewed, which showed hyperkeratosis, irregular acanthosis and mild spongiosis. Dermis showed pan dermal interstitial infiltrate comprised mainly of neutrophils, histiocytes with phagocytic debris, few lymphocytes, eosinophils, and necrotic nuclear debris [Figure 4]. VVG stain depicted reduced elastic fibers and elastophagocytosis [Figure 5]. The patient was managed symptomatically.
|Figure 2: Perivascular and periadnexal lymphohistiocytic infiltrate (H and E, ×20)|
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|Figure 4: Pan dermal interstitial infiltrate comprising of mainly neutrophils, histiocytes with phagocytic debris, few lymphocytes, eosinophils and necrotic nuclear debris (H and E, ×10)|
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|Figure 5: Elastolysis and elastophagocytosis in upper and mid dermis (VVG, ×40)|
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| Discussion|| |
Marshall's syndrome is a rare pediatric disorder first described in 1966 by Marshall et al. in a case series of five African children. The acute phase is characterized by the onset of multiple, erythematous, edematous papules and plaques on face, trunk, and extremities. These plaques resolve with central clearing, hyperpigmentation, and peripheral collarette of scales. The lesions come in crops with or without fever, peripheral leukocytosis, and eosinophilia. Biopsy in the acute stage shows perivascular infiltrate, mainly neutrophils and eosinophils. Chronic phase presents with resolution of plaques to form wrinkled and loosed skin of involved skin with or without extension to normal skin, giving the premature look to the patient. Loss of elastic fibers, elastolysis, and elastophagocytosis is found in chronic phase.,
Elastophagocytosis is the phagocytosis of elastic fibers that can microscopically be seen in the cytoplasm of histiocytes, multinucleated giant cells, or both. In postinflammatory elastolysis, elastin degradation occurs as a result of elastinolytic enzymes. 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.
The differential diagnosis of Marshall's syndrome includes anetoderma and mid dermal elastolysis (MDE). Anetoderma occurs mainly in women aged 20–40 years with few reports in younger age., It is characterized by smaller atrophic plaques which yield on pressure, admitting the finger through the surrounding ring of normal skin., If the smaller lesions coalesce to form larger atrophic plaques, they are indistinguishable from acquired cutis laxa. However, on VVG stain, anetoderma shows upper dermal elastolysis. While, in Marshall's syndrome upper, middle and/or deep dermal elastolysis can be seen. Acquired cutis laxa type I is associated with local or generalized loose skin, pendulous folds, which gives the patient to look like an old man and with the systemic involvement., MDE is a rare disorder, occurs in young to middle-aged females. The lesions are found on neck, trunk, and extremity but rarely on face. MDE is preceded or accompanied by burning or urticarial in half of the cases, manifests as erythematous patches and plaques, perifollicular papules and telangiectasia that resolve to leave behind asymptomatic patches of finely wrinkled skin with pigmentary changes. In MDE, the elastolysis is confined to the mid reticular dermis.
Marshall's syndrome is managed symptomatically. Dapsone and prednisolone have been tried. Plastic surgery, face uplift, and botulinum toxin injection can be used.,
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Conflicts of Interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]