Case for diagnosis

Abstracts

É relatado caso de elastólise da derme média em paciente do sexo feminino, de 37 anos, que apresentava áreas de enrugamento fino da pele, principalmente nas flexuras e pápulas perifoliculares com aspecto em peau d´orange na região paravertebral, conferindolhe aspecto precocemente envelhecido. A histologia mostrava fragmentação e rarefação das fibras elásticas restritas à porção média da derme.

Derme; Envelhecimento da pele; Tecido elástico

WHAT IS YOUR DIAGNOSIS?

Case for diagnosis^*

Maria Luiza Pires de Freitas ^I; José Antônio Sanches Junior^II; Paulo Guilherme de Oliveira Salles^III

^IM.D., Dermatologist. Belo Horizonte (MG), Brasil

^IIM.D, Ph.D. Professor at the Department of Dermatology, University of São Paulo Medical School. (FMUFMG)

^IIIM.D., Pathologist M. Sc. in Medical Pathology (FMUFMG) Ph.D. student of Medicine (FMUFMG) MBA in Health Systems Management IBMEC/ MG Place of

^{Mailing address}

ABSTRACT

A case of mid-dermal elastolysis is reported in a 37-year-old female patient, who presented skin areas with fine wrinkles, mainly in fold areas and also perifollicular papules similar to peau d'orange in the paravertebral area, lending her a prematurely elderly appearance. Histological examination revealed fragmentation and rarefaction of elastic fibers, restricted to the mid-dermis portion.

Keywords: Dermis; Elastic tissue; Skin aging

DISEASE HISTORY

White 37-year-old female patient presented with areas of skin-colored fine wrinkles, following cleavage lines, which were asymptomatic, with no sings of inflammation, atrophy or herniation.

Lesions had been noticed by her a few months before the visit, appeared slowly and become increasingly extensive. They were predominantly located on folds - cervical, axillary and infra-mammary - and on flanks (figures 1 and 2). Moreover, she presented skin-colored perifollicular papules, with a peau d'orange aspect in the paravertebral region.

She reported recurrent urticary associated with large angioedema plaques, of long evolution. Recently, manifestations had become more frequent, with more persistent lesions and with bad response to therapy with hydroxyzine 50mg/day and prednisolona 40mg/day, which were used in some of them. She accomplished better control of the urticary by using doxepine 30mg/day, remaining asymptomatic for several months. Wrinkling areas held no temporal or topographic coincidence to urticary lesions.

Serology for borrelia IgM and IgG, rheumatoid factor, ANF, anti-thyroid antibodies, blood count, urinalysis, VHS, hepatic function tests, protein electrophoresis, complement dosage and anti-HTLV 1 were performed, all within limits of normality.

Histopathological examination showed an epidermis with no alterations and dermis exhibiting fragmentation and rarefaction of elastic fibers, restricted to the mid portion (figures 3A and 3B).

Immunohistochemistry displayed signs of discrete dermatitis, with mild lymphocytic infiltrate constituted by T-cells surrounding superficial dermal vessels, with no characteristics of lymphoma. We verified no reactivity of the cells in the inflammatory infiltrate to any of the following stains: anti-CD20, anti-CD3, anti- CD43 and anti-CD45.

Patient maintained follow-up for a three-year period, developing new lesions on the anterior portion of the thighs.

COMMENTS

Mid-dermal elastolysis (MDE) is a rare skin disease, described by Shelley and Wood in 1977,¹ with unknown etiology and pathogenesis, and uncertain treatment and prognosis.

Clinically, it manifests by well-delimited circumscribed areas of fine wrinkling of the skin (type I) or by non-confluent perifollicular papular protrusions (type II).^2,3

There are only two cases reported in the literature with simultaneous presentations of both clinical types.⁴

Wrinkles appear prematurely, since it occurs mainly in females aged between 30 and 40 years, lending them a premature elderly appearance. Inflammatory signs are often not previously present, even though in some cases a mild erythema is observed in association with the lesions, thus suggesting the possibility that the elastolysis is secondary to an inflammatory process.⁵

Up to the present, there are no reports of systemic affection.⁵ Roughly half of the cases are preceded by erythema, burning sensation, or urticary. Sun burn, mammary silicon prosthesis implantation, annular granuloma, auto-immune diseases, including SLE, Hashimoto's thyroiditis and rheumatoid arthritis are described as factors which can be associated to MDE.³

MDE presents well-defined clinical and histopathological features, which differ it from other elastic tissue diseases. In anetodermia (macular atrophy), lesions are smaller, located mainly on the trunk, and, upon palpation, one has the impression of a herniary orifice. Post-inflammatory elastolysis presents with inflammatory signs that precede the picture, such as papules and plaques with intense erythema, which evolve to atrophic lesions with fine skin wrinkling; affects almost always face, ears and cervical region, and is more usual in children. Acquired cutis laxa is characterized by generalized laxity and fold areas with redundant and often pendular skin.2,4 Granulomatous cutis laxa is a rare variety of T-cell lymphoma, also predominant in women. Clinically, lesions are pendular and may co-exist in areas with infiltrated plaques.

Up to the moment, there is no therapeutic approach with satisfactory results for MDE.

REFERENCES

Conflict of interests: None

Received on Dec. 22 of 2006

Approved by the Consultive Council and accepted for publication on July. 07 of 2006

• 1. Shelley WB, Wood MG. Wrinkles due to idiopathic loss of mid-dermal elastic tissue. Br J Dermatol. 1977;97: 441-5.
• 2. Sterling JC, Coleman N, Pye RJ. Mid-dermal elastolysis. Br J Dermatol. 1994;130:502-6.
• 3. Gambichler T, Linhart C, Wolter M. Mid-dermal elastolysis associated with Hashimotos thyroiditis. J Eur Acad Dermatol Venereol. 1999;12:245-9.
• 4. Agha A, Hashimoto K, Mahon M. Mid-dermal elastolysis: case report and review of the literature. J Dermatol. 1994;21:760-6.
• 5. Lewis KG, Dill SW, Wilkel CS, Robinson-Bostom L. Middermal elastolysis preceded by acute neutrophilic dermatosis. J Cut Pathol. 2004;31:72-6.

*

Work done at Department of Dermatology of the University Hospital, Federal University of Minas Gerais – UFMG – Belo Horizonte (MG), Brazil.

Publication Dates

• Publication in this collection
  21 June 2007
• Date of issue
  Dec 2006

History

• Accepted
  28 July 2006
• Received
  22 Feb 2006