Dermatologie

Cas 11200614

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  F de 20 ans, présente une dépression de 2 cm sur la cuisse évoluant depuis plusieurs mois. La dépression est bien délimitée, ferme, a bordure abrupte. Le revêtement apparait aminci, décoloré, mais la sensibilité est normale. Pas d'érythème. Pas de notion de traumatisme.   F 20 yo with a single sharply demarcated, firm patch mesuring 2 cm on the thigh, of many months duration, marked by a depression of the skin with an abrupt edge. The affected skin appears thinned and discolored, but the consistency and feel of the affected skin remains normal. No redness noted. No trauma in the history.  
  Biopsie chirurgicale.   Surgical biopsy  
    HE ZOOM X 100  
     
     
     
  Coloration de Verhoeff   Verhoeff stain  
 

Diagnostic proposé

 

 

 

 

 

 

 

Atrophodermie idiopathique de Pierini et Pasini

 

Proposed diagnosis

 

 

 

 

 

 

 

Idiopathic atrophoderma of Pasini and Pierini

 
  Arguments      
     
  Commentaire   Comment  
 

La plupart des réferences considèrent l'atrophodermie idiopathique comme une forme de sclérodermie localisée.

Most references consider idiopathic atrophoderma as a form of localised scleroderma.  

Reference from emedicine:

Background
Idiopathic atrophoderma of Pasini and Pierini (IAPP) is a form of dermal atrophy that manifests as single or multiple sharply demarcated, hyperpigmented, nonindurated patches. These patches are marked by a slight depression of the skin with an abrupt edge, usually on the backs of adolescents or young adults. The lesions may be discrete or confluent. The affected skin appears thinned and discolored, but the consistency and feel of the affected skin remains normal.
In 1923, Pasini described a case of pigmentary atrophoderma that was both clinically and histologically unique from any known atrophy, including localized scleroderma, under the name progressive idiopathic atrophoderma. In 1936, in Argentina, Pierini and Vivoli extensively studied and defined the condition and its possible link to morphea. In 1958, the disorder was first introduced into the American dermatologic literature by Canizares et al,[ who reviewed Pierini’s findings and renamed it idiopathic atrophoderma of Pasini and Pierini. Canizares et al believed that idiopathic atrophoderma of Pasini and Pierini differed sufficiently from morphea to classify it as a distinct entity.
Since then, reports of the co-occurrence of morphea and occasionally lichen sclerosus et atrophicus with idiopathic atrophoderma of Pasini and Pierini suggest a close relationship between idiopathic atrophoderma of Pasini and Pierini and morphea. In 2000, Yokoyama et al reported that skin glycosaminoglycans extracted from idiopathic atrophoderma of Pasini and Pierini lesions are different from those in typical morphea lesions.

Pathophysiology
The cause of atrophoderma of Pasini and Pierini is not known. The pathophysiologic events that cause the discrete lesions seen clinically, as well as the timing of their appearance, are also unknown. Some authors have suggested a role for infection with Borrelia burgdorferi.

 

Epidemiology

United States
The exact incidence of idiopathic atrophoderma of Pasini and Pierini is not known. The small number of cases reported may reflect its asymptomatic nature rather than its true incidence.
International
Idiopathic atrophoderma of Pasini and Pierini is more frequently reported in Europe than in North America, paralleling the incidence of acrodermatitis chronica atrophicans and suggesting involvement of European Borreliastrains.

 
 

Mortality/Morbidity

Idiopathic atrophoderma of Pasini and Pierini is a benign, asymptomatic disease and is not associated with any significant complications or mortality.

 
 

Race

Idiopathic atrophoderma of Pasini and Pierini is more common in whites and is rarely reported in blacks or Asians.

 
 

Sex

Idiopathic atrophoderma of Pasini and Pierini is more frequently encountered in women than in men, with a ratio of 6:1.

 
 

Age

Idiopathic atrophoderma of Pasini and Pierini usually begins insidiously in individuals during the second or third decade of life. However, it has been described in individuals as young as 7 years and as old as 66 years, with one report of congenital atrophoderma.

 
 

History

Idiopathic atrophoderma of Pasini and Pierini is typically a benign, asymptomatic condition. The disorder usually begins during adolescence or early adulthood with a slightly erythematous lesion appearing on the trunk, commonly on the back or lumbosacral region, followed in frequency by the chest, arms, and abdomen.
At first, only a single lesion may be present, but more often, multiple lesions 1-12 cm in diameter are present. Within 1-2 weeks, these lesions become hyperpigmented, appearing slate-gray to brown. Initially, localized lesions may slowly spread for months to years before becoming quiescent. Discrete new lesions may appear for 10-20 years, and old lesions may slowly enlarge, giving the skin a moth-eaten appearance. Transformation to generalized morphea has not been observed.

 
 

Physical

Lesions are single or multiple, irregularly round or ovoid patches, varying in size from a few centimeters to large areas across the trunk. A predilection for the legs is reported. The face, hands, and feet are usually spared. Distribution is often symmetric and bilateral; however, reports have described solely unilateral cases. Lesions have traditionally been described as hyperpigmented; however, Saleh et al described a retrospective study of 16 Lebanese patients in whom lesions were more hypopigmented (56%) and skin-colored (25%). The lesions are usually asymptomatic and lack inflammation. The skin lesions may coalesce over time to form large, irregular, pigmented areas. The areas usually are brown but may have a blue hue.
Eventually, the pigmentation lightens, and the involved skin becomes depressed below the level of the surrounding skin. This change produces the characteristic, sharply defined "cliff-drop" borders, ranging from 1-8 mm in depth, although they can have a gradual slant. Close examination of the skin with side lighting demonstrates the unique cliff-drop border, giving the impression of an inverted plateau. Multiple lesions may have an appearance similar to Swiss cheese.
The skin surrounding the patches appears normal. No erythema or lilac ring, as in morphea, is observed. Once indentation occurs, the lesions may become quiescent, stopping any further enlargement. The surface of the skin feels and appears normal, and no induration or sclerosis is noted.
In the late stage, superficial blood vessels may be visible in the depressed patches. No palpable difference can be felt between normal and affected skin in the late stage. White, shiny sclerodermatous indurations are sometimes seen in the central parts of the lesions, and concurrent but separate characteristic plaques of morphea may be noted, supporting a possible relation between these disorders.

 
  Causes

Idiopathic atrophoderma of Pasini and Pierini may represent a late atrophic stage of morphea.
Some findings suggest that the spirochete B burgdorferi may be involved in the pathogenesis of some cases of idiopathic atrophoderma of Pasini and Pierini. Buechner and Rufi studied the sera of 26 patients with typical lesions. Ten (53%) of the 26 patients had immunoglobulin G anti– B burgdorferi antibodies, and 6 (14%) of control subjects had these antibodies. No immunoglobulin M antibodies were found.

 
 

Differentials

  • Anetoderma
  • Lichen Sclerosus et Atrophicus
  • Morphea
  • Postinflammatory Hyperpigmentation
 
 

Laboratory Studies

Routine baseline studies of the blood and urine may help to exclude other conditions, but they do not help in the diagnosis of idiopathic atrophoderma of Pasini and Pierini.
Screening tests such as the enzyme-linked immunosorbent assay may be performed to detect anti– B burgdorferi antibodies.
One recent anecdotal case report advocates thyroid gland evaluation in all patients with sclerodermalike disorders because of pathophysiological links between cutaneous fibrosis and the thyroid gland.

 
 

Procedures

Skin biopsy is not always necessary to make the diagnosis; however, it may be useful to exclude other entities.
Dermal atrophy is more easily evaluated with wedge excision than punch biopsy. An elliptical biopsy specimen is sectioned longitudinally from an area that includes normal skin and the cliff-drop border of the lesion. If dermal atrophy is present, the transition between normal dermis to atrophied dermis is discernible.

Histologic Findings
Histopathologic changes, often minimal and nondiagnostic, consist of a decrease in the size of the dermal papillae with flattening of the rete pegs. The epidermis is usually normal or slightly atrophic. Melanin may be increased in the basal layer. Interstitial edema and a mild perivascular infiltrate consisting of lymphocytes and histiocytes may be present. Collagen bundles show varying degrees of homogenization and clumping in the mid and reticular dermis. When compared with adjacent normal skin, dermal thickness is reduced. The sweat glands, pilosebaceous units, and appendages are not affected. Elastic fibers appear normal after elastic tissue staining and with electron microscopic studies.
If preexisting patches show sclerodermatous changes, histology may reveal varying degrees of collagen sclerosis resembling morphea. Direct immunofluorescence of early lesions may show nonspecific immunoglobulin M and C3 staining in the dermal papillary blood vessels or at the dermoepidermal junction.  CD34 dermal dendrocytes are reduced, just as in morphea.

 
 

Medical Care

No treatment is consistently effective, but some patients respond to topical corticosteroids, antibiotics, or antimalarials.
Results of medical treatment with antibiotics have been inconclusive. In patients with new early-stage idiopathic atrophoderma of Pasini and Pierini, especially those with a positive B burgdorferi antibody titer, the standard recommended therapy for Lyme disease is suggested. A retrospective evaluation of 25 patients treated with oral penicillin (2 million IU/d) or oral tetracycline (500 mg 3 times/d) for 2-3 weeks showed clinical improvement with no new active lesions in 20 patients. The same study also showed no progressive disease in 4 of 6 patients who did not receive treatment.
Anecdotal reports have described beneficial treatment with the use of hydroxychloroquine and potassium aminobenzoate.

 
 

Surgical Care

Surgical treatment has generally not been helpful in improving the appearance of the atrophied skin.
Arpey et al showed the Q-switched alexandrite laser (755 nm) to be effective in diminishing the hyperpigmentation by 50% after 3 treatments in one case.

 
 

Complications

Idiopathic atrophoderma of Pasini and Pierini is typically a benign, asymptomatic condition; no complications have been reported.

 
 

Prognosis

Idiopathic atrophoderma of Pasini and Pierini has no known effect on overall health. The condition often runs a protracted course over 10-20 years and may self-resolve.