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The concept of atrophoderma of pasini and pierini as morphea may be falling off a cliff


DII small banner By Warren R. Heymann, MD
Nov. 6, 2017


  Pierini-and-Pasini.jpg
Clinical photos of idiopathic atrophoderma of Pierini and Pasini.
Credit: JAAD
Diagnosing Atrophoderma of Pasini and Pierini (APP) is reasonably straightforward — it usually presents in adolescents and young adults, more often in females, with sharply defined, single or multiple round to oval, hyperpigmented, depressed lesions with a “cliff-drop” border. Unusual presentations may include congenital lesions (1) or with a Blaschkoid pattern. (2)

I stopped biopsying these lesions long ago, having found minimal (or maybe imagined!) findings when comparing the border of involved and uninvolved skin. Saleh et al confirmed my impression in their histologic study of 16 patients with APP. In their series, 12 of the 17 biopsy specimens showed no abnormalities on H&E. Only 5 showed a sparse superficial perivascular or perifollicular lymphocytic infiltrate. Histological features of morphea such as dermal sclerosis, perivascular and interstitial lymphoplasmacytic infiltrate and eccrine gland entrapment were absent in all the biopsy specimens. Changes of elastic fibers on Verhoeff-van Gieson and/or orcein-Giemsa stains were variable within a spectrum ranging from normal to severe diminution with fragmentation of elastic fibers. In 35.3% of their cases, there was a moderate to severe decrease with fragmentation in the elastic fibers. (3)
 
The literature commonly refers to APP as an abortive form of morphea. Is it? I can understand why this is a consideration, as overlapping clinical features have been reported. Years ago, I reported a case of lichen sclerosis within lesion of APP in a middle aged woman. (4) It has been suggested that Linear Atrophoderma of Moulin (LAM), appearing Blaschkoid, and presumably due to genetic mosaicism, is part of a spectrum of APP and morphea, This conclusion was based on a case of LAM in which collagen entrapment of eccrine ducts, typical of morphea, were observed. (5) As is the case in morphea, infection with Borrelia burgdorferi has been suggested as pathogenic in APP. (6) I do not believe that this spirochete is pathogenic in these disorders in the United States. (7)
 
Despite these considerations, the presentation of APP is so distinctive, my opinion is that it is a disease sui generis. In an intriguing study, Viera-Damiani et al evaluated 11 patients with APP and found no abnormalities under light microscopy or by automated quantification. Multiphoton imaging revealed no difference in optical density of either collagen or elastic fibers in lesional and unaffected skin; however, horizontal collagen fiber organization in lesional specimens increased toward the lower dermis, whereas elastic fibers featured greater disorganization within the upper dermis. The authors concluded that the atrophic appearance of APP lesions reflects changes in organization, but not in collagen and elastic tissue content, refuting the concept that APP represents burned out morphea. (8)

Patients displaying the cliff sign wouldn’t mind throwing their dermatologist off a cliff when presented with the depressing fact that these lesions tend to be recalcitrant regardless of treatment, be it with topical steroids, antibiotics, or antimalarials. Arpey et al noted improvement of the hyperpigmentation of APP following 3 treatment sessions with the Q-switched alexandrite laser. (9)

Recently, we discussed the improvement of a patient with Focal Dermal Hypoplasia of Goltz with laser therapy (DI&I October 9th, 2017). If the problem with APP lies with the organization of collagen and elastin, it seems worthwhile to attempt reorientation of these fibers with the fractionated carbon dioxide laser. I predict that maneuver will take the edge off patients’ lesions and their psyche.

1. Kang CY, Lam J. Congenital idiopathic atrophoderma of Pasini and Pierini. Int J Dermatol 2015; 54: e44-6.
2. Ruiz-Villaverde R, et al. Atrophoderma of Pasini and Pierini. Sultan Qaboos Univ Med J 2017: 17: e373-4.
3. Saleh Z, et al. Atrophoderma of Pasini and Pierini: A clinical and histopathological study. J Cutan Pathol 2008; 35: 1108-14.
4. Heymann WR. Coexistent lichen sclerosus et atrophicus and atrophoderma of Pasini and Pierini. Int J Dermatol 1994; 33: 133-4.
5. de Golian E, et al. Linear atrophoderma of Moulin: A distinct entity? Pediatr Dermatol 2014; 31: 373-7.
6. Lee Y, et al. A case of atrophoderma of Pasini and Pierini associated with Borrelia burgdorferi infection successfully treated with doxycycline. Ann Dermatol 2011; 23: 352-6.
7. Heymann WR, Ellis DL. Borrelia burgdorferi infections in the United States. J Clin Aesthet Dermatol 2012; 5: 18-28.
8. Viera-Damiani G, et al. Idiopathic atrophoderma of Pasini and Pierini: A case study of collagen and elastin texture by multiphoton microscopy. J Am Acad Dermatol 2017; 77: 930-7.
9. Arpey C, et al. Treatment of atrophoderma of Pasini and Pierini-associated hyperpigmentation with the Q-switched alexandrite laser: A clinical histologic, and ultrastructural appraisal. Lasers Surg Med 2000; 27: 206-12.

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