What is atrophoderma of Pasini and Pierini?

Atrophoderma of Pasini and Pierini (APP) is a rare skin disorder affecting dermal collagen and presenting with dermal atrophy. The classic clinical manifestations of APP are hyperpigmented or hypopigmented, depressed areas of skin on the trunk or extremities (picture 1).

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Can atrophoderma be cured?

The course of atrophoderma is benign, and there is no known effective treatment. The disorder remains active for months to years and lesions persist indefinitely, but there are no reports of systemic involvement or complications.

What is Pierini?

Atrophoderma of Pasini and Pierini (ATPP) is a rare skin condition that results in patchy areas of discolored skin. The main symptom of atrophoderma of Pasini and Pierini is multiple irregularly shaped, dark or light areas of skin in which the tissue under the skin breaks down resulting in a depression (dent).

What is en coup de Sabre?

En coup de sabre (ECDS) is a rare form of localized scleroderma that typically manifests in children and women. It presents as a fibrous pansclerotic plaque extending in a bandlike distribution on the frontoparietal scalp with surrounding scarring alopecia.

What is bullous morphea?

Bullous morphea is a rare variant of localized scleroderma characterized by occasional intermittent blisters. Lichen sclerosus is a chronic inflammatory disease. The coexistence of morphea and lichen sclerosus has been reported in different sites in the same patient and more rarely in the same lesion.

What is linear scleroderma?

Linear scleroderma is a progressive loss of subcutaneous fat with pigment changes in the skin. It is a type of localized scleroderma in which the area of skin affected appears in a band. It typically first appears in young children on one side of the body.

What is generalized morphea?

Generalized morphea is defined as more than 4 indurated plaques larger than 3 cm each and/or involving 2 of more of 7 anatomic sites (head-neck, each extremity, anterior trunk, and posterior trunk) but sparing the face and hands.

Is Parry-Romberg syndrome painful?

Parry-Romberg syndrome is also accompanied by neurological abnormalities including seizures and episodes of severe facial pain (trigeminal neuralgia). The onset of the disease usually begins between the ages of 5 and 15 years.

What causes bullous morphea?

Bullous morphea is a rare form of localized scleroderma (morphea) characterized by bullae on or around an atrophic morphea plaque [1]. The cause of bullae formation in morphea is multifactorial, with lymphatic obstruction from the sclerodermatous process being considered as the most likely cause [2].

What are the symptoms of Pasini and Pierini syndrome?

The main symptom of atrophoderma of Pasini and Pierini is dark or light-colored indented areas of skin with irregular shapes and borders. Some people have pain, itching, or numbness associated with the skin findings. [1] [2] The skin findings in this condition usually appear in adolescence or early adulthood.

What are the clinical entities with depressed lesions or atrophoderma?

Other clinical entities with depressed lesions or atrophoderma include anetoderma, long-standing lupus profundus, eosinophilic fasciitis, lipodystrophy, and atrophoderma of Moulin. Except for atrophoderma of Moulin, these entities can be readily differentiated based on the history, clinical, and histopathologic findings.

How is anetoderma differentiated from atrophoderma of Moulin?

Except for atrophoderma of Moulin, these entities can be readily differentiated based on the history, clinical, and histopathologic findings. In anetoderma, the lesions have a soft crinkly feel due to the significant alteration in the elastic fibers.