Multiple trichoepitheliomas also can be seen in other syndromes including Rombo syndrome, which is characterized by basal cell carcinomas, milia, hypotrichosis, distal vasodilation, and
atrophoderma vermiculata; none seen in our patient.
Patients with extragenital lichen sclerosis and
atrophoderma were included in the analysis, but those with pansclerotic morphea and eosinophilic fasciitis were excluded.
Atrophoderma of Pasini and Pierini is a skin disease manifested with depressed skin in areas that have a histopathology confirmed as atrophy with hyperpigmentation.
Bazex-Dupre-Christol syndrome is characterized by follicular
atrophoderma, hypotrichosis, and hypohydrosis in addition to multiple BCCs.
The clinical differential diagnosis includes aplasia cutis, anetoderma and
atrophoderma, and atrophic DFSP.
Bazex-Dupre-Christol syndrome (BDCS) is clinically characterized by multiple basal cell carcinomas of the face occurring mainly during the second and third decade of life, follicular
atrophoderma predominantly of the dorsum of hands and feet and generalized hypotrichosis, sometimes with pili torti and trichorrhexis nodosa.
Initially, lichen sclerosis et atrophicus,
atrophoderma of Pasini-Pierini, sarcoidosis, mycosis fungoides, post-inflammatory hyperpigmentation were considered in the differential diagnosis of our case.
Atrophoderma Pasini-Pierini is a primary atrophic abortive morphea.
Presence of the antinuclear antibodies and antibodies to Borrelia burgdorferi among patients with morphea en plaque, deep linear scleroderma and
atrophoderma Pasini-Pierini.
It is doubtful, however, if other conditions such as
atrophoderma vermiculatum or honeycomb atrophy of the face, should be included in the same spectrum.
A total of 78% of patients had the plaque variant of morphea, with the rest having the guttate variant, idiopathic
atrophoderma of Pasini and Pierini, linear scleroderma, and profound scleroderma.
A total of 78% of the patients had the plaque variant of morphea, with the remainder having either the guttate variant, idiopathic
atrophoderma of Pasini and Pierini, linear scleroderma, or profound scleroderma.
