Rombo syndrome manifests with vermiculate
atrophoderma in the face, hypotrichosis, cyanotic erythema in the hands and feet, multiple BCCs, and trichoepithelomata (9).
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.
INTRODUCTION: Idiopathic
atrophoderma of pasini and pierini is a form of dermal atrophy that manifests as single or multiple sharply demarcated hyper pigmented, non-indurated patches.
Rombo syndrome shows an autosomal dominant inheritance and produces a similar phenotype except that it is associated with vermiculate
atrophoderma.
Differential diagnoses included scarring secondary to varicella,
atrophoderma vermiculatum, hydroa vacciniforme and infantile acne but these were excluded by the chronic course, clinical findings and lack of seasonal variation.
