Atrophoderma is a rare benign dermatosis with an onset usually in childhood or youth. It is slowly progressing. The disease is a variant of the follicular disorders of keratinization. Treatment is challenging and often frustrating.
Anca Chiriac 1-3 and Uwe Wollina 4
1 Department of Dermatology, Nicolina Medical Center, Iași, Romania.
2 Department of Dermatology, Apollonia University, Iași, Romania.
3 P. Poni Institute of Macromolecular Chemistry, Romanian Academy, Iași, Romania.
4 Department of Dermatology and Allergology, Municipal Clinic Dresden, Dresden, Germany
Atrophoderma vermiculatum (AV), is a rare skin disease, with unknown aetiology, characterized by a peculiar clinical aspect, “worm-eaten”, reticular, or honeycomb atrophic lesions on the face. The onset is, in most cases, during childhood or puberty and has a slow progressive evolution. The lesions are spread on the cheeks, pre-auricular area and forehead [1, 2]. We present a case of de novo atrophoderma vermiculatum on the temporal area, in an old healthy woman.
A 67-year-old woman was addressed to Dermatology for new onset lesions on the forehead, observed by the patient few months prior to present consultation. She described a sudden onset of the lesions, with no progressive evolution and no evident correlation. She was completely asymptomatic, but concerned about the cause and the esthetic prejudice. She has been treated for the previous months with emollients, but not improvement or worsen of the skin lesions was reported.
Dermatological examination described multiple small, homogenous, round, atrophic lesions in a honeycomb pattern, localized on the forehead, closed to the eyebrows (Fig. 1). The lesions did not appear like scars, but more like punch-out atrophic “stamps”. No inflammatory lesions were noted. A meticulous clinical examination of the entire body failed to reveal other anomalies, no other members of the family were in evidence with similar lesions or other skin diseases. A skin biopsy punch type 4 mm was denied by the patient due to the risk of scarring on the face, taking into consideration a high degree of anxiety already present and induced by existing skin lesions.
A clinical diagnosis of atrophoderma vermiculatum was admitted and several therapeutic procedures were performed, such as topical retinoids, medium peelings, dermabrasion and laser therapy (carbon dioxide) but without beneficial effects. The patient decided to abandon any medical procedure, although the quality of life was affected.
Atrophoderma vermiculatum and folliculitis erythematosa reticulate are the same disease, probably caused by abnormal keratinization of the pilosebaceous follicle [1,2]. For some authors atrophoderma vermiculatum, keratosis pilaris atrophicans faciei and keratosis spinulosa decalvans share some similarities and can be grouped together under an umbrella term: keratosis pilaris atrophicans .
There are few cases of AV reported in the literature, the majority in children. The onset in the second half of life – as in our case – is very rare. Most cases are symmetrical, but monoliteral disease has rarely been described . The aetiology is still a matter of discussion, although autosomal dominant inheritance was a hypothesis . The disease may be associated in children with associated pathologies such as congenital heart block, congenital cataract, hyper-IgE syndrome, neurofibromatosis, oligophrenia, TGFBR2-related Loeys-Dietz syndrome, Rombo syndrome or Down syndrome [4-6].
Treatment is puzzling and frustrating, despite the variable schemes: topical steroids, keratolytics, intralesional steroids, topical retinoids, oral retinoids, dermabrasion, collagen implantation, laser therapy .
Particularities of present case are the age of the patient, the sudden occurrence of the lesions, the impressive clinical picture and the limitation of medical approach.