Leukaemia-associated eosinophilic folliculitis (Ofuji's disease).

BACKGROUND: Ofuji's disease is an eosinophilic dermatosis mostly affecting male subjects. It is characterized by the appearance of follicular papulopustolosis, at times on an erythematous base, which tend to form an annular configuration. PATIENTS AND METHODS: Two cases are described: the first is a 45-year-old man affected by acute myeloid leukaemia and Ofuji's disease; the second is a 61-year-old man affected by chronic lymphatic leukaemia and Ofuji's disease. Culture tests were negative in both cases. In both patients no hypereosinophilia was found. CONCLUSIONS: The simultaneous presence of the two pathologies in these patients can only give rise to hypothesis. In the first case it could be attributed to interleukin (IL)-5, which causes both eosinophilic hyperproduction and blast differentiation. In the second case the hypersecretion of intercellular adhesion molecule type 1 (ICAM-1) in Ofuji's disease could be attributed to a modified transcriptional gene belonging to the class of immunoglobulin codifying genes located on the altered chromosomes in a certain percentage of leukaemic patients.

Lichen planopilaris: report of 30 cases and review of the literature.

BACKGROUND: Lichen planopilaris (LPP) affects primarily the scalp, resulting in scaling, atrophy, and alopecia with scarring. The purpose of our study was to obtain original data on LPP and to evaluate the efficacy of topical therapy in comparison with systemic therapies. METHODS: We examined 30 patients affected by LPP between 1996 and 2001, performing clinical, laboratory, histopathologic and direct immunofluorescence examinations. Twenty-one of the patients (70%) were women and nine (30%) were men. The average age at presentation was 51.5 years. The average duration of the disease was 13 months at the time of the diagnosis. All patients received topical steroids for a total of 12 weeks. RESULTS: Resolution of the inflammatory process and blocking of the cicatricial progression were observed in 66% of cases, a mild reduction of fibrosis and cicatrization in 20% of patients, and no response in 13%. CONCLUSIONS: We concluded that topical therapy may be a valid alternative to systemic therapies, especially in patients with lesions in the early phase.

Turner's syndrome associated with psoriasis and alopecia areata.

Turner's syndrome is a sex chromosomal abnormality characterized by gonadal failure, short stature, skeletal and medical anomalies due to structural defects or monosomy of the X chromosome. The association between this syndrome and autoimmune diseases has been reported in the literature. This case report highlights the finding of two immunological skin diseases, alopecia areata and psoriasis, in an 18-year-old girl with Turner's syndrome. By the time of her referral to the Department of Dermatology, Verona University (Italy) the girl had suffered from psoriasis on the scalp for 5 years and alopecia for 6 months; the diagnoses were confirmed by histological evaluation. Topical therapy was useful for the treatment of the psoriatic lesions but not for the alopecia areata. Alopecia areata and psoriasis occurring together in Turner's syndrome may indicate some genetic relationship and could support the concept that these patients have the tendency to develop autoimmune or immunological diseases. Anxiety, depression and unsatisfactory relationships could have been important trigger factors in our patient. Multidisciplinary management, including psychological, educational and behavioural techniques, in addition to other therapies, could be useful in treating these conditions.

Granulomatous folliculitis as a manifestation of post-herpetic isotopic response.

We report a case of postherpetic granulomatous folliculitis in a 52-year-old female. The several cutaneous granulomatous eruptions following herpes zoster reported in the literature include annular, sarcoid and tuberculoid granuloma, granulomatous vasculitis and granulomatous folliculitis. The mechanism of granuloma formation is probably triggered by a delayed hypersensitivity response to the virus.

Psoriatic scarring alopecia: observations in four patients.

BACKGROUND AND DESIGN: Although acute or chronic hair loss in psoriasis of the scalp can be a symptom of the disease, until now there has been no agreement as to whether or not it is only restricted to erythrodermic, generalized pustular and scalp plaque psoriasis. The purpose of this study was to evaluate patients with chronic scalp psoriasis and alopecia and to determine if the alopecia was of the scarring type. RESULTS: We report four cases of psoriatic scarring alopecia and describe the clinical and histologic features of these patients. The diagnosis was made by scalp biopsies (vertical and transverse sections) and other causes of scarring alopecia were excluded. CONCLUSIONS: Psoriasis can cause scarring alopecia.

Simultaneous occurrence of lichen striatus in siblings.

Lichen striatus (LS) is a self-limiting linear papular dermatosis of unknown etiology seen mostly in children. We report LS occurring in two pairs of siblings: two sisters who had LS at an interval of 6 months and a brother and sister who had the dermatosis contemporaneously after an episode of flulike fever. In all four patients family history was positive for atopy. LS is frequently associated with atopic diseases. The abnormal immune status of patients with atopy may be a predisposing factor in the induction of LS. In our patients the simultaneous occurrence of LS in siblings after a flulike fever appears to corroborate the hypothesis that a viral infection is a possible candidate, as other authors have proposed. The rarity of familial cases of LS is, in our opinion, due to the exceptional confluence of difference sporadic events (atopy, viral infection caught at a specific period of life such as childhood, and a genetic predisposition) simultaneously present in the same patient.

Demodicidosis in immunocompetent young children: report of eight cases.

Demodex folliculorum and Demodex brevis are obligate parasites of the human pilosebaceous unit. They are the most common permanent ectoparasites in adults, but their incidence on children's skin is rare. Only few cases of demodicidosis have been reported in children aged below 5 years and most of them were suffering from leukemia or HIV infection. The aim of this study is to describe demodicidosis in young immunocompetent children. The clinical details of 8 healthy children are given. There were 4 males and 4 females aged between 10 months and 5 years referred to us for evaluation of a facial eruption characterized by erythema, papulopustules and variable edema. In 7 of these patients, skin scrapings were performed and in 1 a 4-mm punch biopsy. Numerous D. folliculorum were found in skin scrapings of 7 cases and at a histologic examination of skin biopsy in 1 case. We employed topical metronidazole gel 1% in all patients and we obtained a 100% recovery without relapses after a 1- to 3-year follow-up. In 2 of our cases Demodex infestation had a mild form resembling pityriasis folliculorum and the other cases presented a rosacea-like form. The reason why these young immunocompetent children developed demodicidosis is still under evaluation and investigation.