Scleredema of Buschke associated with lichen sclerosus: Three cases.

Scleredema adultorum of Buschke is a rare fibromucinous, scleroderma-like connective tissue disease most commonly found in a post-infectious setting or linked to hematological disorders or diabetes. Lichen sclerosus et atrophicus is an autoimmune condition only in 2.5% of cases localized exclusively at an extragenital site, occurring in up to 34% of patients in association with other autoimmune diseases such as vitiligo, thyroid disorders, alopecia areata, lichen planus, morphea, pernicious anemia and systemic lupus erythematosus. In particular, a stronger link with an autoimmune background in lichen sclerosus et atrophicus has been observed in women who showed higher prevalence for autoimmune conditions and circulating autoantibodies. Literature reveals a genetic susceptibility linked to specific HLA types. We report three patients who developed lichen sclerosus et atrophicus superimposed on skin involved by scleredema adultorum of Buschke. Although the association of lichen sclerosus et atrophicus with scleredema adultorum of Buschke could be coincidental, both diseases could be considered part of the spectrum of sclerodermoid disorders with common underlying pathogenetic mechanisms; which could explain the sequential or simultaneous occurrence of both lesions in our patients.

Treatment of Sweet's syndrome in pregnancy.

Pregnancy-associated Sweet's syndrome is a rare occurrence (2%), with good prognosis, spontaneous resolution after delivery, and not increased infant morbidity and mortality. However, differential diagnosis is not easy for physician not familiar with skin lesions. Systemic involvement, even though unusual, might occur in nearly every organ of the body, including pericardium, myocardium, and placenta, as well as one report of early fetal miscarriage, questioning the possibility of risks underestimation. We present two further cases, one occurred in a 31-year-old woman at 26 weeks of gestation and the other on a 26-year-old woman at 24 weeks of gestation, primigravidae. Both presented with tender papules and nodules on their face and upper body parts. Laboratory examinations and skin biopsy histology were pathognomonic. Monitoring of general maternal and fetal conditions showed no signs of sufferance, but the decision to accelerate skin symptoms release, being time to delivery quite distant, challenge the treatment options. There are no recommended treatments for Sweet syndrome and the choice is very limited during pregnancy. A short course of oral steroids was very effective, with lesions healing in few days, no relapses or fetal complications. When pregnant patients exhibit fever, neutrophilia, arthralgia or myalgia, and tender erythematous plaques or nodules, Sweet syndrome should be considered. The trained dermatologist is in the leading position to address the differential diagnosis, reassure the patient, and avoid complications, even if they are rare.

Obesity-Associated Lymphedematous Mucinosis: Two Further Cases and Review of the Literature.

Cutaneous mucinoses are a group of conditions characterized by increased amounts of acid mucin in the dermis. They can be generalized or localized and occur isolated or in the setting of systemic diseases. Obesity-associated lymphedematous mucinosis is a distinct variant of mucinosis occurring in obese patients without any thyroid dysfunction. So far, only few cases of this rare condition have been reported in the English literature. Here, we describe two new cases and discuss some histological differences with the pretibial myxedema.

D-penicillamine elastosis perforans serpiginosa: description of two cases and review of the literature.

Long term D-penicillamine (DPA) therapy to treat Wilson disease can induce elastosis perforans serpiginosa (EPS), a very rare degenerative skin disease characterized by a transepidermal elimination of elastic fiber aggregates. The iatrogenous disease depends on DPA capacity to chelate copper and cause its depletion. Lysyl-oxidase is a copper dependent enzyme crucial to the dermal elastic fiber cross-linking, which is strongly affected by DPA copper depletion. Direct binding of the drug to collagen precursors also affects elastic fiber assemblage and maturation. The abnormal elastin accumulates into the middle dermis and produces a characteristic bramble brush or "lumpy-bumpy" appearance. In this way it acts as a foreign body and is progressively extruded through the epidermis. Clinically, the disease presents with multiple firm keratotic papules and nodules arranged in annular plaques over the neck, axillae, antecubital fossae, and forearms. The rarity of the disease frequently causes misdiagnoses and the process continues unabated causing concerns about systemic elastopathy.

Bullous skin eruption in an HIV patient during antiretroviral drugs therapy.

Dermo-epidermal blistering is an uncommon presentation of adverse drug reactions. Several drugs are associated to such eruptions, but review of current knowledge does not list antiretroviral drugs. A 37-year-old Caucasian HIV-positive woman presented with a 6-week history of diffuse annular blistering affecting the trunk and limbs. Lesions appeared both on erythematous and normal-appearing skin. The patient was in treatment with antiretroviral (lamivudine + didanosine + nelfinavir) for 2 years. A history of previous adverse reactions to betalactams, nonsteroidal anti-inflammatory drugs, and a nevirapine-induced hepatitis was also referred. Histopathology showed a dermo-epidermal blister; direct immunofluorescence was positive for IgG, C3c at the basement membrane zone; enzyme-linked immunosorbent assay was positive for BP180 antigen. Oral prednisone 1 mg/kg daily for 20 days led to poor improvement. Discontinuation of the antiretrovirals was followed by a rapid healing. Blisters reappeared at first re-introduction essay 1 month later. Awareness of iatrogenic dermo-epidermal blistering is necessary to suspect the diagnosis and avoid long-term immunosuppressant treatment. Complete spontaneous recovery after withdrawal of the responsible drug and relapse at rechallenge are the main criteria for the diagnosis. Factors related to the state of the HIV infection, and/or immunodeficiency may have contributed in precipitating the reaction in the present authors' case.

Pityriasis rosea-like adverse reaction: review of the literature and experience of an Italian drug-surveillance center.

Pityriasis rosea is a common, acute eruption of uncertain etiology. A rash very similar to this idiopathic disease is also attributed to several drugs, and recovery, which depends on withdrawal of the responsible drug, can be delayed by its late identification. A prospective study to record all cases of adverse cutaneous reactions presenting with pityriasis rosea like manifestations was conducted at the center for drug-surveillance of the dermatology department of Cagliari University. We developed an intensive surveillance program from June 2002 to May 2005, adopting the WHO Collaborating center for Drug Monitoring causality assessment criteria and algorithm. Eight cases, six male and two female, were studied in a 3-year period. None had previously suffered from drug intolerance or allergy. Clinical manifestations were very similar to pityriasis rosea. Responsible drugs were mainly angiotensin-converting enzyme inhibitors, alone or in combination with hydrochlorothiazide, followed by one case each for hydrochlorothiazide plus sartan, allopurinol, nimesulide, acetyl salicylic acid. Recovery was obtained in all cases with drug withdrawal. Final causality assessment was probable for all eruptions. Frequency of drug pityriasis rosea-like eruptions is probably underreported. The mildness of the eruption, mimicking a very common and self-limiting disease does not prompt physicians to verify the use of medications until persistence, severity of lesions and itching require re-evaluation of the original diagnosis.

Cutaneous Crohn disease in a child.

Cutaneous Crohn disease refers to granulomatous skin manifestations not contiguous with gastrointestinal affected areas. It is a very rare condition, especially in children, and is easily misdiagnosed when characteristic gastrointestinal symptoms are absent. We report a 10-year-old Caucasian girl with a 6-month history of erythematous, firm tumescence of the left labium majus pudendi and moist vegetations circumscribing the anal ostium. Histologic analysis of skin biopsy specimens from both types of lesions showed a characteristic granulomatous noncaseating infiltrate throughout the dermis. Endoscopic examination and a colon biopsy specimen showed chronic granulomatous inflammation consistent with Crohn disease. Treatment with prednisolone 20 mg daily, metronidazole 250 mg three times daily, topical corticosteroids, and mupirocin ointment produced marked improvement of the vulvar edema, whereas the perianal lesion had a relapsing course. Early recognition of extra-intestinal manifestations of Crohn disease, which are extremely rare in children, may be difficult. Coexistence of contiguous and noncontiguous lesions, representing the very first signs of the disease, are further peculiarities in our patient.

Adverse cutaneous reactions to selective cyclooxygenase 2 inhibitors: experience of an Italian drug-surveillance center.

BACKGROUND: Selective cyclooxygenase (COX) 2 nonsteroidal anti-inflammatory drugs (NSAIDs) have been associated with a general lower incidence of side effects compared with nonselective NSAIDs. Postmarketing information has highlighted the need to reassess the risk evaluation for specific organs, including the skin. OBJECTIVE: A prospective databank to record all cases of adverse cutaneous reactions associated with the use of COX inhibitors was conducted at the Centre for Drug Surveillance of the Dermatology Department of Cagliari University. MATERIAL AND METHODS: An intensive surveillance program from November 2000 to October 2004, adopting the World Health Organization Collaborating Centre for Drug Monitoring causality assessment criteria and algorithm. RESULTS: Seventeen cases, 4 male and 13 female, were studied. None had previously presented any drug intolerance or allergy. Clinical manifestations were mainly maculopapular exanthema followed by urticaria-angioedema. A severe case of leukocytoclastic vasculitis was also observed. Responsible drugs were celecoxib (13 cases; 76%), rofecoxib (3 cases; 18%), and etoricoxib (1 case; 6%). All cases recovered with drug withdrawal. Causality was probable for all eruptions, except for the fixed drug eruption, for which causality was certain. DISCUSSION: Although most cases were associated with celecoxib, the observation of severe eruptions owing to rofecoxib and etoricoxib in this prospective study is consistent with a class effect of COX inhibitors on the skin, which merits further studies to explain the fine underlying mechanisms.

Kerion Celsi in a newborn due to Microsporum canis.

The present study deals with a case of a 40-day-old girl with kerion Celsi caused by Microsporum canis. The source of the infection were the parents who presented tinea corporis caused by M. canis. Systemic treatment was carried out with terbinafine and complete recovery was achieved. Tinea capitis is unusual in children during their first year of life and its evolution towards kerion is very rare in newborns.