Anna Luisa Pinna

Cutaneous Crohn Disease in a Child

Abstract:  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.

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.

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.

Bullous skin eruption in an HIV patient during antiretroviral drugs therapy

ABSTRACT:  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.

Treatment of Sweet's syndrome in pregnancy

Pregnancy‐associated Sweets 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.

Successful treatment of erythrodermic pityriasis rubra pilaris with acitretin

Dear Editor, Pityriasis rubra pilaris (PRP) or Devergie’s disease is a rare papulosquamous disorder of unknown etiology, with chronic course and often deceiving treatment (Klein, Landthaler, & Karrer, 2010). Presentation with a rapidly evolving erythroderma in adult type 1 form is a dramatic condition, whose differential diagnosis includes psoriasis, atopic or contact dermatitis, drug reactions, and cutaneous T cell lymphomas (Mistry, Gupta, Alavi, & Sibbald, 2015). Skin biopsy might be nonspecific, with consistent overlapping with psoriasis. We report, with the consent of the patient, an emblematic case, with clinical–histological clues, and brilliant response to acitretin to highlight the role of the dermatologist in early detection and effective management, supporting internists and pathologists not familiar with such rare conditions. The 51-year-old Caucasian man was hospitalized in the internal medicine ward of our University Hospital for a sudden erythematousedematous eruption, with ectropion, severe itching, and generalized malaise. An early skin biopsy was not diagnostic. The past medical history was unremarkable, except for a recently prescribed fluconazole treatment for a fungal infection. Drug was withdrawn and intravenous methylprednisolone treatment started (40 mg daily), but the patient continued worsening to erythroderma. Dermatologic consulting put in evidence peculiar roundish islands of spared skin in the context of a generalized erythematous–edematous scaling eruption, as well as an orange hue palmoplantar keratoderma (Figure 1). A collegial revision of the histologic samples showed a psoriasiform dermatitis (Figure 2), with mild irregular acanthosis, areas of orthokeratosis, focal

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.

Langerhans cell histiocytosis in an adult

The term Langerhans cell histiocytosis (LCH) includes a group of diseases of different clinical-evolutionary expressions but with the same histological and ultrastructural patterns, characterized by an abnormal reactive proliferation of these cells of the monocyte-macrophage system in different organs and tissues 11 ]. Known as histiocytosis X, which included Hand-Schuller-Chrlstian disease, Letterer-Siwe disease, eosinophilic granuloma of the bone and autoregressive congenital reticulohistiocytosis of Hashimoto-Pritzker, this group of diseases has recently been classified in localized and disseminated forms by the Histiocyte Society [2|. The diseases are commonly considered pediatric diseases and are rarely present in adults 13-5].