T.-A. Duong

Systemic involvement of acute generalized exanthematous pustulosis: a retrospective study on 58 patients

Acute generalized exanthematous pustulosis (AGEP) is a severe cutaneous adverse reaction characterized by rash with sterile pustules, high fever and elevated circulating neutrophil counts.

Linear IgA bullous dermatosis: comparison between the drug‐induced and spontaneous forms

Linear IgA bullous dermatosis (LABD) is a rare autoimmune blistering skin disorder characterized by linear deposits of IgA along the dermoepidermal junction, visualized by direct immunofluorescence (DIF). It is usually spontaneous and drug induced.

Histopathology of drug rash with eosinophilia and systemic symptoms syndrome: a morphological and phenotypical study

The histopathological features of drug rash with eosinophilia and systemic symptoms (DRESS) syndrome remain poorly characterized.

Dermatological emergencies: a comparative study of activity in 2000 and 2010

Background and objective  Studies of dermatological emergencies (DE) are few. We evaluated the activity in our DE unit in a 1‐month retrospective study and compared the results with a similar study performed in the same department in 2000.

Drug reaction with eosinophilia and systemic symptoms and severe involvement of digestive tract: description of two cases

large cleaved cells to be of B-cell lineage (CD20 positive) accompanied by large numbers of reactive T cells (CD3 positive). The larger, neoplastic cells expressed bcl-6, but did not react with antibodies to CD5, CD10, bcl-2, cyclin D1 or MUM1. Proliferation activity (Ki67) was relatively high (50% of cells). Histopathological features were diagnostic of PCFCCL. The patient had neither regional nor distant lymphadenopathy and no hepatosplenomegaly. Bone marrow examination and computed tomography of the chest, abdomen and pelvis were negative for systemic lymphoma. She was treated with superficial radiotherapy resulting in complete resolution of plaques and patchy hair regrowth. PCFCCL has a predilection for the head and neck, and scalp involvement is well documented. The unusual finding in our patient was of alopecia. One case of PCFCCL presenting simultaneously with alopecia areata has been described, but to the best of our knowledge PCFCCL presenting with scarring alopecia has not previously been reported. The background of PCOS in our patient and the presence of miniaturized hair follicles in the histology specimen raises the possibility of coexistent androgenetic alopecia. Superficial radiotherapy or surgical excision of localized disease is widely accepted as first-line treatment for PCFCCL and prognosis is excellent with a 5-year survival of over 90%. Chemotherapy, topical 5% imiquimod and the recombinant, chimeric anti-CD20 antibody rituximab have also been used successfully. Disease rarely spreads to extracutaneous sites, but the incidence of local recurrence after treatment is high, with rates quoted between 25% and 68%. Our patient had marked scarring alopecia by the time she presented to dermatology. Regrettably, one of the main disadvantages of radiotherapy in treating this entity, with its characteristic lesions on the head or neck, is the risk of permanent alopecia. Early signs of patchy hair regrowth in our patient have, however, been encouraging. Intralesional rituximab may hold some promise for these patients with the potential to eradicate disease in the scalp with minimal or reduced scarring and alopecia. Its use is not yet widespread and it may be associated with a relatively increased risk of relapse at distant cutaneous sites. In conclusion, we report an unusual case of PCFCCL of the scalp presenting with alopecia. Our patient responded well to local radiotherapy but in view of the possibility of aggravating alopecia in such patients, further evaluation of the newer, potentially hair-preserving treatments may be warranted for first-line treatment of such cases. Importantly, this case also illustrates the value of taking a biopsy in scarring alopecia.

Thyroïdite auto-immune et syndrome d'hypersensibilité médicamenteuse (DRESS) associés à une réactivation virale HHV6

INTRODUCTION DRESS syndrome is a severe adverse drug reaction with visceral involvement. Its physiopathology includes immunological disorders associated with human herpes virus (HHV) reactivation. We report two cases of auto-immune thyroiditis occurring in the context of DRESS syndrome associated with HHV-6 reactivation. OBSERVATIONS Case 1 : A 39-year-old woman presented DRESS syndrome with HHV-6 reactivation, cutaneous, lymph node, hepatic and renal disorders treated with systemic corticosteroids for 10 months. Following discontinuation of the corticosteroids, she developed Gravess disease, which was stabilized with carbimazole and a beta-blocker. CASE 2: A 31-year-old woman was hospitalized for DRESS syndrome with delayed HHV-6 reactivation and severe hepatic involvement. She was successfully treated by topical steroids. Six weeks later, she presented De Quervain thyroiditis associated with moderate relapsing DRESS, which were treated by sodium levothyroxine and topical steroids. DISCUSSION There is currently debate about the implication of viral reactivation, in particular HHV-6, in chronic DRESS, relapse and development of auto-immune diseases. These observations highlight the potential risk of patients developing auto-immune diseases and underline the need for prolonged clinical and laboratory follow-up of patients with DRESS.

Infections cutanées chez les patients atteints de pemphigoïde bulleuse traités par dermocorticoïdes

BACKGROUND Potent topical corticosteroids (TCS), such as clobetasol propionate are more efficacious than systemic corticosteroids in the treatment of bullous pemphigoid (BP) and in reducing the rate of systemic infectious complications. However, TCS can have cutaneous side effects, such as atrophy and purpura. The risk of cutaneous infections due to TCS in BP is known but has never been studied, despite prolonged use of high doses. Since we noted three cases of fatal necrotizing fasciitis (NF) in patients treated with TCS for BP over a one-year period in our institution, we decided to analyse the frequency of cutaneous infections in all BP patients hospitalized in our department. PATIENTS AND METHODS In this retrospective single-centre study, all files of patients presenting BP treated with TCS and hospitalized between April 2008 and April 2009 were reviewed. When the clinical file indicated a cutaneous infectious problem, bacteriological data were requested from the bacteriology laboratory. For each patient, clinical data, history, ongoing treatment, type of cutaneous infectious complication, general symptoms and details of outcome were collected. RESULTS In the 30 files studied, we found ten cutaneous infections in nine patients: minor complication (three cases of impetiginisation), moderate complications (two erysipelas, one lymphangitis with sepsis, one flexor tendon phlegmon with cutaneous fistula), and severe complications with a fatal course (three NF, one of which had involved erysipelas of favourable outcome a few months earlier). These cutaneous complications occurred after various treatment times (ten days to two years) and various dosages of TCS (two-three tubes/day to two tubes every two days). Three of the nine patients with cutaneous infections had diabetes, in particular two of the three patients with FN. In contrast, four of the 21 patients without cutaneous complications had diabetes. Patients with cutaneous infections did not have more extensive BP or receive more TCS than the others. In two of three patients with NF, an immunosuppressant drug (methotrexate or mycophenolate mofetil) had been recently initiated (inferior to one month). The offending organism was Staphylococcus aureus in seven cases (methicillin-resistant in three cases) and Streptococcus A in five cases (three NF, one lymphangitis and one impetiginisation). The outcome was fatal in the three cases of NF but was favourable with local and/or systemic antibiotic therapy in the remaining cases. CONCLUSION In this study, we noted cutaneous superinfection in nine of 30 (30%) patients receiving topical corticosteroids for bullous pemphigoid, among which were three cases of fatal NF due to streptococcus A (10%). The infectious risks associated with TCS must not be neglected, particularly since treated patients are old and fragile, and frequently have multiple well-known risk factors for NF (e.g. extensive lesions, diabetes, etc.). In the event of signs of cutaneous superinfection, especially in cases of diabetes and prolonged TCS treatment, bacteriological analysis should be conducted. Adequate treatment should be initiated without delay, especially in cases of beta-haemolytic streptococcal infection.

Mémoire originalInfections cutanées chez les patients atteints de pemphigoïde bulleuse traités par dermocorticoïdesCutaneous infections in bullous pemphigoid patients treated with topical corticosteroids

BACKGROUND Potent topical corticosteroids (TCS), such as clobetasol propionate are more efficacious than systemic corticosteroids in the treatment of bullous pemphigoid (BP) and in reducing the rate of systemic infectious complications. However, TCS can have cutaneous side effects, such as atrophy and purpura. The risk of cutaneous infections due to TCS in BP is known but has never been studied, despite prolonged use of high doses. Since we noted three cases of fatal necrotizing fasciitis (NF) in patients treated with TCS for BP over a one-year period in our institution, we decided to analyse the frequency of cutaneous infections in all BP patients hospitalized in our department. PATIENTS AND METHODS In this retrospective single-centre study, all files of patients presenting BP treated with TCS and hospitalized between April 2008 and April 2009 were reviewed. When the clinical file indicated a cutaneous infectious problem, bacteriological data were requested from the bacteriology laboratory. For each patient, clinical data, history, ongoing treatment, type of cutaneous infectious complication, general symptoms and details of outcome were collected. RESULTS In the 30 files studied, we found ten cutaneous infections in nine patients: minor complication (three cases of impetiginisation), moderate complications (two erysipelas, one lymphangitis with sepsis, one flexor tendon phlegmon with cutaneous fistula), and severe complications with a fatal course (three NF, one of which had involved erysipelas of favourable outcome a few months earlier). These cutaneous complications occurred after various treatment times (ten days to two years) and various dosages of TCS (two-three tubes/day to two tubes every two days). Three of the nine patients with cutaneous infections had diabetes, in particular two of the three patients with FN. In contrast, four of the 21 patients without cutaneous complications had diabetes. Patients with cutaneous infections did not have more extensive BP or receive more TCS than the others. In two of three patients with NF, an immunosuppressant drug (methotrexate or mycophenolate mofetil) had been recently initiated (inferior to one month). The offending organism was Staphylococcus aureus in seven cases (methicillin-resistant in three cases) and Streptococcus A in five cases (three NF, one lymphangitis and one impetiginisation). The outcome was fatal in the three cases of NF but was favourable with local and/or systemic antibiotic therapy in the remaining cases. CONCLUSION In this study, we noted cutaneous superinfection in nine of 30 (30%) patients receiving topical corticosteroids for bullous pemphigoid, among which were three cases of fatal NF due to streptococcus A (10%). The infectious risks associated with TCS must not be neglected, particularly since treated patients are old and fragile, and frequently have multiple well-known risk factors for NF (e.g. extensive lesions, diabetes, etc.). In the event of signs of cutaneous superinfection, especially in cases of diabetes and prolonged TCS treatment, bacteriological analysis should be conducted. Adequate treatment should be initiated without delay, especially in cases of beta-haemolytic streptococcal infection.

Subcorneal pustular dermatosis triggered by Mycoplasma pneumoniae infection: a rare clinical association

Case Report A man in his early twenties without past history was admitted for a severe pulmonary infection (PI) confirmed by computed tomography. Two days after amoxicillin instatement, he underwent macular exanthema, and amoxicillin was replaced by levofloxacin. Despite amoxicillin discontinuation, the eruption worsened, becoming pustular. Clinical examination evidenced an eruption of 5–10-mm non-follicular pustules, some with pathognomonic hypopion (i.e. pus in the lower half of the lesion) on normal or slightly erythematous skin, mainly on the trunk and sparing mucous membranes (Fig. 1a). The absence of fever at onset of the eruption 2 days after drug intake did not fit a pustular drug eruption such as acute generalized exanthematous pustulosis (AGEP) or EM; moreover, the face and folds were unaffected, pustules exhibiting hypopion were too large for AGEP, and no target lesion or Nikolski sign for EM were observed. Pustule bacterial and viral cultures were negative. Blood count, biochemistry, autoimmune screening and viral serologies (in particular HIV 1 and 2, VHB, VHC) were normal or negative. MP enzyme-linked immunosorbent assay (ELISA) evidenced positive IgM and confirmed MP infection 10 days later with high IgM and IgG titre. Polymerase chain reaction (PCR) for MP on throat and pustule samples was negative. Histopathological examination showed subcorneal unilocular pustules containing neutrophils on a slightly papillomatous epidermis, without necrotic keratinocytes, with perivascular infiltrate of lymphocytes and polymorphonuclear neutrophils (PMNs) and papillary dermal oedema (Fig. 1b). Direct immunofluorescence (IF) was unspecific and indirect IF negative. The patient was diagnosed with SPD in association with MP pneumonitis. Topical corticosteroids provided complete remission in 1 week and the patient was discharged. No relapse occurred within 24 months.

Livedoid and Necrotic Skin Lesions Due to Intra-arterial Buprenorphine Injections Evidenced by Maltese Cross–Shaped Histologic Bodies

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