Herberto Bettencourt

Anti-TNF-alpha induced psoriasiform eruptions with severe scalp involvement and alopecia: report of five cases and review of the literature.

We describe 5 cases of anti-tumor necrosis factor-alpha (anti-TNF-α) induced psoriasiform eruptions with severe scalp involvement inducing inflammatory alopecia and review the literature on this subject. All our 5 patients were provided topical therapy, with good results in only 1 case. The remaining 4 were provided systemic therapy (methotrexate ± cyclosporine): 3 concomitantly suspended the anti-TNF-α treatment (2 are currently clear/almost clear but 1 has so far only observed mild improvement) and 1 switched anti-TNF-α (recurrent flare-ups of the disease continue). So far, no patient has developed scarring alopecia. To our knowledge, a total of 15 cases of anti-TNF-α induced psoriatic alopecia have been described. Anti-TNF-α was discontinued in 9 of the 15 patients and systemic therapy was provided to 9 of the 15 patients. Nonetheless, 2 patients developed scarring alopecia. We conclude that in anti-TNF-α induced psoriasiform eruptions some patients may respond to topical treatment, however in cases of severe scalp involvement anti-TNF-α suspension and systemic treatment should be considered in order to avoid scarring alopecia.

Acute hemorrhagic edema of childhood after H1N1 immunization

Acute hemorrhagic edema (AHE) is an uncommon self-limited disorder affecting young children triggered by infection, drugs, or immunization. A 2-year-old boy was observed due to sudden onset of painful and edematous purpuric papular and plaque lesions of the face and upper extremities that started 2 weeks after H1N1 immunization. The patient also developed exuberant edema on the face and dorsum of the hands. Complete blood count, biochemistry, and urinalysis results were normal. Histopathological examination revealed perivascular and periadnexial lymphocytic infiltrate with neutrophils and eosinophils, and leukocytoclastic vasculitis. Blood PCR technique was negative to several viruses, namely adenovirus, cytomegalovirus, Epstein Barr, enterovirus, HHV6, parvovirus B19, and H1N1. Symptomatic treatment and parents reassurance was promptly provided. However, new lesions continued to develop and in this setting systemic corticosteroid was prescribed. Complete clinical resolution was achieved within 2 weeks and no relapse was observed. The temporal relationship with H1N1 immunization, absence of previous drug intake, as well as exclusion of viral infections led the authors to propose that H1N1 vaccine was the predisposing factor in AHE development in our patient. To our best knowledge, this is the first reported association between AHE and H1N1 immunization.

Primary Langerhans cell histiocytosis of the vulva: acitretin as a glucocorticoid-sparing agent

Serra Kayac etin, MD Ankara Numune Education and Research Hospital Pathology Clinic Ankara Turkey E-mail: seraycakmak@gmail.com References 1 Speeckaert MM, Speeckaert R, Lambert J, et al. Acute generalized exanthematous pustulosis: an overview of the clinical, immunological and diagnostic concepts. Eur J Dermatol 2010; 20: 425–433. 2 Guevara-Gutierrez E, Uribe-Jimenez E, Diaz-Canchola M, et al. Acute generalized exanthematous pustulosis: report of 12 cases and literature review. Int J Dermatol 2009; 48: 253–258. 3 Mayo-Pamp ın E, Fl orez A, Feal C, et al. Acute generalized exanthematous pustulosis due to pseudoephedrine with positive patch test. Acta Derm Venereol 2006; 86: 542– 543. 4 Treudler R, Grunewald S, Gebhardt C, et al. Prolonged course of acute generalized exanthematous pustulosis with liver involvement due to sensitization to amoxicillin and paracetamol. Acta Derm Venereol 2009; 89: 314–315. 5 Sim HS, Seol JE, Chun JS, et al. Acute localized exanthematous pustulosis on the face. Ann Dermatol 2001; 23: S368–S370. 6 Betto P, Germi L, Bonoldi E, et al. Acute localized exanthematous pustulosis (ALEP) caused by amoxicillinclavulanic acid. Int J Dermatol 2008; 47: 295–296.

Acute generalized exanthematous pustulosis to amoxicillin associated with parvovirus B19 reactivation

We report the case of a 22-year-old male patient with 2 episodes, 4 months apart, of acute generalized exanthematous pustulosis (AGEP) associated with oral intake of amoxicillin and simultaneous reactivation of parvovirus B19 infection proven by positive polymerase chain reaction test in the skin fragment and blood sample and elevation of the IgG antibodies titer. To our knowledge, this is the first report of AGEP resulting from the interaction between drug hypersensitivity and the reactivation of parvovirus B19. A combination of an immunological reaction to the drug and virus infection could be responsible for the clinical picture.

Necrobiotic xanthogranuloma with giant cell hepatitis, successfully treated with intravenous immunoglobulins

A 44-year-old Caucasian woman with a year history of IgG kappa light-chain monoclonal gammopathy and giant cell hepatitis was referred to our department due to an arm lesion evolving for 2 months. On physical examination, an asymptomatic, erythematous-violaceous indurated plaque on the extensor surface of the left arm (Fig. 1A) and multiple yellowish, nontender, edematous, confluent papules on the periorbital area (Fig. 1B) were observed. The patient complained of recurrent red eye episodes and myalgia. Laboratory tests revealed elevation of transaminases and complement consumption; protein electrophoresis showed a diffuse increase in gamma globulins and a peak of free kappa light-chains; echocardiography was normal. Biopsies of the arm and periorbital lesions showed similar features, namely an infiltrate of histiocytes, including some multinucleated giant and foamy cells, extending into the hypodermis, foci of necrobiosis, cholesterol clefts, and a moderate lymphocytic infiltrate with occasional plasma cells (Fig. 1C–E). The diagnosis of necrobiotic xanthogranuloma (NXG) was established. Liver biopsy revealed hepatocytes with giant cell transformation, microvesicular steatosis, and intralobular necroinflammatory lesions. A bone marrow biopsy excluded a malignancy. Oral prednisolone (1 mg/kg/day) was started and later tapered off and replaced by oral budesonide (9 mg/day), yielding an initial reduction in transaminases values, but followed by another raising (ALT/AST 159/466, normal range <31 U/I). The cutaneous picture worsened increasingly with periorbital necrosis and ulceration (Fig. 2A). As the patient wanted to get pregnant, intravenous immunoglobulins (IVIg), 0.5 g/kg/day (4 consecutive days every 4 weeks) were administered concomitantly with oral budesonide. After three cycles of IVIg, the skin lesions improved remarkably (Fig. 2B) and transaminase values normalized. When 15 cycles of IVIg have been completed, the interval was extended to 6 weeks, maintaining the outcome. An attempt to reduce budesonide to 6 mg/day was followed by elevation of transaminases, leading to a return to the original dose. The serum free kappa light-chains remained Address correspondence and reprint requests to: Ana Filipa Bastos Pedrosa, MD, Department of Dermatology and Venereology, Centro Hospitalar São João EPE, Alameda Prof. Hernani Monteiro, Porto 4200-319, Portugal, or email: anabastospedrosa@gmail.com.

XANTOGRANULOMA NECROBIÓTICO ASSOCIADO A HEPATITE DE CÉLULAS GIGANTES

We describe a 44-year-old woman with recurrent episodes of red eye, myalgias, hypercholesterolemia, hypocomplementemia, monoclonal gammopathy and giant cell hepatitis that was observed due to erythematoviola- ceous plaque on the right arm. Skin biopsy revealed necrobiotic xanthogranuloma. This case illustrates the importance of accurate characterization of skin lesions in the setting of a systemic disease with difficult approach and highlights the second reported case of necrobiotic xanthogranuloma associated with giant cell hepatitis. KEYWORDS – Necrobiotic xanthogranuloma; Giant cells; Hepatitis; Paraproteinemias.

INFECÇÃO CUTÂNEA POR MYCOBACTERIUM HAEMOPHILUM EM DOENTE IMUNODEPRIMIDO

Mycobacterium haemophilum skin infection is a rare disease with a difficult diagnosis and a challenging treatment. We report the case of a patient on chronic corticotherapy for myositis with nodules on the lower limbs in which Mycobacterium haemophilum was identified by PCR technique. This case emphasizes the need for a high index of suspicion for the diagnosis. In fact, this infection can currently be underdiagnosed due to the special requirements for culture. It should be considered in patients with chronic granulomatous processes in combination with negative my- cobacteriological examination. KEYWORDS – Mycobacterium haemophilum; Immunocompromised host; Mycobacterium infections; Skin diseases, bacterial.Mycobacterium haemophilum skin infection is a rare disease with a difficult diagnosis and a challenging treatment. We report the case of a patient on chronic corticotherapy for myositis with nodules on the lower limbs in which Mycobacterium haemophilum was identified by PCR technique. This case emphasizes the need for a high index of suspicion for the diagnosis. In fact, this infection can currently be underdiagnosed due to the special requirements for culture. It should be considered in patients with chronic granulomatous processes in combination with negative my- cobacteriological examination. KEYWORDS – Mycobacterium haemophilum; Immunocompromised host; Mycobacterium infections; Skin diseases, bacterial.

DERMATOSES EM GRÁVIDAS E PUÉRPERAS OBSERVADAS NUM SERVIÇO DE URGÊNCIA – AVALIAÇÃO DE 86 CASOS

Introduction: Besides morbidity related to skin lesions and pruritus, dermatologic disorders during preg- nancy cause psychological concern and some of them carry a fetal risk. Objective: Assessment of the type, frequency and clinical characteristics of the dermatoses seen in pregnant and postpartum women seeking support from the Emer- gency Department. Methods: Retrospective study of pregnant and postpartum women observed by dermatologists at the Emergency Department between September 2006 and September 2010. Results: The study included 79 pregnant and 7 postpartum women, with a median age of 33 years. Specific dermatoses of pregnancy were diagnosed in 42 patients (48.8%). Polymorphic eruption of pregnancy was the most frequent specific dermatosis (n=16), followed by eczema in pregnancy (n=12), prurigo of pregnancy (n=8), pemphigoid gestationis (n=5), and pruritic foliculitis of pregnancy (n=1). Other dermatoses were diagnosed in 44 patients (51.2%), including: pityriasis rosea (n=11), infections and infestations, dyshidrotic eczema, contact dermatitis, lupus erythematosus and acute generalized exanthe- matous pustulosis. In the cases with an atypical presentation, the biopsy helped in the characterization of the disease. The fetal outcome was assessed in 43 cases, only 3 cases of preterm delivery being registered. Discussion: The onset of the specific dermatoses of pregnancy was more likely to occur in the late pregnancy, especially the third trimester. In pregnant women with exuberant or atypical skin lesions, laboratory and histological study are indispensable for the specific diagnosis, allowing appropriate therapeutic approach and fetal risk assessment. KEYWORDS – Skin Diseases; Pregnancy Complications; Pruritus.