C. Pernet

Telaprevir-Induced Acquired Perforating Dermatosis

Discussion | The term “saxophone penis” generally describes a physical examination finding of pronounced curvature and deformity of the penis along its longitudinal axis.1 Another synonym is “ram horn penis.”1 The exact mechanism of this deformity is unknown and may vary depending on acute or chronic occurrence. One proposed explanation describes contraction, and in some cases fibrosis, of the connective tissue on the dorsal side, creating a dependent ventral side, which has richer vascularity and can lead to edema and exaggerated dorsal curvature.1 While there are many potential causes of saxophone penis findings, they are generally limited to conditions affecting penile lymphatics or vessels. Infectious causes include lymphogranuloma venereum,1 S aureus,2 and mycobacterial infections.3 Other potential causes may include primary lymphedema3 and, theoretically, trauma secondary to penile fracture. Although our patient had superficial infection with S aureus, his dramatic improvement despite lack of antimicrobial treatment would support an inflammatory reaction to imiquimod as opposed to a primary bacterial infection. Imiquimod, 5%, cream is a topical immunologic therapy approved for the treatment of external genital warts, superficial basal cell carcinomas, and actinic keratosis. A wide spectrum of cutaneous adverse effects have been associated with topical imiquimod through an increase in TH1 cytokines, including hypopigmentation and vitiligo,4 lichen planopilaris,4 lupus erythematosus–like reactions,4 pemphiguslike skin lesions,5 urticaria, and angioedema.6 Before initiating imiquimod therapy, physicians should thoroughly counsel patients about the potential adverse effects and should provide specific guidelines as to when to contact the prescribing physician. As with our patient, the use of imiquimod resulting in saxophone penis deformity may cause considerable physical discomfort and emotional distress and in some cases may be functionally incapacitating if left untreated.

Somatostatin-producing endocrine tumour of the duodenum associated with type 1 neurofibromatosis.

© 2010 The Authors. doi: 10.2340/00015555-0844 Journal Compilation

Acral acquired cutis laxa associated with IgA multiple myeloma, joint hyperlaxity and urticarial neutrophilic dermatosis

© 2014 The Authors. doi: 10.2340/00015555-1846 Journal Compilation

Erosive pustular dermatosis of the scalp developing after a burn from a hair-bleaching procedure

Hair-styling procedures can produce chemical and/or thermal burns to the scalp. We describe a case of erosive pustular dermatosis (EPD) of the scalp related to a burn caused by a hair-bleaching procedure, which is the first such case reported, to our knowledge. A 25-year-old woman presented with an 18-month history of superficial ulceration on her scalp after a hair highlighting procedure at a hairdressing salon. The patient had undergone highlighting and dyeing of her hair on several previous occasions without any problem, and her medical history was not relevant. During the bleaching procedure, each lock of hair had been coated with the bleaching product containing alkaline persulfates, then wrapped with aluminum foil and heated with a hairdryer to speed up the process. A few minutes after the end of the process, the patient experienced a stinging and burning pain on her scalp localized to the region, where the chemical had been applied and heated. During the next few days, an oval-shaped ulcer developed on the vertex of the patient’s scalp, which did not heal in spite of daily hydrocellular dressings, topical fusidic acid cream and amoxicillin treatment for several weeks as prescribed by a general practitioner. On physical examination, ulcerations with peripheral erosions and crusts were seen on the vertex of the patient’s scalp (Fig. 1). Surface swabs were taken, and microbiological studies were all negative other than a heavy growth of methicillin-sensitive Staphylococcus aureus.

Necrolytic acral erythema following hepatitis B vaccination.

predominates, whereas more children report a lack of awareness of this urge. Interestingly, in a case series by RodriguesBarata et al. their patient experienced scalp hair regrowth but she continued to have eyebrow and eyelash loss after receiving NAC. This could suggest that the benefits of NAC may also be incomplete or only partial. Regarding the dosing regimen for NAC in trichotillomania, the evidence is sparse. Relatively small randomized controlled trials have titrated the dose from 600 to 2400 mg daily over 12 weeks. In the study of Grant et al., none of their 25 adult participants experienced any side-effects. In the paediatric cohort of Bloch et al., the only adverse effects above the placebo group included a rash (5%), depression (5%) and difficulty in swallowing the tablets (10%). Anaphylactoid reactions seen with intravenous NAC were not observed. Dean et al. report that seizures in humans and pulmonary hypertension in animal studies have occurred after very high doses. The optimal duration of treatment is still unknown, and cross-specialty evidence does not describe its use beyond 6 months. Our patient has been on NAC 1200 mg daily for 8 months without ill effect and has experienced a complete and sustained therapeutic benefit. Benefits of NAC have been reported in studies on schizophrenia, autism, cocaine abuse, nicotine addiction, gambling and nail biting. We postulate that primary idiopathic habitual conditions like skin picking, scratching and rubbing may also involve a central pathway and could warrant a trial of NAC. From published studies, NAC seems to be a relatively safe medication and is widely available. Therefore, we look forward to further trials on the use of NAC in compulsive psychocutaneous disorders.

Solitary sclerotic fibroma of the skin: a possible clue for Cowden syndrome

ejd.2012.1655 Auteur(s) : Charlotte Pernet1,2, Luc Durand2, Didier Bessis1, Bernard Guillot1, Olivier Dereure1 o-dereure@chu-montpellier.fr 1 Department of Dermatology 2 Laboratory of Pathology University of Montpellier I, Hopital Saint-Eloi, 80 avenue Augustin Fliche, 34295 Montpellier Cedex 5, France Sclerotic fibroma (SF) or storiform collagenoma of the skin is a rare subset of dermal fibroma that may occur either as a solitary sporadic nodule in healthy individuals, or as solitary or multiple nodule(s) [...]

Dermatological manifestations in cardiofaciocutaneous syndrome: a prospective multicentric study of 45 mutation-positive patients

Data on dermatological manifestations of cardiofaciocutaneous syndrome (CFCS) remain heterogeneous and almost without expert dermatological classification.

Primary leptomeningeal melanocytic tumour with a plaque-like blue nevus in a patient with ocular albinism

A 17-year-old girl displayed symptoms of intra-cranial hypertension revealed by chronic diffuse cephalalgia. She underwent surgery for strabismus in childhood and had clinical evidence of ocular albinism (OA), characterized by a partially transilluminated iris at eye examination, and a heterogeneous retinal pigmentation at fundoscopy. This condition was also diagnosed in her father and sister. The diagnosis was confirmed by GPR143 (OA1) sequencing in blood leucocytes, which showed a germline mutation [...]

‘Cobblestone’ papillomatous linear papules of the upper lip: a new sign of Costello syndrome

the potential for high cure rates and tissue preservation, which is especially important for minimizing deformity in this important anatomical area. To date, six cases have been treated with MMS (studies 20–22, 25, 26 and 29 in Table 1). To ensure negative tumour margins and spare as much normal tissue as possible, MMS was felt to be the treatment of choice for our patient. Patients should be followed up regularly postoperatively in view of the greater potential for metastasis than with BCC at other anatomical sites. Although NAC BCC is rare, it is important to recognize the potentially aggressive nature of these tumours if they are not managed appropriately. Once the diagnosis is made, MMS should be strongly considered as a treatment option for tumours at this high-risk location as a tissue-sparing measure to minimize deformity of this important anatomical area and to minimize the risk of recurrence.

Érythème nécrolytique acral

faveur de la responsabilité de cet inh-mTOR, d’autant qu’il a déjà été associé à l’apparition d’œdèmes bilatéraux (plus rarement unilatéraux) des membres inférieurs, souvent plurifactoriels, chez des malades greffés. Huit cas de lymphœdèmes acquis dont 2 des membres supérieurs, confirmés par lymphoscintigraphie, ont étés rapportés. Dans un cas l’arrêt de la rapamycine a permis la régression de l’œdème. Un seul autre cas avec atteinte du sein, régressant après l’arrêt de l’inh-mTOR, a été publié. Ces lymphœdèmes acquis, dont l’incidence est faible, sont très probablement liés à l’action anti-angiogénique des inh-mTOR, qui inhibent la lymphangiogénèse. Cet effet est d’ailleurs mis à profit pour réduire la prolifération lymphatique dans la lymphangioleïomyomatose. Conclusion.— Les inh-mTOR favorisent l’apparition de lymphœdèmes. Il est utile de les reconnaître à un stade précoce, car la substitution du traitement par une autre classe thérapeutique permet alors leur régression. La régression est moins probable à un stade avancé. Déclaration d’intérêt.— Aucun. Iconographie disponible sur CD et Internet.