M. Alegre

Brooke-spiegler Syndrome Variant Segregation of Tumor Types with Mixed Differentiation in Two Generations

Brooke-Spiegler syndrome (BSS) is an autosomal dominantly inherited disease characterized by the development of multiple trichoepitheliomas and cylindromas. Other lesions have been reported to occur in patients with BSS, including parotid basal cell adenomas, milia, organoid nevi, basal cell carcinomas, and spiradenomas. Spiradenomas and cylindromas have so many features in common that they have been regarded as polar extremes belonging to a spectrum of cutaneous adnexal neoplasms. We report on a 61-year-old woman with multiple spiradenomas on the scalp and periauricular areas and her 28-year-old daughter, with multiple facial trichoepitheliomas. Occasional features of pilar and ductal differentiation were found in tissue specimens of tumors from the mother and daughter, respectively. Co-existence of multiple spiradenomas and trichoepitheliomas and segregation of the predominant type of tumor in different members of a family with BSS have been reported occasionally, but never, to our knowledge, in combination. The presence of mixed differentiation in tumor specimens from both patients provides additional evidence in support of the folliculosebaceous apocrine unit (FSAU) hypothesis. Mutations in genes regulating proliferation and differentiation of putative stem cells of the FSAU would give rise to different combinations of adnexal skin tumors as well as to other neoplasms.

Successful Treatment of Eccrine Angiomatous Hamartoma With Botulinum Toxin

A 12-year-old girl was referred to our department for evaluation of a 6-cm erythematous, brownish, indurated plaque that has been present on the sacral area since birth (Figure 1). The gluteal cleft was not deviated, and the lesion was not tender to palpation. The patient complained of profuse sweating that would drench her clothes. These symptoms were distressing and unrelated to emotional stress or physical exercise. A magnetic resonance imaging study revealed no lumbosacral spine abnormalities or cord tethering. A skin biopsy specimen demonstrated benign fibrovascular proliferation with a hyperplasia of the eccrine sweat glands in the dermis (Figure 2), which is consistent with an eccrine angiomatous hamartoma (EAH). The hamartomatous component was 2 mm below the epidermis and extended 4 mm into the dermis. In view of the symptoms, surgical excision was proposed, but the patient and her family preferred a different therapeutic approach.

ERYTHEMA MULTIFORME-LIKE REACTION FOLLOWING DIPHENCYPRONE TREATMENT OF PLANE WARTS

A 27-year-old pharmacy graduate consulted her private dermatologist for treatment of flat warts on her chin and left malar area. She was sensitized with diphencyprone (DCP), 2% in acetone, applied on her left arm under a perforated bandage strip for 48 hours, which caused the appearance of several blisters on an erythematous plaque corresponding to the site of occlusion. She applied DCP 0.001% once daily on the area corresponding to the flat warts, immediately after uncovering the sensitization area. After 3 days of treatment, there was a flare-up of the lesion corresponding to the sensitization site, followed several hours later by the appearance of a tender and pruritic rash. The patient presented to our emergency outpatient dermatology clinic because of progression of the rash despite discontinuation of DCP applications and treatment with oral prednisone, 30 mg/day for 3 days, followed by the increased dosage of 50 mg/day for 12 days. Her medical history was otherwise unremarkable. On physical examination there was an erythematous rash, symmetrically distributed on the patients face, trunk, and upper limbs (Fig. 1). The individual lesions were red macules, some of them with dark red central areas and target-like in appearance, that coalesced to form patches with polycyclic borders and some peripheral desquamation. There were also scattered macules on her palms and a subtle reticulation of the oral mucosa. Results of complete Coulter blood counts and of a standard serum biochemistry profile were within normal limits. Histopathologic examination of a 4-mm punch biopsy specimen obtained from a lesion on the patients right arm revealed necrosis of occasional single keratinocytes in the epidermis, some degree of dermal edema, and a moderate to dense perivascular inflammatory infiltrate, composed mainly of lymphomononuclear cells, with focal exocytosis (Fig. 2). The rash eventually cleared with minimal residual hyperpigmentation after 10 additional days of treatment with

Treatment of Meralgia paraesthetica with Topical Capsaicin

Lluis Puig, Department of Dermatology, Hospital de la Santa Creu i Sant Pau, Avda. San Antonio M. Claret 167, E-08025 Barcelona (Spain) Meralgia paraesthetica is an uncommon neurologic disorder usually caused by compression of the lateral femoral cutaneous nerve. This nerve leaves the pelvic cavity at the anterosuperior iliac spine below the inguinal ligament and superficial to the sar-torius muscle. In its course it is vulnerable to entrapment, generally related with obesity, pregnancy or unknown causes [1]. Meralgia paraesthetica has also been described to appear as a result of neuromas [2], malignant tumours of the psoas muscle [3], metastatic carcinomas in the second lumbar vertebra [4] or following coronary bypass surgery [5], due to compression arising from prolonged supine position on the operating table. Meralgia paraesthetica is clinically characterized by a disturbed sensation at the an-terolateral side of the thigh. Patients can complain of pain, numbness, itching ordysaesthe-sia, and the perception of pinprick and touch is often diminished or lost. In some cases they Lluís Puig Department of Dermatology Hospital de la Santa Creu i Sant Pau Avda. San Antonio M. Claret 167 E-08025 Barcelona (Spain) refer a burning pain that can be unbearable. The femoral cutaneous nerve is only sensitive so the motor function remains uninvolved and the reflexes are normal. Electrophysio-logic tests and somatosensory evoked potentials can be useful as diagnostic procedures [6]. Many treatments have been advocated for meralgia paraesthetica [2]. Analgesia can be provided by nerve block or local infiltrations. Surgical procedures include neurolysis, transposition or decompression of the nerve and mobilization of the supra-inguinal ligament. The possible efficacy of topical capsaicin in the treatment of meralgia paraesthetica has not been previously reported, to our knowledge. A 54-year-old man was referred for evaluation of dysaesthesia in the anterolat-eral right thigh. His medical history included partial epileptic crisis due to a congenital arachnoid cyst in the left parietotemporo-occipital area which had been treated with carbamazepine for the last 3 years. He also suffered from aquagenic pruritus which was well controlled with the administration of hydroxyzine before showers. He complained of paraesthesias, numbness, itching and burning sensations localized on the anterolateral aspect of the right thigh of 2 months’ evolution. Physical examination did not reveal any cutaneous abnormality, and the results of complete blood cell counts and biochemical parameters were normal. The sensory response of both femorocutaneous nerves could not be evocated on electrophys-iological examination. The patient was instructed to apply topical 0.025% capsaicin cream five times daily on the lateral aspect of

Bilobed flap for full-thickness nasal defect: a common flap for an uncommon indication.

A 60-year-old woman was referred to the Mohs surgery unit of the Sant Pau Hospital in March 2000 for the treatment of a tumor of the alar rim and tip of the nose. Histopathological examination showed a sclerodermiform basal cell carcinoma. Four stages of Mohs surgery were needed to excise the entire tumor, which involved part of the nasal cartilage and nasal mucosa, resulting in a fullthickness defect (Figure 1). How would you proceed in this case?

Histopathological changes of primary HIV infection. Description of three cases and review of the literature

The histopathological changes observed in the cutaneous rash of three patients who suffered the acute phase of HFV infection are described. In all three patients a perivascular and interstitial inflammatory infiltrate was present in the upper and mid‐reticular dermis. In one biopsy isolated areas of epidermal necrosis were observed and in the two other biopsies a perifollicular inflammatory infiltrate was detected with perforation in one case. Furthermore, a periductal infiltrate was observed in one of these biopsies.

Crusted (Norwegian) scabies: an under-recognized infestation characterized by an atypical presentation and delayed diagnosis

References 1 Bon-Mardion M, Poulalhon N, Balme B, Thomas L. Ungual seborrheic keratosis. J Eur Acad Dermatol Venereol 2010; 24: 1102–1104. 2 Baran R, Perrin C. Linear melanonychia due to subungual keratosis of the nail bed: a report of two cases. Br J Dermatol 1999; 140: 730–733. 3 Spaccarelli N, Wanat KA, Miller CJ, Rubin AI. Hypopigmented onychocytic matricoma as a clinical mimic of onychomatricoma: clinical, intraoperative and histopathologic correlations. J Cutan Pathol 2013; 40: 591–594. 4 Thomas L, Vaudaine M, Wortsman X, Jemec GBE, Drap e JL. Imaging the Nail Unit. In Baran R, de Berker DAR, Holzberg M, Thomas L, eds. Baran and Dawber’s Diseases of the Nails and Their Management, 4th edn. Hoboken, NJ: Wiley-Blackwell, 2012: 101–182. 5 Dalle S, Depape L, Phan A, Balme B, Ronger-Savle S, Thomas L. Squamous cell carcinoma of the nail apparatus: clinicopathological study of 35 cases. Br J Dermatol 2007; 156: 871–874. 6 Cogrel O, Beylot-Barry M, Doutre MS. Subungual squamous cell carcinoma revealed by longitudinal erythronychia. Ann Dermatol Venereol 2008; 135: 883–885.

Double Mucosal and Myocutaneous Island Flap: A One-Stage Reconstruction for Full-Thickness Lower Eyelid Defect

BACKGROUND Basal cell carcinomas (BCCs) on the lower eyelid are not uncommon, and depending on their histological type, they can be highly aggressive and difficult to eradicate. Numerous techniques have been proposed for the reconstruction of the lower lid margin after surgical excision of the lesion. OBJECTIVE To describe a double‐flap technique consisting of a mucosal and myocutaneous V‐Y advancement flap to repair full‐thickness lower lid margin defects in a one‐stage procedure under local anesthesia. METHODS Over a 12‐year period (1995‐2007), 33 patients with BCC underwent lower eyelid reconstruction. After tumor excision, a triangular mucosal flap with a central pedicle was used to repair the inner layer. A similar triangular‐shaped myocutaneous flap was obtained from the inferior orbicularis oculus muscle and sutured in a V‐Y fashion to build the outer layer. RESULTS All 33 patients achieved satisfactory functional and cosmetic results. Slight scleral show and discrete rounded lower eyelid were the main adverse effects. No additional surgery was needed. CONCLUSION This double V‐Y advancement flap is a simple, useful alternative procedure to close full‐thickness defects in the lower lid margin. Aesthetic and functional outcome is good. The authors have indicated no significant interest with commercial supporters.

Major oral aphtous ulceration in HIV-1 infection: successful response after highly active antiretroviral therapy

126 JEADV 2007, 21, 104–143

Mohs micrographic surgery for basal cell carcinomas: results of a Spanish retrospective study and Kaplan–Meier survival analysis of tumour recurrence.

Mohs micrographic surgery (MMS) is a specialized procedure usually limited to specific indications (e.g. high‐risk basal cell carcinomas [BCCs]).