Ana Filipa Duarte

Nail matrix melanoma in situ: Conservative surgical management

Background: Nail unit melanoma (NUM) is a rare variant of acral lentiginous melanoma. The differential diagnosis is wide but an acquired brown streak in the nail of a fair-skinned person must be considered a potential melanoma. Dermatoscopy helps clinicians to more accurately decide if a nail apparatus biopsy is necessary. Methods: We report the case of a 61-year-old Caucasian woman with melanonychia occupying the central portion of the right thumbnail plate with 1 year of evolution. Dermatoscopy showed a brown pigmentation overlaid by longitudinal irregular lines. An excisional biopsy was performed, and pathological examination revealed melanoma in situ. For safety reasons, the nail unit was totally removed down to the phalangeal bone 3 weeks later, and a full-thickness skin graft taken from the arm was used for reconstruction. Conclusion: NUMs pose a difficult treatment challenge. Wide excision with phalanx amputation is not satisfactory for patients with in situ and early invasive melanoma. Full-thickness skin grafting after total nail unit excision is a simple procedure providing a good functional and cosmetic outcome.

Multiple Eccrine Hidrocystomas – from Diagnosis to Treatment: The Role of Dermatoscopy and Botulinum Toxin

Background: Eccrine hidrocystomas are rarely described benign cystic lesions, mainly presenting in middle-aged women in the centrofacial area and usually associated with a chronic course, seasonal variability and no proved and consistently efficacious treatments. Case Report: We report 2 patients, a 45- and a 56-year-old woman, with multiple facial eccrine hidrocystomas suggested by dermatoscopy, confirmed by histology and treated with botulinum toxin A with excellent results. Conclusion: Our report stresses the role of dermatoscopy in the diagnosis and follow-up of these lesions, as well as the impact of repeated treatments with botulinum toxin A in eccrine hidrocystomas suggesting it as a first-line treatment for multiple eccrine hidrocystomas because it is easy to use and has no risk of scars.

UNILATERAL LATEROTHORACIC EXANTHEM AND PRIMARY EPSTEIN-BARR VIRUS INFECTION: CASE REPORT

Unilateral laterothoracic exanthem is a self-limited disease that occurs most commonly in children. It is characterized by unilateral exanthem, often in axillary region. The etiology is unknown, but a viral agent is suspected. We report a 1-year-old white girl with unilateral laterothoracic exanthem associated with Epstein Barr virus infection, suggesting this virus has a possible etiologic role.

Nail plate cohesion seems to be water independent

Brittle nails are a cosmetic issue to most patients but, when dystrophy is severe, nail function may be impaired. Stern et al . 1 studied the water content in brittle nails and concluded that there was no significant difference in water content between brittle and normal fingernail plates and that, possibly, there is no relationship between nail plate water content and nail brittleness. The nail plate contains three layers characterized by different orientations of keratin molecules. In the intermediate layer, in which hair-like-type α -keratin filaments are predominant, the filaments are oriented perpendicularly to the growth axis. The other two layers, dorsal and ventral, are composed of epidermis-type keratin filaments which are oriented parallel and perpendicular to the growth axis. This “sandwich” structure in the corneocytes – keratin-associated proteins stabilized by cysteine disulfide bonds, lipid bilayers, and desmosomes – gives the nail high mechanical toughness and hardness. Lipid bilayers parallel to the nail surface fill certain ampullar dilations of the dorsal plate and intercellular spaces in the ventral layer. 2,3 The relatively low lipid content of the nail plate ( < 5%) implies that water permeation through the plate is high in comparison with that through the stratum corneum. 2

Retronychia--clinical and pathophysiological aspects.

Retronychia represents proximal ingrowth of the nail that occurs when the nail embeds backwards into the proximal nail fold. It is suspected when there is a persistent paronychia, particularly in the setting of trauma. Important clinical criteria for diagnosis are inflammation of the proximal nail fold, granulation tissue emerging from under the nail fold, thickening of the proximal portion of the nail plate and interruption of nail growth. The condition is rarely diagnosed and often misinterpreted, and is therefore unnecessarily treated with systemic antibiotics and antifungals. Avulsion of the nail confirms the diagnosis and it is the curative treatment. Conservative treatment with an adhesive technique is a valid option in early cases. We report 20 cases of retronychia diagnosed in our department between 2010 and 2013.

Chalazion surgery: Advantages of a subconjunctival approach

Abstract The meibomian glands, present within the tarsus, produce an oily fluid which is part of the tear film. The obstruction of this gland is common, and results in a meibomian cyst or chalazion. Initially, a chalazion may spontaneously resolve, or respond to conservative measures such as warm compresses and topical antibiotics. However, sometimes the lesion becomes persistent and prominent, leading to visual and aesthetic problems, requiring a surgical solution. We report a 23‐year‐old man, with a right lower eyelid chalazion, persisting for more than 1 year, refractory to local measures that was submitted to surgical correction via the subconjunctival approach. This report emphasizes the advantages of this technique, namely the fast and simple procedure, with an excellent cosmetic result.

Leg ulcer and thigh telangiectasia associated with natural killer cell CD56– large granular lymphocyte leukemia in a patient with pseudo-Felty syndrome

Clonal disorders of large granular lymphocytes (LGL) represent a rare spectrum of biologically distinct lymphoproliferative diseases originating either from mature T cells or natural killer cells. Both subtypes can manifest as indolent or aggressive disorders. We report a 77-year-old woman with rheumatoid arthritis, splenomegaly, and neutropenia who developed a painful leg ulcer refractory to treatment and thigh telangiectatic lesions. Because of the association of rheumatoid arthritis, splenomegaly, and nonspecific neutropenia, the diagnosis of Felty syndrome was initially made. Further investigation allowed the diagnosis of a CD56(-) natural killer-cell LGL leukemia and documented skin infiltration by natural killer cells. Cutaneous manifestations of LGL leukemia have been rarely reported. This report of pseudo-Felty syndrome with CD56(-) LGL leukemia, presenting with a leg ulcer and telangiectasia, enhances the role of dermatology in the diagnosis of hematologic neoplasia.

Photoletter to the editor: Congenital atrichia associated with an uncommon mutation of HR gene

Congenital atrichia (CA) is a rare form of irreversible alopecia with an autosomal recessive mode of inheritance, usually associated with a mutation in the human hairless (HR) gene located at chromosome 8. Papular lesions may develop as an additional phenotypic feature. Herein we describe a case of CA supported by trichoscopy, histology and genetic analysis. The patients single brother had also universal alopecia. To our knowledge this is the second report of a specific pathogenic mutation (c.2818C>T) of the HR, which until now had only been identified in a family with CA and papular lesions, emphasizing the difficulty to establish a strict correlation between HR genotyping and the phenotype.

Stevens-Johnson syndrome/ erythema multiforme major and Chlamydia pneumoniae infection in young patients

Erythema multiforme major (EMM) is an acute, self-limited mucocutaneous disease characterized by the abrupt onset of symmetrical fixed red papules evolving to target lesions. It is triggered mainly by infections, such as herpes simplex virus (HSV) and Mycoplasma pneumoniae, or drugs. In instances of extensive skin lesions with “giant” targets, prominent involvement of several mucous sites and fever, it may be difficult to distinguish from Stevens-Johnson syndrome (SJS), a rarer, life-threatening reaction which is mainly drug-induced. We report a 7-year old boy with SJS and Chlamydia pneumoniae infection and 3 patients with erythema multiforme (EM) and co-infection with Chlamydia pneumoniae: a 3-year old girl and a 29-year old man developed EMM lesions associated to Mycoplasma pneumoniae and Chlamydia pneumonia and a 20-year old woman with EMM associated to herpes simplex type 2 and Chlamydia pneumoniae infection. None of the 4 patients had history of drug intake in the last two months. Chlamydia pneumoniae is an intracellular bacteria responsible for respiratory infections. Despite the fact that its role in SJS/EMM has been rarely reported, our cases suggest that it may cause SJS and trigger EM when co-infecting a patient, either with Mycoplasma pneumoniae or herpes simplex. We conclude that infection by Chlamydia pneumoniae should be suspected and ruled out in every patient with SJS/EMM, especially in those with signs of respiratory infection.

Docetaxel-induced acral erythema and nail changes distributed to photoexposed areas

Docetaxel is a semisynthetic taxane used in the treatment of several malignancies. A 60-year-old male patient suffering from a non–small cell lung cancer with bone metastasis was under treatment with docetaxel and developed acral erythema involving photoexposed areas. Onycholysis and melanonychia were also observed. Drug-induced acral erythema usually involves the palms and soles, but it may occur in more atypical areas such as the backs of the hands and feet and also the face, as occurred in this patient. The nail changes are usually observed after several cycles of docetaxel, but tend to resolve gradually over several weeks despite continued treatment. Early recognition of skin and nail changes in the setting of docetaxel treatment, especially with an unusual presentation, is of paramount importance not only for patient reassurance, but also to avoid unnecessary withdrawal of drug treatment.