Folliculitis decalvans-like pustular plaques on the limbs sparing the scalp

Folliculitis decalvans is a neutrophilic cicatricial alopecia characterised by progressive pustular folliculitis. Folliculitis decalvans is seen as a condition usually limited exclusively to the scalp and rarely affects the limbs. We present a case of a 63-year-old man with a 3-year history of progressive pustular folliculitis with inflammatory patches and central scarring alopecia on both forearms and a circumscribed patch on his right lower leg. His presentation, clinical course and isolation of Staphylococcus aureus together with the histopathological findings all supported a folliculitis decalvans-like pustular folliculitis limited to the limbs. Biopsies revealed follicular pustules, gross interfollicular fibrosis with plasma cells and concentric perifollicular fibrosis with lymphocytes, all features seen with folliculitis decalvans. The positive response to antibiotics combined with topical corticosteroids mirrored the response seen with scalp folliculitis decalvans. In contrast to the previously reported cases, the patient had no evidence of folliculitis decalvans on the scalp.     

Coats plus syndrome (cerebroretinal microangiopathy with calcifications and cysts‐1): A case report

We present a 6‐year‐old girl with skin hyperpigmentation, leukoplakia, and onychodystrophy, the classic mucocutaneous triad usually associated with dyskeratosis congenita. The patient also had premature graying of the hair, bone marrow failure, hepatitis, exudative retinopathy, osteopenia with multiple long bone fractures, and intracranial calcifications and brain cysts. Coats plus syndrome is a rare disease with a clinical and genetic overlap with dyskeratosis congenita. This disease is reviewed, with a focus on the pathogenesis of the genetic anomalies and its background as a telomere biology disorder.     

Chronic urticaria associated with autoreactivity to progesterone

Autoimmune progesterone dermatitis is a rarely recognized cutaneous disorder revealed by recurrent and cyclic skin eruption with variable morphology, during the luteal phase. We report the case of a 42-year-old woman with chronic urticaria since the age of 38 with premenstrual exacerbation. Intradermal progesterone tests revealed a strong reactivity against this hormone. Treatment with thalidomide was effective but induced a peripheral neuropathy. Oophorectomy was performed with clearing of the cutaneous lesions.     

Atypical retiform hemangioendothelioma arising in a patient with Milroy disease: a case report and review of the literature

Retiform hemangioendothelioma (RH) is a rare vascular neoplasm with a high rate of local recurrence and low metastatic potential. We describe an unusual case of RH in a 45‐year‐old patient with Milroy disease, with a prominent solid component diffusely involving a chronic lymphedematous leg. This case is consistent with the postulated relationship between lymphedema and vascular neoplasms developing as a result of local immune dysfunction, and highlights the need to closely monitor patients with Milroy disease for pathologic changes. Our case highlights a unique example of RH with atypical features. There are several noteworthy unusual clinical and histologic findings including diffuse involvement of an entire limb, solid component with cytologic atypia, D2‐40 expression, and first‐time‐reported association with Milroy disease. Given the atypical histologic presentation of cytologic atypia, solid areas and atypical immunohistochemical profile with D2‐40 positivity, this case could cause diagnostic difficulty, especially in the setting of such a broad clinical differential.     

Variable response to propranolol therapy for infantile hemangiomas

Since propranolol was accidentally discovered to induce involution of hemangiomas, it is being used by many clinicians with a favourable response. We present our experience with use of propranolol and report three infants with hemangiomas who were treated with propranolol. First infant had a marked involution of the lesion; second infant had a appreciable response while the third infant had a initial minimal response with progressive increase in size of the lesion.     

Angiokeratoma corporis diffusum in a Spanish patient with aspartylglucosaminuria

Angiokeratoma corporis diffusum (ACD), initially considered to be synonymous with Fabry's disease, represents a well-known cutaneous marker of some other lysosomal enzyme disorders. Aspartylglucosaminuria (AGU) is a rare hereditary disorder mostly affecting the Finnish population, with only a few sporadic patients of non-Finnish origin. To date, only three patients with AGU have been reported with cutaneous lesions of ACD. A 19-year-old Spanish woman presented with a 10-year history of progressive ACD affecting the limbs, buttocks and trunk. After the age of 6 years she had developed progressive mental deterioration, coarse facies and macroglossia with a scrotal appearance. Peripheral blood smears showed many vacuolated lymphocytes. Enzyme analysis in cultured fibroblasts revealed a decreased activity of aspartylglucosaminidase. By the age of 31 years the patient had developed a bipolar psychosis, polycystic ovarian disease and severe impairment of cognitive skills. This is the first case of AGU detected in a Spanish patient presenting with cutaneous lesions of ACD. To our knowledge, macroglossia with a scrotal appearance and polycystic ovarian disease have not been reported in previous cases of AGU.     

Patch testing with a mixture of 2 phenol-formaldehyde resins

1310 patients were routinely patch tested with a paratertiary-butylphenol-formaldehyde resin (PTBP-F-R), a resol resin based on phenol and formaldehyde (P-F-R-2), and a mixture of these 2 resins. Approximately 2.5 times more patients with contact allergy to phenol-formaldehyde resins were diagnosed when routinely patch tested with P-F-R-2 in addition to PTBP-F-R. Although patch testing with a mixture of both resins was not as good as patch testing with the 2 resins separately, it was better than testing only with PTBP-F-R, since 1.6 times more patients with contact allergy to phenol-formaldehyde resins were still diagnosed. P-F-R-2 is therefore recommended for routine patch testing, preferably as a separate patch test but otherwise as a mixture with PTBP-F-R.     

Three unusual histopathological presentations of angiolymphoid hyperplasia with eosinophilia

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a poorly understood benign vasculoproliferative disorder. Histopathologically, the lesions are composed of a dermal or subcutaneous proliferation of blood vessels of varying sizes with plump and bland endothelial cells, which often show vacuolization and protrusion of the cytoplasm into the lumen. The vascular proliferation is admixed with a diffuse inflammatory infiltrate composed of lymphocytes, eosinophils and mast cells. Lymphoid follicles with germinal centers can sometimes be seen. In addition, fibrosis is a common accompanying feature. Some clinical and histopathological variants of ALHE have already been described in the literature. In this report, we present 3 rare associations of ALHE that have not been previously described. Case 1 was a 73‐year‐old woman with a lesion on her right medial thigh. Examination showed ALHE admixed with a chronic lymphocytic leukemic (CLL) infiltrate. Case 2 was a 55‐year‐old woman with a lesion on her right anterior ankle, which was a syringocystadenoma papilliferum co‐existing with an ALHE. Case 3 was a 54‐year‐old man with a lesion on the left medial thigh, which showed overlapping features of IgG4‐related disease associated with areas of ALHE. Given these multiple and diverse associations, it seems likely that ALHE may be a reactive rather than neoplastic process.     

Dermatoscopic finding of white circles in an invasive pigmented melanoma

Dermatoscopic white circles, seen with both non-polarized and polarized dermatoscopy, are a known clue to actinic keratosis in a flat lesion and invasive squamous cell carcinoma in a raised lesion. We have not discovered a previous published example of this clue in a melanoma. We present a case report of a 70-year-old Australian male with a pigmented superficial spreading melanoma on the face, Breslow thickness 1 mm, with dermatoscopic white circles displayed with both polarized and non-polarized dermatoscopy, and with dermatopathological correlation.     

Should all patients with dermatitis herpetiformis follow a gluten-free diet?

Aim To assess the effect of a gluten-free diet in Irish patients with dermatitis herpetiformis, and whether treatment with a gluten-free diet is as important for patients with a normal small bowel biopsy us for those with villous atrophy. Background Though a gluten-free diet is recommended in the management of dermatitis herpetiformis, many patients find it intolerably restrictive. To date we have recommended it only to patients with abnormal small bowel histology. Methods Forty patients with dermatitis herpetiformis who attended our clinic between 1979 and 1994 were studied retrospectively. Villous atrophy was present in 20 (64%) of 31 initial small bowel biopsies in patients not on a gluten-free diet. Results The median time to a 50% reduction in dapsone requirements was 6 months in patients who followed a gluten-free diet. (n = 14). 10.5 months in those who had a gluten-reduced diet (n = 4) and 10.5 months in those who took a normal diet (n = 22). Four of 14 patients (29%) on a gluten-free diet were able to discontinue medication in 1–5 years compared with 2 of 22 (9%) on a normal diet. The mean time to a 50% reduction in dapsone requirements was similar in patients with and without villous atrophy. 9.3 versus 9.0 months in patients on a gluten-free diet and 12.0 versus 15.3 months in patients on a normal diet. Conclusion We conclude that a gluten-free diet should be strongly encouraged in all dermatitis herpetiformis patients, since those with normal small bowel biopsy findings benefit equally from the diet as do those with villous atrophy.     

Meyerson's naevus: a clinical and histopathological study of 11 cases

We undertook a clinical and histopathological analysis of patients presenting with Meyerson's naevi. Eleven patients with the characteristic histological features of a Meyerson's naevus were identified over a 5-year period. Diagnostic criteria included epidermal spongiosis and a dermal inflammatory infiltrate associated with a banal junctional or compound naevus. Cases were excluded if naevus cells showed moderate to severe atypia or regression. Patients were contacted by phone and interviewed regarding their lesions. The most common clinical appearance was a solitary, pruritic, erythematous eruption encircling a pre-existing pigmented naevus. The trunk and proximal upper extremities were preferentially affected. Only one clinician listed Meyerson's naevus in the clinical differential diagnosis. All cases demonstrated a pigmented junctional or compound naevus with epidermal spongiosis, parakeratosis and a perivascular lymphohistiocytic inflammatory infiltrate with scattered eosinophils. The inflammatory infiltrate consisted almost exclusively of CD3+ lymphocytes, the majority of which were CD4+. However, a substantial number were CD8+. In all patients, the lesions cleared with excision or spontaneously, without recurrence or progression to melanoma. The aetiology of this entity remains unclear and most clinicians are unlikely to be familiar with it.     

Novel Treatment of Neck Wrinkles with an Intradermal Radiofrequency Device

Neck wrinkles commonly develop owing to the aging process. However, recently, the number of patients with neck wrinkles has been increasing. Also, an increasing number of young patients have presented with this condition, possibly because of the effect of the head-down posture that they adopt when using their computer or smartphone. We report two cases of young adults with a prominent neck wrinkle. In case 1, a 29-year-old woman with a neck wrinkle was treated with six intradermal radiofrequency (RF) procedures. Her neck wrinkle was significantly improved with the RF treatment. In case 2, a 32-year-old woman with a wrinkle and generalized light brownish tiny papules on the neck was treated with three intradermal RF procedures simultaneously with 30% glycolic acid peeling. Her wrinkle and skin tone were improved dramatically. We conclude that intradermal RF has a considerable efficacy for reducing neck wrinkles.     

Burn-induced linear IgA dermatosis

There have been several reports of linear IgA dermatosis (LAD) associated with drug exposure and lymphoproliferative malignancy, but trauma and burns have been suggested only in patients with bullous pemphigoid. We present a case of burn-induced LAD in a 48-year-old caucasian male presenting with a recent history of blistering eruption on the periphery of a cicatricial area caused by boiling methyl alcohol. Clinically, he presented a widespread bullous eruption. The direct immunofluorescence examination of a perilesional biopsy revealed an intense homogeneous linear pattern of IgA deposition consistent with the diagnosis of LAD. The patient responded to therapy with systemic steroids.     

Folliculotropic mycosis fungoides (stage IIA) progressing to Sézary syndrome: a case report

Folliculotropic mycosis fungoides is associated with a worse prognosis than classical mycosis fungoides (MF), but whether this is due to resistance to skin-directed therapy or to biological differences is unclear. We discuss a case of a patient with folliculotropic MF (stage IIA) who progressed to develop Sézary syndrome (SS), stage IVB, over 6 years. A 40-year-old man presented with pruritic plaques affecting his head and trunk, characterized by follicular plugging. The histology was consistent with folliculotropic MF and T-cell gene analysis studies revealed a T-cell clone in the skin only. His condition gradually deteriorated and 5 years after presentation, T-cell gene analysis studies revealed the presence of a clone in the blood identical with that seen in the skin. His condition progressed with the development of erythrodermic disease and a leukaemic blood picture and he subsequently died of systemic nodal and visceral involvement. We present the first report detailing the stepwise progression of a patient with stage IIA folliculotropic MF to SS. This case demonstrates that MF and SS represent a clinical spectrum of the same disease.     

Fractional carbon dioxide (CO2) laser combined with topical tretinoin for the treatment of different forms of cystic acne

Nodulocystic acne is prone to scarring and difficult to treat with treatments other than oral isotretinoin. The aim of this article is to discuss the role of a single session of a fractional carbon dioxide (CO₂) laser combined with a topical treatment with a tretinoin and antibiotic gel for a month as a successful treatment to improve nodulocystic acne and chronic microcystic acne. Two cases were involved: the first with nodulocystic acne lesions that persisted after oral retinoids and the second with chronic microcystic acne resistant to topical treatments. After only one session of treatment with the CO₂ laser and the topical treatment, a complete healing of the nodulocystic acne lesions was observed with minimal secondary effects. The microcystic acne showed great improvement. No other topical or oral treatment was needed. This treatment could be a safe and effective treatment for nodulocystic acne lesions and microcystic acne when other treatments fail. More studies should be performed to confirm our results.     

Repeated open application test with methyldibromo glutaronitrile, a multicentre study within the EECDRG

Contact allergy to and allergic contact dermatitis from methyldibromo glutaronitrile (MDBGN) have frequently been reported. This study was initiated to help determine the optimal patch test preparation for MDBGN. In 51 patients with a doubtful or a positive patch test reaction to at least 1 of 4 test preparations with MDBGN in petrolatum at 1.0% w/w, 0.5%, 0.3% and 0.1%, a repeated open application test (ROAT) with moisturizers with and without MDBGN at 0.03% w/w was performed on the upper arms for 2 weeks. 18 of the 51 (35.3%) patients developed a positive ROAT. In all patients, there was a positive ROAT only to the moisturizer with MDBGN (P < 0.001). A statistically significant association was also found between the patch test reactivity (PTRL) and the outcome of the ROAT (P < 0.001). If only considering those with a PTRL above 0.3%, thus with negative or doubtful test reactions to 0.1% and 0.3%, there were still statistically significantly more patients with a positive ROAT to the moisturizer with MDBGN than to the moisturizer without MDBGN. The study demonstrates that patch testing with MDBGN at 0.3% and 0.1% will miss clinically relevant patch test reactions to MDBGN.     

A high rate of cross-sensitivity between Parthenium hysterophorus and Xanthium strumarium in Indian patients with contact dermatitis

Parthenium hysterophorus is well known to produce contact dermatitis in India. Xanthium strumarium is another weed also belonging to the Compositae family present in India since much earlier times, but not recognised among the plants causing contact dermatitis. We have found a very high rate of cross-sensitivity between the two plants in patients presenting air-borne contact dermatitis. Of the 28 patients with air-borne contact dermatitis. 22 revealed positive patch tests with aqueous extracts of both plants, one patient showed a negative patch test with Parthenium hysterophorus and a positive test with Xanthium strumarium , while five patients were negative with both plants. In a control group of 15 patients with contact dermatitis due to other agents, only one patient showed a positive patch test with Xanthium strumarium , while the patch test with Parthenium hysterophorus was negative. Patch tests performed with serial 10-fold dilutions of standard extracts of both plants to determine the degree (titre) of contact hypersensitivity (TCH) revealed variable litres in different patients. Some patients had a much higher titre with Parthenium hysterophorus while others had a higher titre with Xanthium strumarium. The antigen in Xanthium strumarium , like that of Parthenium hysterophorus , could be extracted with water, acetone, ethanol, chloroform, benzene, ether and carbon tetrachloride. Infra-red spectrophotometry of extracts in ether revealed similar spectra in the two plants. The antigens in the two plants seem lo be very similar.     

Effect of calcipotriol on epidermal cell populations in alefacept-treated psoriatic lesions

BACKGROUND AND OBJECTIVES: The effect of the established antipsoriatic treatment with topical calcipotriol (with a maximum of 100 g per week) in addition to systemic treatment with alefacept, a new biological agent for psoriasis, on epidermal cell populations in the psoriatic lesion was investigated using a combination of the Zenon labelling technique and microscopic image analysis. Epidermal cell populations were measured quantitatively with this sensitive method. PATIENTS/METHODS: Frozen sections of non-treated psoriatic epidermis and psoriatic epidermis treated with either alefacept intramuscular or alefacept intramuscular in combination with topical calcipotriol for 12 weeks were compared immunohistochemically. Antibodies against keratin 6, 10 and 15 were labelled with the Zenon technique, whereas antibodies against the Ki-67 antigen and beta-1 integrin were covalently Fluorescein Isothiocyanate (FITC)-labelled. Using image analysis, these markers were measured in the epidermis in a standardized manner. RESULTS AND CONCLUSIONS: Treatment of psoriasis with alefacept resulted in a good clinical response in several patients and in a normalization of epidermal expression of the immunohistochemical parameters for differentiation and proliferation. The addition of topical calcipotriol resulted in a faster clinical improvement with a similar overall clinical response and a similar response of epidermal cell populations as compared to treatment with alefacept monotherapy after 12 weeks of treatment. This study also suggests that the appearance of keratin 15 has a predictive value for the duration of remission. It can be concluded that the addition of a low-dose calcipotriol treatment does not contribute to the clinical efficacy of alefacept, both at the clinical level and with respect to markers for epidermal proliferation and differentiation.     

Safety of infraorbital hyaluronic acid injections: Outcomes of a meta-analysis on prospective clinical trials

Background

Hollowing of the infraorbital region represents a common concern among aesthetic patients. In the past decade, an increasing number of patients have resorted to noninvasive aesthetic procedures to treat these concerns. The objective of this study was to evaluate the safety profile of infraorbital hyaluronic acid injections for aesthetic rejuvenation.

Methods

Through a systematic review and meta-analysis of prospective clinical trials, investigators sought to answer the research question “Does the use of needle versus cannula during infraorbital HA injections result in the same incidence rate of adverse events?” The primary outcomes of interest were the incidence rates of ecchymosis and edema in subject groups treated with a needle or cannula.

Results

Subjects treated with needles had a statistically significant greater incidence rate of ecchymosis, compared to those treated with cannula. Conversely, subjects treated with cannula had a statistically significant greater incidence rate of edema, compared to those treated with needles.

Conclusions

The incidence rates of adverse events following the administration of hyaluronic acid injections in the infraorbital region vary depending on whether a needle or cannula is used; with needles being associated with a greater risk of ecchymosis and cannulas being associated with a greater risk of edema. These findings should be discussed with patients prior to treatment consultation. Finally, as with most techniques, it is usually prudent to develop expertise with one technique before using a second, especially in cases where both approaches can be used and have different adverse event profiles.     

Ambulatory monitoring of ultraviolet erythema in photosensitive subjects

Little is known about whether patients with photosensitive disorders exhibit a different ultraviolet erythema time course from subjects with a normal response to sunlight. We have described the application of an instrument for ambulatory monitoring of the development of ultraviolet erythema by a reflectance method in a group of patients with chronic actinic dermatitis (CAD) and in a group of normal subjects. Investigations of the time course have been reported previously but the techniques used relied upon manual measurement. Consequently sampling frequencies have been considerably lower than the one-minute sample rate used here. We have not demonstrated any difference in the rate at which erythema develops and peaks between patients with CAD and subjects with a normal response to sunlight.