Athanassios Kolivras

Cryopyrin-associated periodic syndrome: an autoinflammatory disease manifested as neutrophilic urticarial dermatosis with additional perieccrine involvement

A female newborn presented with a congenital urticarial rash that consisted of fluctuating well‐demarcated pink or pale reddish macules or slightly raised papules and plaques. In addition, purulent cerebrospinal fluid was present in the absence of evidence of congenital infection. Skin biopsy revealed a sparse infiltrate throughout the entire dermis, including the eccrine adventitia. The infiltrate was composed mostly of neutrophils, but rarely lymphocytes and eosinophils could also be seen. No vasculitis was present. Because of the presenting attributes, a diagnosis of cryopyrin‐associated periodic syndrome (CAPS) was considered and the neonatal‐onset multisystem inflammatory disorder (NOMID) that represents the most severe expression of the CAPS clinical spectrum was favored. Diagnosis was confirmed by identification of a mutation in the cold‐induced autoinflammatory syndrome‐1 gene and by an observed response to treatment with the interleukin‐1 receptor antagonist anakinra. Both the clinical and histopathological findings of the presented case may represent a distinct entity within the spectrum of aseptic neutrophilic dermatitis. We refer to this spectrum as neutrophilic urticarial dermatosis (NUD), which may serve as a cutaneous marker of autoinflammation. NUD with perieccrine involvement should prompt consideration of CAPS, especially NOMID, in the context of neonatal multisystem disease.

Cutaneous histopathological findings of Aicardi–Goutières syndrome, overlap with chilblain lupus

We report a 2‐year‐old girl with developmental delay who, from the age of 1 year, developed perniotic lesions of the hands and feet initially diagnosed as chilblain lupus. Histological examination showed features of epidermal necrosis with intraepidermal bulla formation, interface dermatitis, lymphocytic vasculitis with fibrinoid necrosis and thrombi formation, both superficial and deep dermal lymphocytic infiltrate, lymphocytic eccrine hidradenitis and absence of marked dermal edema. Subsequent investigations suggested a clinical diagnosis of Aicardi–Goutières syndrome (AGS), a rare genetic leukoencephalopathy. Recently, both AGS and familial chilblain lupus, an autosomal dominant form of systemic lupus erythematosus (SLE), have been shown to be allelic thus suggesting a common pathogenic basis. In addition, a phenotypic overlap is apparent between SLE and AGS. To our knowledge, this is the first comprehensive dermatopathological report of the cutaneous lesions seen in AGS, and our paper highlights the importance of considering AGS in the differential diagnosis of perniosis and chilblain lupus.

Tinea capitis in Brussels: epidemiology and new management strategy.

Background: We found pre-established directives inadequate to cope with the current increase in anthropophilic tinea capitis in Brussels. Objectives: To study new epidemiological profiles and to define new strategies for management and prevention. Patients and Methods: A total of 122 children affected by tinea capitis were followed in our department from October 1, 2001, until September 30, 2002. The results were assessed retrospectively. Results: Anthropophilic tinea capitis represented 89.34% of the cases. The implicated anthropophilic dermatophytes were by decreasing frequency: Microsporum langeronii (39.34%), Trichophyton soudanense (28.69%), Trichophyton violaceum (18.03%) and Trichophyton tonsurans (3.28%). Conclusion: The responsible pathogens reflect immigration flows originating mostly from Black and North Africa. Precise recommendations for each visit are detailed.

Thalidomide in Refractory Vulvar Ulcerations Associated with Crohn’s Disease

We report a case of recurrent vulvar ulcerations developed on vegetations associated with Crohn’s disease. Lesions responded to low-dose thalidomide treatment. A biopsy revealed a neutrophilic infiltrate with rare giant cells. Vulvar manifestations were consistent with metastatic Crohn’s disease or with a reactive neutrophilic dermatitis. Thalidomide reduces the activity of tumor necrosis factor (TNF) α by accelerating the degradation of its mRNA. Nowadays, it has become a promising drug in a broad variety of disorders in which TNF-α seems to play a pivotal role, including inflammatory bowel diseases. This is the first case report presenting the efficacy of thalidomide in the treatment of vulvar involvement in Crohn’s disease.

Nail Destruction in Pemphigus vulgaris

Involvement of the nails in pemphigus vulgaris (PV) is rare and is usually seen when the disease is severe. The most common clinical manifestations are chronic paronychia and onychomadesis. Finger nails are more frequently involved than toe nails. We report a case of severe and persistent PV in which an exacerbation was preceded by an erosive and destructive lesion of the right hallux nail unit. A nail bed biopsy was performed to rule out a subungual tumour. The image of suprabasal acantholysis yielded the diagnosis of PV. This localisation and clinical manifestation of PV have only been reported once.

POEMS Syndrome Revealed by Multiple Glomeruloid Angiomas

POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin disorders) is a rare multisystemic disease associated with plasma cell dyscrasia. A 68-year-old woman with chronic renal insufficiency and arterial hypertension included in her medical history was admitted to the hospital with confusion, somnolence and asthenia. She presented ascites, hepatosplenomegaly, leg oedema, distal dysesthesias, leuconychia and multiple nodular purple red angiomas on the trunk, upper limbs and fingers. Hypothyroidism was revealed in the laboratory investigations and monoclonal IgG peak in immunoelectrophoresis. Electromyography showed both demyelinisating and axonal degenerative neuropathy. The diagnosis of POEMS syndrome was based on the dermatopathological examination of a cutaneous angioma; histology revealed features of glomeruloid angioma, a specific marker of this syndrome.

Recurrent cheilitis and lip oedema caused by (meth)acrylates present in ultraviolet-curable nail lacquer.

Keywords: acrylates; allergic contact dermatitis; cheilitis; gel polish; lip oedema; methacrylates; UV-curable nail lacquer

Revisiting nephrogenic systemic fibrosis in 6 kidney transplant recipients: a single-center experience.

BACKGROUND Nephrogenic systemic fibrosis (NSF) is a fibrotic disorder occurring in patients with renal dysfunction. Exposure to gadolinium (Gd)-based contrast agents (GBCAs) during renal impairment is associated with development of NSF. METHODS A cross-referenced search of kidney transplantation and radiology databases at a single institution revealed the prevalence of NSF in the transplant population. Clinical records and skin biopsy specimens from 6 patients with kidney transplant given a diagnosis of NSF were reviewed to identify contributing factors. RESULTS Between January 1999 and December 2006, NSF was diagnosed in 6 of 705 patients with kidney transplant (0.9%). Renal function was impaired in all patients. Of 33 patients with kidney transplant exposed to GBCAs, 5 (15.2%) developed NSF. Disease onset ranged from 7 days to 11 months after exposure to GBCAs. All 5 patients exposed to GBCAs who developed NSF were also treated with a beta-blocker and clinical improvement was observed with discontinuation. The sixth case NSF appeared unrelated to Gd, without a known exposure, and testing of tissue via mass spectrometry revealed no Gd. Symptoms of NSF in this patient disappeared after administration of darbepoetin was switched from subcutaneous to intravenous injection. One patient with NSF who manifested the highest Gd level in tissue died 22 months after disease onset. LIMITATIONS The study represents the retrospective experience of only a single center. CONCLUSIONS NSF can develop in kidney transplant recipients with altered graft function. In these patients, exposure to GBCAs appears associated with development of NSF. The role of beta-blockers in the course of the disease merits further investigation.

Gianotti‐Crosti Syndrome Following Hepatitis A Vaccination

Abstract:  We report a case of Gianotti‐Crosti syndrome in a child following hepatitis A vaccination in order to insist that currently available hepatitis A vaccines are highly immunogenic and that Gianotti‐Crosti syndrome is a possible minor adverse reaction.

Eruptive tumors of the follicular infundibulum presenting as hypopigmented macules on the buttocks of two Black African males

We report two cases of eruptive tumors of the follicular infundibulum (TFI) with an unusual clinical presentation which has not been described previously in literature. In both cases, the appearance was strikingly similar, consisting of multiple asymptomatic hypopigmented macules on the buttocks of two Black African males, aged 38 and 55 years old. In both cases, the eruption had evolved over several months. The individual lesions were of similar size, approximately 1 cm, with irregular and ill‐defined borders. Histopathological examination revealed a superficial and horizontal plate‐like proliferation of keratinocytes emanating from the epidermis with multiple slender attachments. Pale keratinocytes were present within the epithelial plates. A Fontana stain showed a loss of melanin pigment from the epithelial plates. Orcein (elastic) stain highlighted an increase of the number of the elastic fibers surrounding the tumor. On the basis of these findings, a diagnosis of eruptive TFI was established for both cases. Among the various presentations of TFI, only the eruptive variant appears to be clinically distinctive, with asymptomatic hypopigmented macules usually located on the face, neck and upper trunk. Eruptive TFI should also be added to the clinical differential diagnosis of multiple hypopigmented macules on the buttocks of Black patients.