Isabelle Masouyé

Epidermal Fatty-Acid-Binding Protein in Psoriasis, Basal and Squamous Cell Carcinomas: An Immunohistological Study

BACKGROUND In human keratinocytes, we have recently characterized a low-molecular-weight cytosolic protein of 15 kD that specifically binds fatty acids (FAs) with high affinity, the epidermal FA-binding protein (E-FABP). The distribution of E-FABP in skin diseases is not known. OBJECTIVE To localize by immunohistochemistry the expression of E-FABP in psoriasis, basal and squamous cell carcinomas in order to obtain indirect information, at the cellular level, on the transport of the FAs. RESULTS E-FABP was localized in the upper stratum spinosum and stratum granulosum in normal and non-lesional psoriatic skin. In contrast, lesional psoriatic epidermis strongly expressed E-FABP in all suprabasal layers, like nonkeratinized oral mucosa. The basal layer did not express E-FABP reactivity in any of these samples. Accordingly, basal cell carcinomas were E-FABP negative whereas only well-differentiated cells of squamous cell carcinomas expressed E-FABP. CONCLUSION It is unlikely that E-FABP plays a significant role in FA uptake by basal cells. Our data rather indicate that E-FABP expression is related to the commitment of keratinocyte differentiation and that the putative role of E-FABP should not be restricted to the formation of the skin lipid barrier. Since the pattern of E-FABP expression mimics cellular FA transport, our results suggest that lesional psoriatic skin and oral mucosa have a higher metabolism/transport for FAs than normal and non-lesional psoriatic epidermis.

Multiple cutaneous osteomas of the face associated with chronic inflammatory acne

Multiple miliary osteoma cutis of the face represents a rare and frequently unrecognized complication of chronic inflammatory acne. Their differentiation from microcomedones and macrocomedones may be challenging. The case of a 46‐year‐old Asian woman who suffered from chronic inflammatory acne is described. She had multiple papular lesions of the cheeks that did not respond to various topical and systemic therapies including oral isotretinoin. Light microscopy studies as well as ultrasound and computed tomography (CT) scan investigations demonstrated the presence of multiple osteoma cutis. Needle microincisions followed by mechanical extirpation of the bony formation resulted in a considerable cosmetic improvement of her skin disease. Knowledge of this rare complication of acne is mandatory, as its treatment is different from that of retentional and inflammatory acne and frequently relies on surgical modalities. Our novel technique consisting of needle microincisions with curettage of the lesions is simple and safe, leading to good cosmetic results.

Bullous pemphigoid and multiple sclerosis: More than a coincidence?

In three patients with long-standing multiple sclerosis, bullous pemphigoid developed. The diagnosis of bullous pemphigoid was based on histologic findings, direct and indirect immunofluorescence, and Western blots showing IgG reacting with the 220 to 240 kD bullous pemphigoid antigen in the serum of three patients. Contrary to previous observations, bullous pemphigoid associated with multiple sclerosis was not different from bullous pemphigoid alone. Three similar cases have been reported previously, so the occurrence of bullous pemphigoid in patients with multiple sclerosis may be more than a coincidence.

Congenital insensitivity to pain with anhidrosis

Congenital insensitivity to pain with anhidrosis is an autosomal-recessive disorder resulting from defective neural crest differentiation with loss of the first-order afferent system, which is responsible for pain and temperature sensation. There is also a neuronal loss in the sympathetic ganglia. Lack of sweating, hyperthermia, and infections of bones are main features of the disorder; however, contradictory results have been published regarding eccrine sweat gland innervation. A 5-year-old male patient with typical clinical manifestations of congenital insensitivity to pain with anhidrosis is presented. Immunohistochemistry with antibodies against S100 protein and neuron-specific enolase failed to reveal nerve fibers in the vicinity of the eccrine sweat glands. The roles of the nerve growth factor and tyrosine kinase receptor gene mutations in the pathogenesis of the disease are also discussed.

Mucocutaneous Lichen Planus With Esophageal Involvement Successful Treatment With an Anti-CD20 Monoclonal Antibody

Laurent Parmentier, MD, PhD; Blaise-Alain Bron, MD; Christa Prins, MD; Jacky Samson, MD; Isabelle Masouyé, MD; Luca Borradori, MD; Service de Dermatologie (Drs Parmentier, Prins, Masouyé, and Borradori) and Division de Chirurgie Orale (Dr Samson), Hôpitaux Universitaires de Genève, and Médecin Gastro-Entérologue (Dr Bron), Geneva, Switzerland. Dr Parmentier is now with Universitätsklinik für Dermatologie, Inselspital, Bern, Switzerland.

A Case of Amicrobial Pustulosis of the Folds Associated with Neutrophilic Gastrointestinal Involvement in Systemic Lupus Erythematosus

Background: Amicrobial pustulosis of the folds is a recently described entity characterized by relapsing pustular lesions involving predominantly the cutaneous folds and the scalp. The disease typically occurs in the context of an autoimmune or systemic disease and should be included within the spectrum of neutrophilic dermatoses. Observation: We describe a patient with a history of systemic lupus erythematosus, who developed amicrobial pustulosis of the folds. Strikingly, the patient also exhibited recurrent oral and gastrointestinal manifestations that paralleled the course of the cutaneous signs. Conclusions: Our observation indicates that, in analogy to the other neutrophilic dermatoses, amicrobial pustulosis of the folds can also be complicated by the development of extracutaneous neutrophilic involvement, knowledge of which is critical for its diagnosis and proper management.

Fox-Fordyce Disease: Successful Treatment with Topical Clindamycin in Alcoholic Propylene Glycol Solution

We report a 66-year-old woman with a 3-year history of intensely pruritic follicular papules in the axillar, pubic and inguinal areas. Previous treatment with topical fusidic acid and gentamicin sulfate was ineffective. The clinical and histological examination was consistent with Fox-Fordyce disease. Application of clindamycin in an alcoholic propylene glycol solution led to the clearing of the lesions within 1 month. Nine months later, the treatment was stopped, and no recurrence was observed.

Calcifying panniculitis following subcutaneous injections of nadroparin-calcium in a patient with osteomalacia

Calcifying panniculitis is a rare form of calcinosis cutis belonging to the spectrum of calciphylaxis that has almost invariably been described in patients with severe renal disturbances. We report a patient with osteomalacia without chronic renal failure, who developed calcifying panniculitis following subcutaneous administration of nadroparin‐calcium. Light microscopy studies of biopsy specimens revealed multiple foci of microcalcification within the adipose lobules, in the interadipocyte spaces, in connective tissue septa and in the media of small arteries in the subcutis. The patient had an elevated level of intact parathyroid hormone, whereas the calcium‐phosphorus product was normal. The lesions slowly resolved upon discontinuation of nadroparin. We conclude that calcifying panniculitis is a rare complication associated with the subcutaneous administration of nadroparin‐calcium that may rarely also occur in the absence of renal disturbances. Low molecular weight calcium‐containing heparins should probably be used with caution in the presence of hyperparathyroidism.

Comparative analysis of the expression of ERBIN and Erb-B2 in normal human skin and cutaneous carcinomas.

Background  ERBIN is a binding partner of Erb‐B2, an orphan receptor within the Erb‐B family critically involved in the regulation of cell growth and differentiation. Although its function remains unclear, ERBIN is thought to affect the polarity of epithelial cells and cell growth via the Ras signalling pathway.

Localized childhood vulval pemphigoid treated with tacrolimus ointment.

Background: Localized vulval childhood pemphigoid is a rare variant within the pemphigoid group. Although its prognosis seems favorable, the best therapeutic strategy remains unclear. Observation: We here describe the case of an 8-year-old girl presenting with a 5-year history of relapsing vulval pain and lesions suggestive of lichen sclerosus. Clinical features, light microscopy and direct immunofluorescence microscopy were consistent with vulval cicatricial pemphigoid, although the autoantigen(s) involved could not be characterized. Her disease responded to treatment with topical tacrolimus ointment 0.1% within 3 months without any evidence for disease activity, except for slight residual scarring. After 12 months, her treatment was stopped without relapse. Conclusion: This observation suggests that in this rare immune-mediated blistering disease topical tacrolimus is an interesting therapeutic option without the adverse effects associated with topical steroids.