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Background: CD44 is a membrane glycoprotein and the major cell-surface receptor of hyaluronate (HA). Lack of CD44 expression in mouse epidermis leads to an abnormal HA accumulation in the dermis, indicating an important role of CD44 in local HA metabolism. Decrease of epidermal CD44 expression in patients of lichen sclerosus et atrophicus is potentially responsible for dermal deposition of HA in this disease. Stromal HA accumulation is associated with decreased or lost expression of CD44 in perifollicular solitary cutaneous myxoma, myxoid dermatofibroma, and dermatofibrosarcoma protuberans. Methods: We examined the expression of CD44 and HA in the skin biopsy specimens of 10 patients with follicular mucinosis by using CD44-specific antibodies and biotinylated HA-binding protein (HABP), respectively. Results: No difference of CD44 expression was observed in the follicular keratinocytes when compared with those of unaffected interfollicular epidermis. The follicular zones of mucin deposition were strongly positive for HA. A weak interkeratinocyte staining for HA was also observed in the interfollicular epidermis. However, HABP staining revealed a stronger reactivity in the follicular keratinocytes surrounding the mucin-accumulated areas compared to the interfollicular keratinocytes. Conclusion: Our results suggest an active secretion of HA by follicular cells in follicular mucinosis.

We describe a 74-year-old woman who rapidly developed bilateral voluminous nodules on the upper eyelids, following 4 months' treatment for chalazion. Histological and immunohistochemical findings were suggestive of cutaneous pseudolymphoma (CPL), and extensive screening for malignant lymphoma (ML) remained negative. Cutaneous pseudolymphomas are inflammatory diseases that can simulate ML either clinically, histopathologically, or both. They are a rare cause of nodules of the upper eyelids, usually characterized by a benign evolution, that can be secondary to ultraviolet sensitivity, adverse reactions to systemic medications and contact sensitization, among others. In our case, epicutaneous patch tests were strongly positive for a series of allergens contained in the ophthalmological preparations used; therefore, they were suggestive that the patient had CPL, lymphomatoid contact dermatitis type. In addition, the patient had been treated previously with several drugs, known to cause CPL and immune dysregulation. Complete regression of the lesions required treatment with systemic steroids and chlorambucil. No relapse occurred within a 5-year follow-up period.

Adult-onset Still's disease (AOSD) is a systemic disorder characterized by intermittent fever, evanescent rash, arthralgias or arthritis and predominantly neutrophilic leucocytosis. We report on a 16-year-old woman with Still's disease who developed, in addition to the typical rash, persistent papular lesions on her face, neck and upper and lower back. Although the presence of fixed skin lesions is not a characteristic feature of AOSD, their appearance at the onset of the disease and their evolution suggest that they represent a specific manifestation of the disease.

We report the case of a human immunodeficiency virus-positive patient presenting linear Darier disease with varicella-zoster virus superinfection following the lines of Blaschko. The lesions healed after treatment with brivudine.

Background: Dermatofibroma (DF) is a common benign histiocytic tumor, which has several clinicopathological variants. Myxoid DF is one of these variants, which is characterized by a stromal mucin deposition. CD44 is a polymorphic transmembrane glycoprotein and the principal cell surface receptor of hyaluronate (HA), the major component of the extracellular matrix. In a recent study, we have observed an abnormal accumulation of HA in the superficial dermis of transgenic mice with a keratinocyte-specific CD44 expression defect. We have also shown that HA was accumulated in large amounts in the superficial dermis of lichen sclerosus et atrophicus (LSA) lesions and that the epidermal CD44 expression of LSA skin was significantly decreased or lost. In an another study, we have suggested that a decrease in CD44 expression in follicular epithelial proliferations might be correlated with an abnormal HA accumulation in perifollicular solitary cutaneous myxoma. Recently we have also demonstrated that classical DF lesions displayed a strong CD44 expression in tumor cells and a weak HA positivity in tumor stroma whereas CD44 expression was significantly reduced or absent in dermatofibrosarcoma lesions and the tumor stroma showed strong HA staining. Objective and Methods: Here we present 3 cases of myxoid DF, in which we explored the nature of the mucinous material in myxoid stroma by colloidal iron and hyaluronic acid binding protein stainings, as well as the expression of CD44 in the tumor cells by immunohistochemistry. Results: We show that HA is accumulated in the stroma of all myxoid DF lesions with a significant decrease in CD44 expression in the tumor cells. Conclusion: Our results suggest that a decrease in CD44 expression in the tumor cells may result in stromal myxoid changes characterized by an abnormal HA accumulation in myxoid DF.

The genesis of familial and sporadic basal cell carcinomas involves activation of the Sonic hedgehog signal transduction pathway. Other known factors for the development of basal cell carcinoma are ultraviolet exposure, X-rays, race, age, gender and decreased DNA repair capacity. We here report the case of a right earlobe sporadic basal cell carcinoma in a 65-year-old woman. This case is unusual because of its earlobe localization and its association with multiple auricular acupuncture treatments. This observation suggests a connection between local traumas, which occur in the course of acupuncture treatment and ear piercing, and the genesis of basal cell carcinoma. The incidence of minor adverse events associated with acupuncture is high, but serious events are uncommon. Acupuncture is not known to date for promoting the development of tumors. This connection remains to be elucidated.

We report on a case of chronic cutaneous borreliosis with manifestations clinically compatible with morphea and lichen sclerosus et atrophicus. The histopathologic features of these lesions were those of acrodermatitis chronica atrophicans. Our case illustrates the concept that clinical aspects of morphea and lichen sclerosus et atrophicus pertain to the spectrum of cutaneous borreliosis.

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.

A 68-year-old woman presented with a symmetrical enlargement of the face and submandibular area associated with essential thrombocytosis. Biopsy of the lesions revealed a septal and lobular panniculitis disclosing a lymphohistiocytic and plasma cell proliferation with lymphophagocytosis by histiocytes, compatible with Rosai-Dorfman disease. The lesions regressed spontaneously without therapy over a period of 4 months. Diagnosis was confirmed by electron-microscopic examination and immunocytochemical presence of S-100 antigen within the histiocytic cells. A 2-year follow-up did not demonstrate any evidence of recurrence.

Two female patients who fulfilled the criteria for L-tryptophan-induced eosinophilia-myalgia syndrome (EMS) had, together with morphea-like and fasciitis-like sclerotic changes of the skin, lesions that clinically mimicked pseudoxanthoma elasticum (PXE). Histology was compatible with the diagnosis; electron microscopy did not reveal calcium deposits. PXE-like changes may represent an additional feature of the pleomorphic L-tryptophan-induced EMS.

Giant cell lichenoid dermatitis is a recently described pathological entity, which can be seen as an unusual lichenoid drug eruption, a manifestation of sarcoidosis or within herpes zoster scars. Histopathological findings include focal vacuolar alteration of the basal layer with cytoid bodies, dermal and intraepidermal multinucleated giant cells and a mixed chronic inflammatory infiltrate with a lichenoid pattern consisting of lymphocytes, histiocytes, eosinophils and plasma cells. Here, we report a giant cell lichenoid dermatitis in a 41-year-old male patient who developed, 3 days after intravenous treatment with amoxicillin-clavulanic acid for erysipelas of the left leg, a clinical picture suggesting a baboon syndrome characterized by an erythematous and pruritic eruption on the axillary, inguinal and popliteal areas and the anterior side of elbows. This is the first reported case of giant cell lichenoid dermatitis in a patient with baboon syndrome.

Solitary morphea profunda (SMP) is a distinct form of localized scleroderma, a cutaneous disorder of unknown origin. Here, we describe a patient presenting with noninflammatory cupuliform depressed plaques, without any significant skin induration, pigmentation or texture change, that appeared on the left shoulder at a site of previous intramuscular vaccination. Light microscopy studies revealed excessive dermal collagen deposition with thickened hyalinized collagen bundles. Magnetic resonance imaging studies demonstrated tissue fibrosis with thinning of the subcutaneous fat tissue and involvement of the adjacent deltoid muscle, which was confirmed by electromyographic tests. Borrelia serology was negative. Our observation of SMP differed from previously described cases, since it mimicked localized lipoatrophy. Our observation highlights the wide spectrum of clinical presentations of localized scleroderma. The latter should be considered in the presence of lipoatrophy-like lesions for proper workup and therapy.

A 2-month-old girt acutely developed a brownish, firm nodule on the left thigh. The lesion was surgically removed when the patient was age 3 months, and histology showed a massive dermal infiltration by large histiocytic cells with abundant ground glass cytoplasm. Most cells were S100 protein positive. Electron microscopic examination revealed the presence of Birbeck granules in about 30% of the histiocytes, as well as laminated dense bodies and wormlike bodies. No recurrence was observed during follow-up for three years. We believe that this case, like the one previously reported, represents a clinical variant of Hashimoto-Pritzker disease.

Erosive pustular dermatosis of the leg is a distinct form of spongiform amicrobial pustulosis. The disorder typically affects the lower limbs of elderly patients presenting with chronic venous insufficiency and stasis dermatitis, and has a chronic course. Three elderly patients with chronic venous ulcers are described, who developed pustules and moist eroded lesions on the leg. The clinical and histological features were typical for erosive pustular dermatosis. The lesions rapidly responded to topical treatment with either tacrolimus or corticosteroids. Of note, this condition was associated with a diverticular disease in two patients, while in another patient an epidermoid carcinoma of the tongue was present. Erosive pustular dermatosis of the leg is an uncommon but distinct skin disorder typically associated with trophic changes of the lower limbs. Our observations raise the question of the relation of erosive pustular dermatosis of the leg with the group of neutrophilic dermatoses. Topical immunotherapy with tacrolimus may constitute a novel therapeutic option for this frequently recalcitrant condition.

Background: Myxomas are rare cutaneous tumors which may be solitary or associated with Carney’s complex, NAME or LAMB syndromes. The mucinous material which constitutes the stroma of cutaneous myxomas is predominantly composed of hyaluronate (HA), the major component of the extracellular matrix. CD44 is a polymorphic integral membrane glycoprotein which serves as the principal cell surface receptor for HA. Objective and Methods: Here we present 2 cases of solitary cutaneous myxomas displaying microscopically a perifollicular localization, in which we explored the nature of the accumulated mucinous material by colloidal iron and HA-binding protein stainings, as well as the epidermal expression of CD44 protein by immunohistochemistry. Results: We show that HA is accumulated in the stroma of the cutaneous myxoma lesions and that the protein expression of CD44 in the keratinocytes of the trichofolliculoma-like epithelial buds projecting from the hair follicle centering these lesions is significantly decreased. Conclusions: Our results suggest that a decrease in CD44 expression in follicular epithelial proliferations may be correlated with an abnormal HA accumulation in cutaneous myxoma.

Two patients with alopecia syphilitica are presented. Moth-eaten alopecia is the most typical look of secondary syphilis on the scalp. With the decrease in syphilis cases in western countries, this rarer clinical manifestation tends to be misdiagnosed.

We report on a case of chronic cutaneous borreliosis with manifestations clinically compatible with morphea and lichen sclerosus et atrophicus. The histopathologic features of these lesions were those of acrodermatitis chronica atrophicans. Our case illustrates the concept that clinical aspects of morphea and lichen sclerosus et atrophicus pertain to the spectrum of cutaneous borreliosis.

Paraffinoma is a well-recognized complication of paraffin injection. We describe a 44-year-old man who had an ethmoidectomy for chronic sinusitis. A communicating fracture of the ethmoid bone into the orbit occurred intraoperatively. The nasal cavity was subsequently packed with gauze containing a petrolatum-based antibiotic ointment. Bilateral, periocular swelling developed 1 week later. Optical and electron microscopic studies revealed a paraffinoma.

Takayasu arteritis (TA) is an inflammatory arteriopathy involving predominantly the aorta and its main branches. The disease evolves in two phases: a first, nonspecific inflammatory stage and a late ‘pulseless’ stage, in which complications related to arterial stenosis and aneurysm formation predominate. In both phases, skin manifestations, such as inflammatory nodules, erythema-nodosum- and pyoderma-gangrenosum-like ulcers, have been described. We report 2 patients with TA, who had cutaneous necrotizing vasculitis as presenting manifestation of the disease. A review of the literature revealed 8 similar cases. TA does not only involve large arteries, but also small blood vessels. The observation that in TA the inflammatory process of the large arteries affects regions of the walls supplied by the vasa vasorum, the anatomy of which bears resemblance to the cutaneous vessel system, suggests that primary involvement of small vessels contributes to the development of the clinicopathological features of TA. Knowledge of the skin manifestations associated with TA remains important for its diagnosis and prompt instauration of life-saving treatment.

Fusarium is a saprophytic fungus of soil causing disease in plants and animals. In the immunocompetent patient, Fusarium is non-invasive, colonizing wounds, ulcers or nails. In the immunocompromised host, however, especially in those whose neutrophil and macrophage function is deficient, it can cause devastating systemic infections. Skin lesions are an early feature of the disseminated disease. Rapid diagnosis and treatment are mandatory in order to give the patient a better chance of survival, reported mortality rates being as high as 90%.

We present a 58-year-old patient with disseminated aspergillosis and metastatic panniculitis. He suffered from agranulocytosis and blastic transformation of a myelodysplastic syndrome. The skin lesion showed a single subcutaneous inflammatory nodule, which was clearly shown by histolog to be of embolic origin. This clinical presentation has not previously been described. Disseminated aspergillosis is found mainly in immunocompromised and neutropenic patients and the mortality is high. The present case demonstrates how histological examination together with skin biopsy culture can allow the diagnosis.

A diagnosis of aortic insufficiency and mitral stenosis was made in a 24-year-old woman after an episode of heart failure following delivery. A double valvular replacement was performed 4 years later. At that time she suffered from recurrent episodes of erythema nodosum like lesions with an histological diagnosis of cutaneous polyarteritis nodosa. After another 4-year interval she presented with severe arterial hypertension and a biological inflammatory syndrome. An arteriography disclosed stenoses of the abdominal aorta, renal, and iliac arteries as well as occlusion of the right subclavian artery, diagnostic of Takayasu arteritis. We postulate that this woman presented a rare combination of cardiac and skin involvement of Takayasu's disease which preceded the classical arteritis of the aortic branches by 8 and 4 years, respectively.

Laurence Toutous-Trellu, MD; Shahnaz Abraham, MD; Marc Pechere, MD; Pierre Chavaz, MD; Jan Lubbe, MD; Veronique Schiffer, MD; Bernard Hirschel, MD; Jean-Hilaire Saurat, MD; Vincent Piguet, MD, PhD; Departments of Dermatology and Venereology (Drs Toutous-Trellu, Abraham, Pechere, Chavaz, Lubbe, Saurat, and Piguet) and Internal Medicine, Division of Infectious Disease (Drs Toutous-Trellu, Schiffer, and Hirschel), University Hospital Geneva, Geneva, Switzerland.