Dermatology Online Journal

Background: Sweet syndrome is an uncommon skin condition, often idiopathic in origin although it may be reactive to various systemic conditions, recent infections, underlying malignancies, and medications.

Objective & Method: To present a case highlighting a rare clinical presentation and to review the causes of Sweet syndrome with an emphasis on drug-induced etiologies.

Results: We describe a 45-year-old woman who developed Sweet syndrome while receiving nitrofurantoin and ciprofloxacin for a urinary tract infection.  Her course of disease was complicated by arthralgias, episcleritis, headaches, and erythema nodosum-like subcutaneous involvement.  There was marked improvement with discontinuation of the inciting antibiotics and initiation of systemic steroids.

Conclusion: This case illustrates Sweet syndrome related to nitrofurantoin and/or ciprofloxacin. This is the second report of Sweet syndrome related to these antibiotics and the first associated with ocular, joint, and neurologic involvement.

Primary malignant melanoma of the ovary arising in a cystic teratoma is extremely rare. There is no melanocytic component in the normal ovarian tissue. However, melanocytes may be present in a cystic teratoma of the ovary, especially at the basal layer of squamous epithelium. We report a case of primary malignant melanoma of the ovary arising in a cystic teratoma. We demonstrate junctional activity of melanocytes showing malignant transformation with both radial and vertical growth phases, a feature diagnostic for primary malignant melanoma but not always demonstrated in such cases. We compiled and reviewed twelve cases that are also able to demonstrate junctional activity.

Cutis tricolor was first described in a 17-year-old male patient by Happle et al. as a rare coexistence of circumscribed hyperpigmentation and hypopigmentation close to each other on a background of normally pigmented skin. Cutis tricolor has been reported as an isolated cutaneous finding or in various associations. To the best of our knowledge, cutis tricolor in association with teratoma and holoprosencephaly has not been reported in the literature. Herein, we report a male patient who presented with a teratoma and a combination of whorl-like hypopigmentation together with hyperpigmented patches adjacent to each other on intermediately pigmented skin. This case report supports the view that cutis tricolor may be a marker of an underlying neurological abnormality.

Pagetoid reticulosis, also known as Woringer-Kolopp disease, is a rare variant of cutaneous T-cell lymphoma usually presenting as an isolated patch or plaque on the extremities.  Immunohistochemical staining of T-cell markers can be variable, but as the name implies the epidermotropic lymphocytes consistently display a “pagetoid” appearance.  The following case demonstrates clearance of this condition with intensity modulated radiation therapy, whereas most cases are managed with electron beam therapy if radiation therapy is implemented.

Multiple myeloma (MM) is a rare cancer. Cutaneous involvement is uncommon with fewer than 100 cases described in the literature. We report herein a patient with MM, subtype IgA kappa, with unusual clinical presentation including the lower lip.

Contact (allergic) dermatitis is a skin disorder related to natural exposure to various allergens. Systemic contact dermatitis (SCD) describes a cutaneous eruption in response to systemic exposure to an allergen. The exact pathologic mechanism remains uncertain. Herein we describe a 36-year-old woman with symmetric systemic allergic contact dermatitis, unresponsive to conventional treatment, associated with dental alloy-contact hypersensitivity. We did skin patch testing and the blood lymphocyte transformation test (LTT) from the dental allergen series to assess contact allergy to restorative dental materials. On patch testing, positive allergic contact dermatitis reactions to metals occurred (nickel, potassium dichromate, and gold). Nickel hypersensitivity was confirmed by LTT, which also revealed silver-amalgam sensitization. Our case report highlights the need to consider adverse reactions to base-metal dental alloys in the differential diagnosis of cases of systemic allergic contact dermatitis.

Mid-dermal elastolysis (MDE) is an uncommon and probably underdiagnosed disorder of the elastic tissue. Clinical suspicion and histopathological confirmation are essential for the diagnosis. We report the case of a young woman who presented with this disorder after an inflammatory process during pregnancy and we review the main characteristics of this rare entity.

Ruxolitinib, a small molecule JAK-1/2 inhibitor, was approved by the U.S. Food and Drug Administration (FDA) in November 2011, as the first therapeutic for the treatment of intermediate and high-risk myelofibrosis.  The Janus kinase/signal transducers and activators of transcription (JAK/STAT) pathway is one of the most well-studied intracellular signaling networks.  Recent advances in our understanding of the complexities of signal activation and regulation of gene expression has provided opportunities for targeted therapeutic interventions.  Although numerous inhibitors of the JAK/STAT pathway are currently being evaluated in clinical trials, ruxolitinib represents the first FDA approved in-class JAK inhibitor. We report a drug eruption associated with ruxolitinib.

Background: Crusted scabies is a severe, hyperkeratotic, psoriasiform disorder associated with immune suppression. Affected individuals typically present with crusted hyperkeratotic lesions in a variety of locations. This condition can lead to severe complications: institutional outbreaks and secondary bacterial infections associated with sepsis and high mortality.Main observations: A 37-year-old woman with a 12-year history of systemic lupus erythematosus treated with prednisone, methotrexate, and plaquenil presented with a three-week history of a painful scalp rash with adherent yellow scale. Skin biopsy and tissue culture were consistent with a diagnosis of crusted scabies with superficial bacterial infection. The patient was treated with oral ivermectin and permethrin cream, as well as ciprofloxacin for the bacterial infection. At one-week follow-up, the scalp was no longer tender and hyperkeratotic plaques had significantly improved. At one-month follow-up, the affected scalp demonstrated further improvement with decreasing erythema and alopecia with follicular ostia.Conclusions: Our case highlights the atypical presentation of crusted scabies with primary scalp involvement and need for vigilance in recognizing and appropriately treating this condition to prevent the consequences of longstanding infection. Combination treatment with ivermectin and permethrin is appropriate management for this condition.

Hailey-Hailey disease is a rare chronic skin disorder that is inherited in an autosomal dominant manner. The disease is characterized by development of vesicles and bullae typically in the intertriginous areas. On histology, there is widespread intraepidermal acantholysis causing the “dilapidated brick-wall” appearance. Mutations in the ATP2C1 gene, encoding for P-type Ca2+ transport ATPase, is the primary cause of the disease. The disease manifests around puberty and runs a chronic course with remissions and exacerbations. Ultraviolet light exposure, sweating, friction, stress, and cutaneous infections are the known precipitants of the disease. We report a case of a woman with recurrent flare-ups of Hailey-Hailey disease with repeated pregnancies and remission of her disease during non-pregnancy periods. To our knowledge, this is the first reported case of Hailey-Hailey disease exacerbated by pregnancy.

Hair-thread tourniquet syndrome (HTTS) is caused by circumferential constriction of an appendage, usually by hair or thread, leading to obstruction of circulation and ischemia. Although not rare, this entity is not discussed extensively in the Dermatology literature. We present a case of HTTS and discuss the demographics and etiology, and review the most current treatment methods.

Eccrine nevus (EN) is a very rare hamartoma of the skin and with varying clinical manifestations. Histologically, these neoplasms present as a proliferation of normally structured eccrine sweat glands in the dermis. There have been no more than 20 cases previously have been reported in the English literature. Herein we report a 25-year-old man with eccrine nevus on the neck. To our best knowledge, this is the first case that the lesion affecting the neck.

Background: The prevalence of rosacea in skin of color is not well characterized and may be underestimated. Physicians may not recognize and diagnose rosacea correctly in skin of color.Purpose: To assess the prevalence of rosacea in skin of color and determine if patients of color with rosacea symptoms are receiving a diagnosis of rosaceaMethods: We analyzed the National Ambulatory Medical Care Survey (NAMCS) for 1993-2010 for racial and ethnic distribution of patients with rosacea. Common reasons for visit in rosacea patients were tabulated and frequency of rosacea diagnosis was compared in patients of each race with the relevant reasons for visit.Results: Of all patients diagnosed with rosacea, 2.0% were black, 2.3% were Asian or Pacific Islander, and 3.9% were Hispanic or Latino of any race. Leading reasons for visit associated with rosacea included “other diseases of the skin”, skin rash, and discoloration or abnormal pigmentation. Rosacea was the primary diagnosis for 8.3% of whites and 2.2% of blacks complaining of “other diseases of the skin”, for 2.0% of whites and 0.6% of blacks complaining of skin rash, and for 3.0% of whites and 0.0% of blacks complaining of discoloration or abnormal pigmentation. The percentage of rosacea patients who were black or Asian/Pacific Islander did not change significantly over time.Limitations: No specific reason-for-visit code indicating rosacea exists in the NAMCS. Prevalence may be underestimated if some patients do not visit a physician for treatment.Conclusions: Patients of color rarely receive a diagnosis of rosacea, even when they have symptoms suggesting it. Rosacea has not become more commonly diagnosed in skin of color in recent years.

Enoxaparin sodium is a heparin of low molecular weight with antithrombotic properties that acts by inhibiting factor Xa. We present a 59-year-old woman who developed heparin-related bullous hemorrhagic dermatosis, at sites distant from the injections, one month after starting treatment with enoxaparin.

A 52-year-old man with recurrent metastatic rectal carcinoma being treated with cetuximab presented to the emergency department with a diffuse papulopustular eruption on the face, scalp, chest, and groin, accompanied by patchy alopecia of the scalp and facial hair.

Scabies limited to the nail unit is quite unusual, but may persist after treatment of crusted scabies. We present a man with a history of crusted scabies that resolved with treatment, but later the patient reported a chronic problem with crumbly, thickened nails, which were found to be harboring scabies mites.

Cutaneous larva migrans (CLM) is a pruritic dermatitis caused by migration of the animal hookworm larvae into the epidermis. We present a case of CLM in a 31-year-old pregnant woman. The treatment of CLM relies on antihelminthic agents, such as thiabendazole, albendazole, and ivermectin. This case was interesting in that the standard treatment options previously mentioned were contraindicated owing to the patient’s pregnancy. Cryotherapy with liquid nitrogen resulted in complete resolution of her lesion and symptoms.

Although thalidomide is a U.S. Food and Drug Admistration (FDA) approved medication for erythema nodosum leprosum and multiple myeloma, it has many off-label uses, including for discoid lupus erythematosus (DLE), Behçet’s disease, apththous ulcers in HIV patients, and prurigo nodularis. Herein, we present a patient with an overlap of discoid lupus erythematosus and lichen planus who was successfully treated with thalidomide for over 19 years without significant side effects. We propose that some of the most common side effects, including peripheral neuropathy, numbness, parasthesias, sedation, and constipation, can be avoided at lower doses, typically less than 100mg/day.