308-nm excimer laser in the treatment of a localized recalcitrant form of perioral dermatitis: Case report

In the last two decades, 308-nm excimer laser has been increasingly recognized as a therapeutic alternative for several dermatological conditions, being currently FDA approved for the treatment of localized vitiligo and moderately severe localized psoriasis unresponsive to topical treatments. We describe the case of a 17-year-old with a recalcitrant form of dermatitis occupying the entire perioral region, previously unresponsive to several treatments, who was treated with 308-nm excimer laser with an excellent result. 308-nm monochromatic excimer laser has several advantages over other types of phototherapy, including lower UV dose exposure, shorter courses of therapy and a better sparing of adjacent tissue. Although infrequently used, 308-nm excimer laser certainly has a vast potential in Dermatology, particularly regarding recalcitrant and localized inflammatory conditions, such as the one we present.

Necrolytic erythema migrans in celiac disease

Erythema migrans necrolytica is a red, blistering rash that spreads over the skin. It particularly affects the skin around the mouth and distal extremities, but can also be found on the lower abdomen, buttocks, perineum and groin. It is strongly associated with glucagonoma, a glucagon-producing tumor of the pancreas, but is also seen in a number of other conditions, including liver disease and intestinal malabsorption such as celiac disease. We present a case of a patient with a history of poorly followed celiac disease presenting with a clinical picture of ENM. This rare case adds to our understanding of the clinical presentation of NME, as well as highlights the importance of acting in a timely manner to avoid the most redoubtful complications.

Solitary skin-colored nodule on a child’s face

Sir, A healthy, six-year-old boy presented with a slowly grown dome-shaped nodule on the mandibular angle region present for two years. The patient’s past medical and family history were unremarkable. A physical examination revealed a solitary, 1.3 × 1 cm, firm, painless, flesh-colored tumor (Fig. 1). Dermoscopy showed branching, serpentine vessels on a pink background (Fig. 2a). These features disappeared when slight pressure was exerted on the dermoscope and the tumor exhibited a central, white, structureless area (Fig. 2b). An excisional biopsy was performed. A microscopic examination showed a well-circumscribed, paucicellular dermal tumor composed of eosinophilic collagen bundles separated by clefts and forming a storiform pattern. Scattered fibroblasts were found among the collagen bundles. The overlying epidermis was slightly flattened (Fig. 3). The diagnosis of solitary sclerotic fibroma was established. Sclerotic fibroma (SF), also known as storiform collagenoma, is a rare benign skin tumor. It usually manifests itself as an asymptomatic, slowly growing, white-to-skin-colored papule or nodule [1]. It was first described in patients with Cowden’s disease, yet may also occur sporadically [2]. There were no mucocutaneous features of Cowden’s disease (tricholemmomas, oral fibromas, acral keratoses, palmar pits, and gingival and palatal papules) in the patient and her family members. Dermatofibroma, the main differential diagnosis of SF, usually exhibits hyperplastic changes of the epidermis instead of atrophy, and the boundaries of the lesion are unclear [2]. Only two papers have been published describing the dermoscopic findings of SF, consisting of a white background with peripheral arborizing vessels [3]. A white background may be related to an increased dermal collagen density. It is also described in dermatofibroma, typically with a peripheral pigmentation network. Although dermoscopy may improve the clinical diagnosis of SF, histopathological analysis is required.

Treatment with intralesional methotrexate injection in a patient with nail psoriasis

Psoriasis vulgaris is an inflammatory skin disease involving the skin, nails, and joints. While nail involvement is observed in 70–80% of patients with psoriasis, the rate of patients with isolated nail involvement is 5–10%. Dystrophies arising in the nails in psoriasis affect the patient’s quality of life, and local and systemic therapies may be used as treatment. Intralesional methotrexate or corticosteroid injection might be an option in the treatment of patients with the involvement of one nail or some nails or without the involvement of the skin and joints, due to the side effects of systemic and biological agents. Herein, we report a female patient with nail psoriasis resistant to a previously applied topical treatment, the efficacy of intralesional methotrexate without the use of a systemic antipsoriatic agent, and no progression of side effects.

A relapse of pemphigus vulgaris in pemphigus herpetiformis or a phenotypic “switch”

Sir, Pemphigus herpetiformis (PH) was originally described by Jablonska et al. in 1975. Clinically, PH presents itself as a herpetiform dermatitis with immunopathological characteristics of pemphigus [1,2]. We report an exceptional case of typical pemphigus vulgaris (PV) relapsing after 36 years in PH. A 65-year-old patient, followed for PV for 36 years and treated with corticosteroid therapy with a remission for more than thirty years, consulted for pruriginous lesions evolving for the previous eight months. A dermatological examination revealed urticariform pruriginous ring lesions surmounted by small peripheral vesicles spread throughout the body (Fig. 1), sparing the mucous membranes, and without Nikolsky’s sign. After two non-specific skin biopsies, the histological examination revealed an intraepidermal bubble with acantholytic cells and eosinophilic spongiosis (Figs. 2a and 2b). Direct immunofluorescence confirmed the diagnosis of pemphigus and indirect immunofluorescence was at the upper limit. The diagnosis of a PV relapse in PH was retained and a dapsone-based treatment was initiated at a dose of 150 mg/day and stopped seven days later when met with hemolytic anemia. Oral corticosteroid therapy involving prednisone at a dose of 1 mg/kg/day was initiated but, given the persistence of the pruritus, the decision was to combine methotrexate at a dose of 12.5 mg/week. A good evolution and a decline within eight months were observed. An improved pruritus and the disappearance of the skin lesions were achieved after one month of treatment. PV and PH are two different anatomical and clinical entities of the autoimmune disease pemphigus, with distinct clinical, histopathological, and immunopathological characteristics [1,2]. Our observation documents a complete phenotypic “switch” of pemphigus with a transition from PV to PH both clinically, histologically, and immunologically. Several rare cases of PV switching to superficial pemphigus (SP) (“phenotypic switch”) have, since 1991, been reported, with a higher frequency this direction than otherwise; the transition period varies from six months to twenty years [3]. To the best of our knowledge, no case has been described of a progression from PV to PH. Having observed one firsthand, we are first to describe the case of a complete phenotypic switch from PV to PH. The mechanism of such a transition remains poorly understood and is often observed during a relapse. Some authors suggest that the effect of immunosuppressants on the desmoglein DSG3 more marked than on DSG1 could explain the relapse of PS in PH [3,4]. Future studies on the immunological factors and predictors of PV relapses after the discontinuation of treatment would be useful to better understand the mechanisms of a relapse in pemphigus, with or without a phenotypic transition.

Lupus vulgaris mimicking cutaneous leishmaniasis: A case report

Lupus vulgaris (LV) is a progressive, chronic form of cutaneous tuberculosis (CTB). The head and neck regions are the most commonly affected sites, followed by the arms and legs. Occurring in unusual sites may pose diagnostic difficulties. Herein, we report a case of LV present on the dorsal aspect of the right hand in a twenty-year-old Saudi male. It was misdiagnosed as leishmaniasis as the patient lived in an area in which it was endemic, and was treated accordingly with no benefit. A skin punch biopsy was taken and the diagnosis of LV was confirmed. The lesion responded well to anti-tubercular therapy (ATT), yet healed with atrophic scarring. Although rare, clinicians must be aware of the importance of considering CTB as an important differential, as misdiagnosis or delayed diagnosis of this entity may eventually cause prolonged morbidity.

A clinical and epidemiological study of non-venereal genital dermatoses: A cross-sectional, hospital-based study from Nepal

Background: Non-venereal genital dermatoses are the conditions of the genitalia that are not transmitted sexually. They may be confused with venereal diseases and be responsible for concerns among patients as well as diagnostic dilemmas for physicians. This study was conducted to determine the prevalence and describe the patterns of non-venereal genital conditions. Methods: This was a hospital-based, cross-sectional, prospective study conducted in a tertiary center in Kathmandu, Nepal, over a period of one year. Non-probability purposive sampling was employed to select the samples. Two hundred patients were enrolled in the study. Ethical approval was taken prior to the study. Detailed history taking along with a complete cutaneous examination were conducted for all patients and recorded in a preformed proforma. Results: Among 21366 patients, two hundred patients had non-venereal genital dermatoses. The prevalence of non-venereal dermatoses was 0.93 %. The mean age of the patient was 29.5 ± 15 years, ranging from 2 months to 81 years. The male-to-female ratio was 2.7:1. Itching was the most common presentation (46%). Fifty-four different types of non-venereal diseases were encountered and classified into inflammatory lesions (n = 84; 42%), infections and infestations (n = 43; 21.5%), normal variants and benign abnormalities (n = 41; 20.5%), and miscellaneous (n = 21; 10.5%). The most common were, among inflammatory dermatoses, drug reactions (11.5%) and eczema (6.5%) and, among infections and infestations, scabies (9.5%) and fungal infections (7.5%). Conclusion: Non-venereal genital dermatoses are important yet less common dermatological conditions. A number of patients have misconceptions about them as venereal. A comprehensive study of non-venereal dermatological genital conditions is required for careful management to minimize morbidity.

Uncommon sublingual ulceration in an infant

Sir, An eight-month-old girl was referred to our department with an extensive lingual ulceration. The parents noted that she had habitually bitten her tongue since the release of her first teeth at the age of six months. She was a poor feeder and did not sleep well because of the painful lingual ulceration. There was no family history of developmental disorders or congenital syndromes. Intraoral examination revealed a deep, circular, and extensive ulceration of the whole ventral surface of the tongue with intermittent bleeding in the tongue (Figs. 1a – c). An examination of the rest of the intraoral mucosa revealed that the lower central incisors had recently erupted. However, there were two other ulcerations of the palmar surface of the second and third fingers caused by nocturnal finger biting. Neurological examination noted a lack of pain sensitivity related to peripheral neuropathy diagnosed as congenital insensitivity to pain. Based on the clinical features and the particular site on the ventral surface of the tongue against the lower central incisors and ulcerative lesions of the fingers due to self-biting, the lesion was diagnosed as Riga–Fede disease. Because of the size of the ulceration, significant pain during feeding led to inadequate nutrient intake associated with permanent sleep disturbances. Radical treatment was chosen and the lower central incisors were extracted. Topical corticosteroids were prescribed to help with healing. The term Riga–Fede disease has been used to describe a traumatic ulceration that has occurred on the ventral surface of the tongue in newborn babies and infants. It is most commonly related to neonatal or natal teeth but may also occur in infants after the eruption of the primary lower incisors [1]. This benign ulceration occurs as a result of repetitive mechanical trauma caused to the oral mucosal surfaces by the teeth and is most commonly located on the ventral surface of the tongue against the teeth [1,2]. Riga–Fede disease may reveal an underlying developmental or neurologic disorder, including congenital insensitivities to pain [3]. The case of our patient was associated with congenital insensitivity to pain. Failure to diagnose may lead to dehydration and inadequate nutrient intake in the infant because of the significant pain during feeding. No biopsy is needed. The diagnosis of Riga–Fede disease is based on clinical characteristics [1,2]. Treatment should focus on eliminating the source of trauma. Conservative treatment is attempted at first by grinding the sharp edges of the teeth and placing composite resin in a dome shape or by placing a protective ring. If conservative treatment fails to heal the wounds, radical treatment may be necessary, such as extraction of the teeth [2,3]. We believe that Riga–Fede disease must be recognized by clinicians to avoid misdiagnosis and delayed treatment.