Efficacy of ablative laser treatment in Galli-Galli disease.

BACKGROUND: Galli-Galli disease (GGD) represents a rare genodermatosis that is clinically characterized by progressive reticulate hyperpigmentation of the flexures accompanied by itching and pain. To date, no convincing treatment options are known for GGD. We sought to examine the therapeutic potential of ablative laser treatment in a patient with genetically confirmed GGD. OBSERVATIONS: We describe a 68-year-old man with refractory bilateral axillary GGD accompanied by severe itching and pain. His clinical picture and histologic findings were consistent with GGD. Direct sequencing analysis of the KRT5 gene identified the causative mutation, c.418dupA, and confirmed the clinical diagnosis. The patient was treated with the erbium:YAG laser in 2 consecutive sessions for each side and was followed up for 12 months. Ablative laser treatment led to complete resolution of clinical symptoms and a good clinical result with only minimal scarring and dyspigmentation. CONCLUSIONS: This article demonstrates the efficacy of ablative laser treatment in a patient with genetically confirmed refractory GGD. However, further studies with longer follow-up are required to confirm these results.

Acantholytic squamous cell carcinoma of the gingiva: report of a case and review of the literature.

Adenoid squamous cell carcinoma differs from common squamous cell carcinoma in histologic features and its aggressive nature. Microscopically, the tumor shows cystic degeneration of the neoplastic epithelium, producing a prominent alveolar pattern and pseudoglandular structures with acantholytic cells. It occurs most commonly on the lips, rarely intraorally, and it is associated with a poor prognosis. This case concerns a 72-year-old woman who presented with the chief complaint of burning tongue and soreness of the lips. Clinical examination revealed an ulcerated and elevated mass on the edentulous left maxillary ridge, beneath the base of a partial denture. An incisional biopsy rendered the diagnosis of adenoid squamous cell carcinoma. The patient was referred to a specialized maxillofacial surgery center for diagnostic work-up and treatment. She underwent partial maxillectomy and radiotherapy, and 17 months after treatment, she died of uncontrollable recurrence.

Shadow cell basal cell carcinoma with acantholysis.

I present histologic documentation of a unique basal cell carcinoma (BCC) in which shadow cells formed the major cellular component along with extensive acantholysis and the development of ringed shadow cells. This neoplasm contained trichohyalin granules, which are indisputable evidence of follicular differentiation. Shadow cells rarely are encountered within BCCs and generally form relatively small components. Such neoplasms have been labeled BCC with matrical differentiation. Because of nonspecificity and ambiguity, I propose that this terminology be abandoned and replaced by shadow cell BCC.

Acantholytic dermatosis of the vulvocrural area.

Acantholytic dermatosis of the vulvocrural area is a rare skin disorder characterized by solitary or multiple skin-colored to white, smooth papules or plaques. Histopathological features of both Hailey-Hailey disease and Darler's disease are present. There is acantholysis, which may involve the full thickness of the epidermis, and dyskeratosis with corps ronds and grains. There may be marked hyperkeratosis and focal parakeratosis. We report a case of this rare disease and discuss its differential diagnosis and treatment.

Type 2 segmental manifestation of Hailey-Hailey disease: poor therapeutic response to dermabrasion is due to severe involvement of adnexal structures.

In autosomal dominant skin conditions, two different types of segmental manifestation can be distinguished. Type 1 represents heterozygosity for a postzygotic mutation, resulting in a degree of severity similar to that of the nonmosaic phenotype. Type 2 reflects loss of heterozygosity and shows an excessively pronounced involvement superimposed on the ordinary nonsegmental phenotype. We describe the clinical, histopathological and therapeutic aspects of the first case of type 2 segmental manifestation of Hailey-Hailey disease (HHD). A 24-year-old woman with a family history of HHD comprising four generations, presented with lesions of erythema and blistering arranged in a unilateral pattern following the lines of Blaschko. The disorder was first noted at the age of 3 months. At the age of 24 years, additional scattered symmetrical lesions involving the axillary and inguinal folds were noted. Histopathological examination of the severely involved linear skin areas revealed pronounced acantholysis within the deep adnexal structures, whereas clinically unaffected skin showed the typical histopathological features of the heterozygous phenotype with suprabasal clefting and acantholysis sparing the adnexae. Dermabrasion was performed in the areas of segmental involvement. During a follow-up period of one year, no recurrence was noted, but 18 months after dermabrasion a recurrence was present in the left submammary and left perianal regions. This therapeutic resistance to dermabrasion may be explained by the presence of acantholysis within the adnexal structures of the skin as found in type 2 segmental HHD.