Pearon G. Lang

Sulfones and sulfonamides in dermatology today.

Although dapsone and sulfapyridine have been used for years in dermatology, there has not always been a clear understanding of how these agents work. Recent investigation, however, has shed new light on thes agents which now allows a more rational approach to their use. This review is an attempt to familiarize the clinician with how these agents work, in what disease states they are effective, how to administer them, what adverse effects may occur, and how to monitor the patient to detect these adverse effects.

Coexisting lichen planus and bullous pemphigoid or lichen planus pemphigoides

A patient with lichen planus pemphigoides is reported. Initial pathologic and immunopathologic evaluation was consistent with coexisting bullous pemphigoid and lichen planus. However, further immunologic evaluation suggested that the circulating antibody was not directed toward bullous pemphigoid antigen but another basement membrane zone antigen. This finding indicates a need for immunologic re-evaluation of lichen planus pemphigoides.

The clinical spectrum of mid-dermal elastolysis and the role of UV light in its pathogenesis

BACKGROUND We observed four patients with mid-dermal elastolysis (MDE) that was either precipitated or aggravated by UV light (UVL) exposure or was primarily confined to areas of UVL exposure. OBJECTIVE Our purpose was to report four cases of MDE occurring after significant UVL exposure and to demonstrate why we suspect that MDE in some instances may be photoinduced or photoaggravated. We also wish to demonstrate the varied clinical presentation of this disorder. METHODS Because all our patients had MDE involving skin exposed to UVL, biopsies were performed on clinically uninvolved sun-exposed and sun-protected skin in one patient to elucidate further the role of UVL in this process. RESULTS On routine histopathologic examination we found that uninvolved sun-exposed skin but not sun-protected skin demonstrated early MDE. Ultrastructural examination revealed a spectrum of elastic fiber changes in involved and clinically uninvolved sun-exposed skin. Phagocytosis of elastic fibers was not present. CONCLUSION We conclude that UVL exposure was a major causative or aggravating factor of MDE in our patients and that the clinical appearance of this disorder is variable.

Current Concepts in the Management of Patients with Melanoma

Melanoma is a significant health problem. Despite public education and free cancer screenings, the incidence and mortality of melanoma continues to rise; however, many currently diagnosed melanomas are thin lesions, suggesting that education and awareness is having an impact. In addition, there are still subsets of patients who need increased surveillance in order to increase their survival.Although large congenital nevi may be precursors of melanoma, small and medium congenital nevi have an insignificant risk for melanoma development. Large congenital nevi, which are axial in location, appear to be more likely to develop melanoma and are associated with melanocytosis and melanoma of the CNS, both of which portend a poor prognosis.Recently, the recommended margins of excision have become more conservative so that many of the surgical defects can be closed primarily. Lymphoscintigraphy and sentinel node biopsy have replaced elective node dissections, thus decreasing the morbidity associated with the surgical management of melanoma. Although controversy still exists as to whether or not sentinel lymph node biopsy alters a patient’s prognosis, it has been shown to be a powerful prognostic indicator.Although most melanomas are managed by routine surgical excision, other modalities are sometimes employed. For example, cryosurgery or radiation therapy may be indicated in the frail, elderly individual with a large facial lentigo maligna. Mohs surgery is the treatment of choice for head and neck melanomas and those located in areas where maximum preservation of tissue is required and for desmoplastic and acral lentiginous melanomas. Much more work remains in the area of adjuvant therapy, chemotherapy, and immunotherapy. Dacarbazine remains the drug of choice in disseminated melanoma, but remissions are usually short lived. Interleukin and biochemotherapy has yielded good results but the percentage benefiting is small. Although high dose interferon increases disease-free and overall survival in some patients, it remains a controversial drug which is not easily tolerated.In the new staging system for melanoma, ulceration is second only to Breslow’s thickness. In transit (satellite) lesions have also been included in this new system. The new system also recognizes that patients with only microscopic metastatic nodal disease fare better than patients with clinically enlarged metastatic nodes and that it is the number of nodes involved with metastases, not their size, that determines the patient’s prognosis. Except for lesions <1mm thick, the Clark’s level of invasion has been de-emphasized.

Long-term management of basal cell nevus syndrome with topical tretinoin and 5-fluorouracil.

The case of a child with basal cell nevus syndrome whose condition was successfully managed for 10 years with a combination of topical 5-fluorouracil and tretinoin is reported. The concurrent use of these two agents prevented the development of new tumors, inhibited the growth of existing tumors, and caused the regression of superficially invasive basal cell carcinomas.

Severe ocular involvement in a patient with epidermolysis bullosa acquisita

A patient with epidermolysis bullosa acquisita and prominent ocular involvement that resulted in blindness is described. Because the severe eye involvement dominated the clinical picture, she had been diagnosed as having cicatricial pemphigoid. Clinicians should be aware that epidermolysis bullosa acquisita may mimic cicatricial pemphigoid and bullous pemphigoid.

The role of observation in the management of atypical nevi.

Objective: The definition and management of the atypical nevus remains a controversial issue. Some believe that atypical nevi are common variants of benign melanocytic nevi while others believe they are lesions intermediate between benign melanocytic nevi and melanoma. Therefore, the question of whether or not partially removed atypical nevi should be re-excised with clear margins in order to prevent their evolution into melanoma remains unanswered. Although studies have shown that most atypical nevi will never progress into melanoma, re-excision, when biopsy margins are positive, is commonly practiced. We argue that re-excision in such cases is not necessary. Methods: Our cohort study includes 55 previously biopsied atypical nevi that were not re-excised and which were followed for at least 5 years with a mean follow up time of 6.12 years. Results: The experimental group included 26 atypical nevi whose biopsy revealed at least one involved margin. The control group included 29 atypical nevi whose biopsy revealed clear margins. No melanomas were observed to arise in association with a pre-existing atypical nevus in either the experimental or control group during the follow-up period. Conclusions: The results of our study support observation as a safe alternative to re-excision for incompletely removed atypical nevi. A large prospective study with longer follow up would be necessary to better answer the question of how often atypical nevi evolve into melanoma and over what time period this occurs.

Skin cancer of the head and neck

The incidence of skin cancers is increasing at a rate greater than any other cancer occurring in humans. In this era of managed care, patients with a suspicious skin lesion may first present to their primary care physician for evaluation. Therefore, it is important for the primary care physician to be able to distinguish between benign and malignant pigmented lesions, to know how to evaluate such a patient, and to appreciate the importance of appropriate interdisciplinary management of these patients.

Probable coexisting exogenous ochronosis and mercurial pigmentation managed by dermabrasion

A patient with blue-gray discoloration of the face is described. Her history revealed that she had used bleaching creams containing mercury and hydroquinone for many years. Biopsy specimens of the hyperpigmented areas showed deposits that were compatible with both mercury deposition and the diagnosis of exogenous ochronosis. Dermabrasion was successfully employed to remove these deposits.

Quinidine-induced photodermatitis confirmed by photopatch testing

A patient with quinidine-induced photosensitivity confirmed by photopatch testing is presented. The action spectrum for the reaction appeared to be in the UVA range, and the patients minimal erythema dose was lower than expected while the patient was on quinidine.