Scott D. Bennion

Neutrophilic eccrine hidradenitis associated with induction chemotherapy

We report three new cases of neutrophilic eccrine hidradenitis associated with induction chemotherapy which resolves two major points. First, because two of our patients had testicular carcinoma; this firmly establishes that NEH is not exclusively seen in patients with acute myelogenous leukemia or Hodgkins lymphoma as previously reported. Second, because two of our patients did not receive cytarabine which has previously been suspected of being the causative agent, it is apparent that this disorder may be produced by more than one chemotherapeutic agent or combination of agents. The histologic features with a discussion of the spectrum of changes which may be seen are presented.

Keratosis punctata palmaris et plantaris and adenocarcinoma of the colon: A possible familial association of punctate keratoderma and gastrointestinal malignancy

We report a patient with keratosis punctata palmaris et plantaris who developed adenocarcinoma of the colon. Family history revealed at least seven other individuals with punctate keratoderma. The patients mother had punctate keratoderma and carcinoma of the colon, and his maternal grandfather had punctate keratoderma and carcinoma of the pancreas. This is the third reported family, to our knowledge, in which hereditary palmar and plantar hyperkeratosis appears to be associated with gastrointestinal carcinomas.

Human keratinocytes maintain reversible anti-apoptotic defenses in vivo and in vitro

Human keratinocytes proliferate and differentiate in an epidermal environment where induction of apoptosis can be triggered by ultraviolet radiation (UVR), activated lymphocytes and cytokines. The purpose of this study was to determine whether keratinocytes were susceptible to apoptosis induced by ionophore, ultra-violet radiation, cytokines or crosslinking of CD95 (Fas/APO-1). In normal human skin exposed to two minimal erythema doses of ultraviolet radiation, suprabasal cells were the first keratinocytes to demonstrate apoptotic nuclei, and by 48 h apoptotic cells were identified throughout the mid to upper epidermis. However, most keratinocytes resisted apoptosis and UVR-induced apoptosis was not observed in basal cells, or in the most differentiated epidermis. Human keratinocytes and keratinocyte cell lines cultured in vitro developed maximal apoptosis 48 h after radiation. Human keratinocytes cultured in full growth factor supplements were resistant to UVR-induced apoptosis compared to keratinocyte cell lines or to a lymphoid cell line (HL60) susceptible to apoptosis. Keratinocyte cell lines were completely resistant to apoptosis induced by interferon-γ, interferon-α, IL-2, IL-6, TNF-α, IL-1Ra, and GM-CSF. A subset of the cells in cultures of keratinocytes and transformed keratinocyte cell lines died by apoptosis in response to anti-Fas, IL-1α and TNF-α plus IFN-γ and ionophore. Second passage freshly isolated human keratinocytes were much more resistant to apoptosis induced by ionophore, anti-Fas and cytokines than were transformed keratinocyte cell lines. Calcium shift to induce differentiation in second-passage keratinocyte cultures made keratino-cytes even more resistant to UVR-induced apoptosis. This parallels the lack of UVR-induced apoptosis observed in the most differentiated keratinocytes in irradiated human skin. Both keratinocytes and kerati-nocyte cell lines express rather low levels of the anti-apoptotic proteins bcl-2 and bcl-x compared to other apoptosis-resistant cell types. The differences between keratinocytes and keratinocyte cell lines in suscepti-bility to apoptosis are not explained by difference in expression of bcl-2 or bcl-x. Finally, withdrawal of growth factors from keratinocytes decreased cell survival following UVR and increased the induction of apoptosis. Inhibition of protein synthesis with cyclo-heximide also made keratinocytes more susceptible to UVR-induced apoptosis, indicating that anti-apop-totic defences in cultured keratinocytes are dependent on active protein synthesis. These experiments show that the strong keratinocyte defences against apoptosis are stratified within the epidermis, and can be altered by differentiation and growth factor withdrawal.

The influence of ultraviolet light on immunological cytotoxicity in the skin

David A. Norris*’, Kyu Whang2, Kathleen David-BajaP and Scott D. Bennion4 ’Department of Dermatology, University of Colorado School of Medicine and Department of Veterans Affairs Hospital, Denver, CO, USA; 2Department of Dermatology, Soon Chun Hyang University, Seoul, Korea; 3Department of Dermatology, Brooke Army Medical Center, San Antonio, TX, USA and 4Department of Dermatology and Department of Clinical Investigation, Fitzsimons Army Medical Center, Aurora, CO, USA

Chronic herpes gestationis and antiphospholipid antibody syndrome successfully treated with cyclophosphamide

Herpes gestationis shares many features with other bullous diseases, especially bullous pemphigoid. Treatments of benefit in bullous pemphigoid may also be effective for herpes gestationis. Cyclophosphamide, azathioprine, and other immunosuppressive agents have been used successfully in refractory cases of bullous pemphigoid. We describe a patient with severe and persistent herpes gestationis in the postpartum period and the antiphospholipid antibody syndrome, both of which were refractory to high-dose corticosteroid therapy. Treatment with pulsed-dose intravenous cyclophosphamide produced an excellent clinical response.

Palmar Purpura: An Atypical Presentation of Childhood Dermatitis Herpetiformis

Abstract: Dermatitis herpetiformis (DH) is seen most commonly as a pruritic, papulovesicular eruption in young children or adolescents. Differentiation from other bullous diseases of childhood may be difficult. We report the first case of an adolescent in whom pruritic, palmar, purpuric macules and papules were the only manifestations of DH. The patient later developed typical vesiculobullous extensor lesions and symptomatic gluten‐sensitive enteropathy (GSE). All lesions and GSE symptoms resolved with dapsone and a gluten‐free diet. Our purpose is to illustrate an unusual presentation of pediatric DH.

Occurrence of positive immunofluorescence in the dermo-epidermal junction of sun-exposed skin of normal adults

A bright, continuous, granular deposition of immunoreactants at the dermo‐epidermal junction (DEJ) of lesional skin is highly suggestive of cutaneous lupus erythematosus (LE). A recent study of the direct immunofluorescence (IF) of sun‐exposed skin in normal adults has demonstrated findings similar to the bright, continuous granular pattern found in cutaneous LE. This data suggests that positive IF from sun‐exposed cutaneous lupus lesions is nonspecific. Forty‐one healthy adults, without a history of dermatoses or photosensitivity, presenting to the dermatology clinic for the excision of skin cancers were studied. Excess non‐lesional tissue, removed from Mohs excision sites (sun‐exposed face and neck) in order to obtain appropriate cosmetic closure, was examined for the deposition of immunoreactants. The specimens were incubated with iluoresceinated monovalent anti‐human immunoglobulin specific for IgG, IgA, IgM, C3, Clq, and fibrinogen and examined independently by 2 immunodennatologists without prior knowledge of patient or site. None of the samples demonstrated immunoreactant deposition consistent with cutaneous LE. IF of several specimens (21/41) had a weak(1+ or 2+), interrupted pattern of fibrinogen at the DEJ, – a common, non‐specific finding. Weak, interrupted, linear and granular patterns were seen with IgM (10/41), Clq (9/41), IgG (2/41), IgA (2/41), and C3 (1/41). Fibrinogen was the only immunoreactant demonstrating a bright (3+), continuous, granular pattern (4/41). This data suggests that sun‐exposure alone does not induce the deposition of immunoreactants at the DEJ in a pattern similar to that found in cutaneous LE.

Etiologies of the sicca syndrome : Primary systemic amloidosis and others

Background. The sicca syndrome has been defined as the occurrence of xerostomia and xerophthalmia. Sjögrens syndrome is the most common cause of the sicca syndrome; however, these two syndromes are not synonymous and there are many potential etiologies of the sicca syndrome. A less known cause of sicca syndrome is amyloidosis that to date has only been reported in the nondermatology literature.

Hydroa Vacciniforme with Inflammatory Keratitis and Secondary Anterior Uveitis

Abstract: A 6‐year‐old boy had numerous episodes of hydroa vacciniforme. Several of these episodes were accompanied by an anterior aveitis with corneal clouding and stellate keratic precipirates. Wearing sunglasses prevented new eye lesions from developing despite recurrences of skin lesions. Phototesting on facial skin revealed reproductin of skin lesions with uhraviolet B but not ultraviolet A. One should be aware of eye involvement inhydroa vacciniforme, and children who experience this form of photodermatitis should have a careful eye examination and be advised to wear protective sunglasses.