1/5. Gianotti-Crosti syndrome presenting as lichenoid dermatitis.Papular acrodermatitis of childhood (Gianotti-Crosti syndrome) is an uncommon, self-limited disease characterized by an erythematous papular eruption symmetrically distributed on the face and limbs and mild lymphadenopathy, thought to be of viral origin. The histopathologic findings are nonspecific and include focal parakeratosis, mild spongiosis, superficial perivascular infiltrate, papillary dermal edema, and extravasated red blood cells. Interface changes with some basal vacuolization may be present, but are not a conspicuous feature. We present a 2 1/2-year-old boy with multiple papules and plaques on the face and extremities and cervical lymphadenopathy. Histopathologic analysis showed compact orthokeratosis, focal parakeratosis, hypergranulosis, psoriasiform epidermal hyperplasia, and a dense lichenoid lymphohistiocytic infiltrate with extensive exocytosis of mononuclear cells. Immunoperoxidase staining with CD 1 a revealed clusters of langerhans cells in the epidermis and in the papillary dermis. In view of the clinical findings, a diagnosis of Gianotti-Crosti syndrome was made. Although there are a few reports describing a lichenoid pattern of infiltration in Gianotti-Crosti syndrome, this histologic pattern is not widely known. This case is presented to illustrate the fact that Gianotti-Crosti syndrome can present as lichenoid dermatitis, and, especially in children, should be added to the differential diagnoses of lichenoid infiltrates.- - - - - - - - - - ranking = 1keywords = epidermis (Clic here for more details about this article) |
2/5. Benign cutaneous Degos' disease: a case report with emphasis on histopathology as papules chronologically evolve.The following case report details a 53-year-old man with a 6-year history of the benign cutaneous or skin-limited form of Degos' disease. Clinically, the patient demonstrated a diffuse eruption of papules on the upper trunk and arms. Many papules demonstrated the classic porcelain-white centers characteristic of Degos' disease, but others exhibited different clinical morphologies that corresponded to the evolutionary stages of papules originally described by Degos. Over the course of several clinic visits, the patient underwent a total of 5 punch biopsies, the histologies of which were correlated with their clinical morphologies. Early papules were skin-colored and demonstrated a superficial and deep perivascular, periadnexal, and perineural chronic inflammatory cell infiltrate associated with interstitial mucin deposition. The overlying epidermis showed a mild vacuolar interface reaction and the histologic appearances at this early stage resembled tumid lupus erythematosus. Fully developed papules were raised with umbilicated porcelain-white centers and a surrounding erythematous rim. Histologically these exhibited a prominent interface reaction with squamatization of the dermo-epidermal junction, melanin incontinence, epidermal atrophy, and a developing zone of papillary dermal sclerosis that resembled the early stages of lichen sclerosus et atrophicus in miniature. These interface reactions were invariably confined to the central portion of the punch biopsy specimen, corresponding to the central porcelain-white area seen clinically. Additional features of fully developed papules included a prominent lymphocytic vasculitis affecting venules, a mild periadnexal infiltrate of neutrophils and/or eosinophils, and interstitial mucin deposition. In late-stage papules, the porcelain-white areas were better developed and the lesion flattened. Histologically, the degree of inflammation was generally sparse and the overall picture mirrored the classic histologic description of Degos' disease with a central roughly wedge-shaped zone of sclerosis surmounted by an atrophic epidermis and hyperkeratotic compact stratum corneum. These late-stage papules closely resembled a miniaturized version of fully developed lichen sclerosus et atrophicus confined to the center of the punch biopsy specimen.- - - - - - - - - - ranking = 2keywords = epidermis (Clic here for more details about this article) |
3/5. Two men with toxic shock syndrome presenting with targetoid and spotty skin rashes.Two previously healthy men who presented with hypotension, constitutional symptoms, and targetoid and discrete spotty erythematous plaques were diagnosed with toxic shock syndrome based on histopathological findings. Specifically, their biopsies revealed necrotic keratinocytes, neutrophils in the epidermis, and neutrophils surrounding dilated superficial vessels. In one case, the diagnosis of toxic shock syndrome was confirmed with rising titers to toxic shock syndrome toxin-1. Both patients recovered with supportive care and clindamycin administration. We suggest that patients with fever, hypotension, constitutional symptoms and rash should be started on clindamycin and have a skin biopsy as part of their initial evaluation. An understanding that toxic shock syndrome can strike anyone has manifold dermatological manifestations and defined histopathological findings is important for its early diagnosis and effective treatment.- - - - - - - - - - ranking = 1keywords = epidermis (Clic here for more details about this article) |
4/5. food-induced acute generalized exanthematous pustulosis in a pregnant woman.A 21-year-old woman of Romany origin, in the third trimester of her fourth pregnancy, was admitted to the hospital because of a generalized erythematous and pustular eruption and desquamation involving her face, neck, trunk, and extremities. The skin changes were accompanied by fever (100.4 degrees F [38 degrees C]) and malaise. The patient was convinced that the dermatitis was induced by the consumption of "spoilt" pork sausage (bad smell, changed taste) approximately 24 hours earlier. Clinical examination revealed a woman with phototype III skin, black eyes, and black hair, in good general health. Widespread, symmetrical, moderately intense erythema and isolated or coalescing targetoid lesions studded with discrete, pinhead-sized, nonfollicular pustules in the center or at the periphery were distributed over her face, trunk, groins, and upper and lower extremities (Figures 1). On the neck and abdomen, lamellar desquamation was observed (Figure 2). Palms, soles, scalp, mucous membranes, hair, and nails were not affected. Nikolsky's sign was negative. The patient complained of very slight skin burning and itching. The pregnancy was proceeding without any complications and her obstetric status was normal. The woman had neither any accompanying diseases, nor previous personal or family history of psoriasis, nor any known allergies. She had taken no systemic medication (not even vitamins). She had three pregnancies; two ended with the delivery of healthy babies and one of them was aborted at her will. Laboratory studies revealed leukocytosis (13.2 x 109/L), neutrophilia (8 x 109/L), anemia (hemoglobin, 108 g/L), and an elevated erythrocyte sedimentation rate (68-110 mm/h). The results from the following investigations were normal: urinalysis, renal and hepatic function, serum albumin, Ca, Na, K, aspartate aminotransferase titer, cryoprotein, hepatitis b surface antigen, and serum markers for syphilis. Bacterial and fungal cultures of pustular content were sterile. A skin biopsy specimen of lesional skin revealed subcorneal pustules containing leukocytes and necrotic keratinocytes and a mixed perivascular inflammatory infiltrate with isolated eosinophils in the dermis (Figure 3). The patient was treated with systemic methylprednisolone in gradually reduced doses, fluocinonide cream 0.05%, and emollients. As a result, her fever disappeared and her erythema faded. Frequent obstetric examination and cardiotocography were normal and showed no evidence of placental insufficiency. At 40 weeks' gestation, the patient spontaneously gave birth without any complications to a healthy boy. She was discharged with complete resolution of the skin lesions, preceded by massive desquamation of the epidermis. The 1-year follow-up of the patient revealed no relapses or new pustular eruptions.- - - - - - - - - - ranking = 1keywords = epidermis (Clic here for more details about this article) |
5/5. Acute generalized exanthematic pustulosis. An atypical case due to nadoxolol?A 74-year-old woman developed a generalized pustular dermatosis with erythema-multiforme-like and vesiculobullous lesions 10 days after beginning a treatment by nadoxolol. Despite the clinical polymorphism of the eruption, histology constantly revealed the presence of subcorneal pustules, even within the epidermis overlying subepidermal bullae. The eruption spontaneously healed after discontinuation of nadoxolol.- - - - - - - - - - ranking = 1keywords = epidermis (Clic here for more details about this article) |