ABSTRACT
BACKGROUND: Fibrosing alopecia in a pattern distribution (FAPD) is a primary cicatricial alopecia considered a subtype of lichen planopilaris (LPP). OBJECTIVE: The clinical and histopathological features, and treatment response of 24 female patients with FAPD were evaluated. METHODS: Demographic, clinical, histopathological and treatment data of patients with FAPD were retrospectively collected. RESULTS: Twenty-four women were included (mean age 60.7 years). The mean Lichen Planopilaris Activity Index score was 1.50, and the median Sinclair grade was 3. Twelve patients had diffuse alopecia in the centroparietal region, four had frontal accentuation and eight had vertex accentuation of hair loss. Eight had associated loss of facial hair. Predominant trichoscopic features included hair shaft diameter variability (100%), perifollicular erythema and/or scaling (95%) and loss of follicular ostia (95%). Histopathological examination revealed features of scarring alopecia with interface changes in follicular epithelia mainly targeting terminal and intermediate hairs, and less frequently, vellus hairs. Treatment with hair growth-promoting, anti-inflammatory and anti-androgen agents arrested disease progression in 14 patients and resulted in hair regrowth in two patients. Six patients had progression of their hair loss in spite of treatment. LIMITATIONS: Referral bias to a specialist hair clinic, retrospective design and small sample size. CONCLUSIONS: We present clinicopathological features of FAPD which can aid in the diagnosis of this insidious scarring alopecia. Our findings suggest a more favourable outcome if treatment is initiated in early stages of the disease.
Subject(s)
Alopecia Areata , Lichen Planus , Alopecia/drug therapy , Female , Humans , Middle Aged , Retrospective Studies , Scalp , Treatment OutcomeSubject(s)
Botulinum Toxins, Type A/administration & dosage , Dermatitis, Irritant/drug therapy , Neuromuscular Agents/administration & dosage , Surgical Stomas/adverse effects , Adult , Aged , Aged, 80 and over , Botulinum Toxins, Type A/adverse effects , Dermatitis, Irritant/etiology , Female , Humans , Injections, Intramuscular/methods , Male , Middle Aged , Neuromuscular Agents/adverse effects , Treatment OutcomeSubject(s)
Leg/pathology , Nerve Sheath Neoplasms/pathology , Peripheral Nerves/pathology , Peripheral Nervous System Diseases/pathology , Skin Neoplasms/pathology , Aged , Dermoscopy/methods , Humans , Immunohistochemistry/methods , Leg/innervation , Male , Mucin-1/immunology , Nerve Sheath Neoplasms/immunology , Nerve Sheath Neoplasms/surgery , Peripheral Nerves/immunology , Peripheral Nerves/surgery , Peripheral Nervous System Diseases/immunology , Peripheral Nervous System Diseases/surgery , Skin Neoplasms/diagnostic imaging , Skin Neoplasms/immunology , Skin Neoplasms/surgerySubject(s)
Facial Dermatoses/genetics , Hair Diseases/complications , Hair Follicle/abnormalities , RecQ Helicases/genetics , Rothmund-Thomson Syndrome/complications , Rothmund-Thomson Syndrome/genetics , Facial Dermatoses/diagnosis , Frameshift Mutation , Humans , Infant , Male , Rothmund-Thomson Syndrome/diagnosisABSTRACT
Laugier-Hunziker syndrome (LHS) is a rare acquired disorder characterized by macu-lar pigmentation of the lips and oral mucosa, with frequent longitudinal melanonychia. Involvement of other areas, such as the genitalia and fingers, has rarely been described. LHS is a benign condition with no known systemic manifestations. We report the case of a woman who developed melanotic macules on her fingers and elbow 16 years after the onset of pigmentation of her lips. This unusual feature of LHS in our patient was associated with irritant contact dermatitis and viral warts. Only two cases of an association with an inflammatory dermatosis have been reported previously in the literature.
Subject(s)
Dermatitis, Contact/pathology , Hyperpigmentation/pathology , Warts/pathology , Elbow , Female , Fingers , Hand Dermatoses/pathology , Humans , Irritants/adverse effects , Lip Diseases/pathology , Middle Aged , Syndrome , Tongue Diseases/pathologyABSTRACT
Scleromyxoedema is a rare disease characterized by a generalized papular and sclerodermoid cutaneous eruption. It is associated with fibroblast proliferation and mucin deposition in the dermis. Most patients have a monoclonal gammopathy, defined by the presence of IgG. Treatment of scleromyxoedema is challenging, but there is mounting evidence to support the use of intravenous immunoglobulin (IVIg). Individual reports of systemic complications have been described. Dermatoneuro syndrome (DNS) is a rare but sometimes fatal manifestation, which consists of a triad of fever, coma and seizures preceded by a flu-like illness. We describe a patient with scleromyxoedema who developed DNS. Our case highlights interesting findings suggesting that DNS may have a viral aetiology. In addition, this case demonstrates a favourable response of the cutaneous features of scleromyxoedema to IVIg.
Subject(s)
Influenza, Human/complications , Scleromyxedema/complications , Seizures/virology , Humans , Influenza A virus/isolation & purification , Male , Middle Aged , Paraproteinemias/etiology , SyndromeABSTRACT
Atrophoderma vermiculatum (AV) is a rare follicular dermatosis presenting with 'wormeaten' or 'honeycomb' atrophy, usually on the cheeks and sometimes extending to the preauricular areas and forehead. It predominantly affects children and follows a slowly progressive course. The treatment of AV is challenging. We describe a rare case of unilateral AV in a young boy. The scarring in AV is characteristically limited to the face, but our patient had lesions extending to the concha auriculae and the neck. He also had an ipsilateral congenital cataract. Such an association has only been reported once before in the literature.
